925 C OPYRIGHT Ó 2015
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T HE J OURNAL
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S URGERY, I NCORPORATED
Chondroblastoma of Bone in the Extremities A Multicenter Retrospective Study Hairong Xu, MD, Dylan Nugent, MD, Hector L. Monforte, MD, Odion T. Binitie, MD, Yi Ding, MD, G. Douglas Letson, MD, David Cheong, MD, and Xiaohui Niu, MD Investigation performed at the Department of Orthopaedic Oncology Surgery, Beijing Ji Shui Tan Hospital, Peking University, Beijing, People’s Republic of China, and the Department of Sarcoma, H. Lee Moffitt Cancer Center & Research Institute, Tampa, Florida, United States
Background: Chondroblastoma is a rare benign cartilage tumor that commonly occurs in children and adolescents. This study was designed to review the epidemiologic characteristics and outcomes of surgical management in a large series of patients with extremity chondroblastoma. Methods: We performed a multicenter retrospective analysis of 199 patients with extremity chondroblastoma. Clinical data, radiographic images, histological findings, treatment, and outcome were analyzed. Results: There were 145 male patients and fifty-four female patients with a mean age of 18.0 years. The most commonly involved bone was the proximal part of the tibia (fifty-five patients [27.6%]), followed by the proximal part of the femur (fifty-two patients [26.1%]) and the distal part of the femur (thirty-eight patients [19.1%]). Prior to presentation, 73.4% (146 of 199 patients) experienced pain. The mean duration of pain and other symptoms was 8.7 months. The physis was open in 25.7%, it was closing in 22.2%, and it was closed in 52.1% of the patients at the time of presentation. One hundred and twenty-six patients had at least twenty-four months of follow-up; their mean follow-up duration was 62.1 months (range, twenty-four to 190 months). Initial treatment was curettage for 119 patients (94.4%) and en bloc resection for seven patients (5.6%). The local recurrence rate was 5.0% after curettage and 0% after resection. The only significant factor related to recurrence was the location of the lesion in the proximal part of the humerus (p = 0.001). Conclusions: Chondroblastoma occurs most frequently in the proximal part of the tibia and the proximal part of the femur with significant male predilection. In this series, recurrence was most frequent in the proximal part of the humerus. Our results suggest that curettage and bone-grafting provide favorable local control and satisfactory functional outcome for patients with this disease. Level of Evidence: Therapeutic Level IV. See Instructions for Authors for a complete description of levels of evidence.
Peer Review: This article was reviewed by the Editor-in-Chief and one Deputy Editor, and it underwent blinded review by two or more outside experts. The Deputy Editor reviewed each revision of the article, and it underwent a final review by the Editor-in-Chief prior to publication. Final corrections and clarifications occurred during one or more exchanges between the author(s) and copyeditors.
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hondroblastoma was first recognized by Kolodny, who described it as a “giant cell variant” in 19271. Before the term “benign chondroblastoma” was coined by Jaffe and Lichtenstein2, “epiphyseal chondromatous giant cell tumor”3 and “calcifying giant cell tumor”4 were also used to describe this entity. As noted in some literature5-9, this tumor represents approximately 1% to 2% of all primary bone tu-
mors and 9% of all benign bone tumors. It is frequently discovered in adolescents with a mean age of fifteen years. Male patients are affected almost twice as frequently as female patients10-12. Clinical manifestations include pain in the majority of patients, local swelling, effusion, and limited motion of the adjacent joint13. Radiographically, chondroblastoma typically
Disclosure: None of the authors received payments or services, either directly or indirectly (i.e., via his or her institution), from a third party in support of any aspect of this work. None of the authors, or their institution(s), have had any financial relationship, in the thirty-six months prior to submission of this work, with any entity in the biomedical arena that could be perceived to influence or have the potential to influence what is written in this work. Also, no author has had any other relationships, or has engaged in any other activities, that could be perceived to influence or have the potential to influence what is written in this work. The complete Disclosures of Potential Conflicts of Interest submitted by authors are always provided with the online version of the article.
J Bone Joint Surg Am. 2015;97:925-31
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http://dx.doi.org/10.2106/JBJS.N.00992
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presents in the epiphysis of long bones of the extremities. Although the tumor may secondarily involve the metaphysis and even the diaphysis (probably by extension across the physis), primary metaphyseal or diaphyseal occurrences of this disease without epiphyseal or apophyseal involvement are exceptionally rare14. Surgical management is the mainstay of treatment for chondroblastomas that threaten the physis or joint surface. Meticulous curettage, alone or in combination with chemical or mechanical adjuvants, and bone-grafting or cementation are the gold standard of treatment13. However, resection is still an option for selected patients. There are also reports of the use of radiofrequency ablation15-17 or radiation, alone or in combination with surgery, for the treatment of chondroblastomas18,19. Reported local recurrence rates vary from 30%10,13,20,21. Malignant transformation of recurrent tumors and cases with benign pulmonary metastasis have been described in the literature, but are extremely rare22-25. Because of the rarity of chondroblastoma, the variation in treatments provided over time, and the smaller number of patients in other retrospective reports, it is difficult to draw conclusions about the clinical, radiographic, and treatment outcomes of this disease. We therefore performed a multicenter retrospective review of extremity chondroblastoma with a cohort of surgically treated patients. The objectives of this study were to describe the clinical, radiographic, and epidemiologic characteristics of chondroblastoma; to analyze the recurrences and complications associated with surgical treatment; and to report functional outcomes after treatments, which have been rarely documented in a large series20.
Patients underwent follow-up with clinical examination and radiographs at three-month intervals for the first year and then at six-month intervals until five years. For patients with more than twenty-four months of follow-up or early recurrence (less than twenty-four months), outcomes related to treatment were analyzed. Initial treatment included curettage or resection. Intralesional curettage was performed with a series of curets and with a high-speed burr. The operative field was copiously washed out with an irrigation gun for three to five minutes. Adjuvant application of phenol was used in 154 patients. In those 154 patients, the resultant cavity was filled with allograft, autogenous bone graft, bone substitute, cement, or a combination of these. En bloc resection was performed in seven patients whose defects would have been unreconstructible after curettage. Endoprostheses or segmental allografts were used for reconstruction after resection. The failure modes of prosthetic replacements in the six patients who received this management for reconstruction were classified with use of the system described 26 by Henderson et al. . Local recurrences and complications were recorded. Recurrence was confirmed by histological analysis in all patients. In patients with recurrent disease, clinical and pathologic features were analyzed to identify possible factors contributing to recurrence. Functional outcome was evaluated with the Musculoskeletal Tumor 27 Society (MSTS) scoring system when patients were nineteen years of age or older and with the Pediatric Outcomes Data Collection Instrument 28 (PODCI) questionnaire when patients were younger than nineteen years of age.
Materials and Methods
No external funding was received in support of this research.
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his was a multicenter retrospective study based on a review of patients’ medical records, imaging, and pathology. All patients who were identified with a chondroblastoma of an extremity, from 1980 to 2012 at the Beijing Ji Shui Tan Hospital (Beijing, People’s Republic of China), from 1993 to 2012 at the Moffitt Cancer Center (Tampa, Florida), and from 1993 to 2012 at the All Children’s Hospital (St. Petersburg, Florida), were reviewed. Of the 227 patients identified, 199 were included in the final analysis after excluding twenty-eight patients whose pathology was reviewed only for consultation. Pathology in all patients was independently reviewed by two pathologists (H.L.M. and Y.D.) experienced in musculoskeletal tumors. Medical records were reviewed for each patient. The age at the time of presentation and sex were identified. The clinical symptoms and the findings of the initial examination, including joint effusion, limitation of movement, flexion deformity, and muscle atrophy, were documented. The intervals between the first symptom and the time of diagnosis were also recorded. Imaging studies at the time of presentation, including radiographs, computed tomography (CT), and magnetic resonance imaging (MRI) (where available), were reviewed for each patient (see Appendix). The status of the 20 physis at the adjacent joint was categorized as open, closing, or closed . The location of each lesion was classified as epiphyseal, metaphyseal, or diaphyseal. The presence of physeal destruction was determined by review of radiographs, CT, and MRI. Mineralization and enhancement were identified on CT scans when that study was available. For patients with MRI scans, the presence of bone marrow edema and adjacent joint involvement were recorded.
Statistical Methods Statistical analysis was performed with use of SPSS software, version 20 (IBM, Armonk, New York). Continuous variables such as age and time of follow-up were expressed as the mean, the median, and the standard deviation. Categorical variables such as recurrence and complications were described as a percentage rate. One-way analysis of variance (ANOVA) was used to determine the differences in functional score. Chi-square analysis was performed to evaluate the difference in various percentages.
Source of Funding
Fig. 1
Age and sex distribution of extremity chondroblastoma (n = 199). The male: female ratio was 2.7:1. The mean patient age (and standard deviation) at the time of diagnosis was 18.02 ± 5.28 years.
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Results Epidemiologic Characteristics his series included 145 male patients and fifty-four female patients, a ratio of 2.7:1 (Fig. 1). The mean age at the time of diagnosis was 18.0 years (range, nine to thirty-eight years). The locations of the lesions were the lower limb (175 patients [87.9%]) and the upper limb (twenty-four patients [12.1%]). The predominant locations in the former were the femur (ninety patients [45.2%], with 57.8% of the lesions occurring proximally and 42.2% of the lesions occurring distally) and the tibia (fifty-seven patients [28.6%], with 96.5% of the lesions occurring proximally and 3.5% of the lesions occurring distally), and the predominant location in the latter was the humerus (twenty-four patients [12.1%], with 95.8% of the lesions occurring proximally and 4.2% of the lesions occurring distally), but other locations were also recorded (twenty-eight patients [14.1%]) (Fig. 2). Overall, the most commonly involved bone was the proximal part of the tibia (fifty-five patients [27.6%]), followed by the proximal part of the femur and the distal part of the femur.
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Clinical Features Pain was the primary presenting symptom in 73.4% (146 of 199 patients). The mean interval between the onset of symptoms and the time of diagnosis was 8.7 months (range, one to thirty-six months). The interval was one year or more in fiftyseven patients (28.6%) and two years or more in twenty patients (10.1%). At the time of diagnosis, sixty-five patients had a joint effusion, forty-five patients had limitation of movement, and twenty-six patients had a flexion deformity of the involved joint. Treatment and Recurrence One hundred and twenty-six patients had at least twenty-four months of follow-up postoperatively or developed early recurrence and were selected for analysis of treatment and outcome. The mean follow-up duration in this group was 62.1 months (range, twenty-four to 190 months). Among these, eighty-nine patients had at least three years of follow-up. Of them, fifty patients had at least five years of follow-up. This group included eighty-eight male patients and thirty-eight female patients with a mean age of 18.8 years (range, nine to thirty-five years). Tumors were located in the proximal part of the femur in thirty-eight patients (fifteen tumors in the femoral head and twenty-three tumors in the greater trochanter), followed by the proximal part of the tibia in twenty-eight patients, the distal part of the femur in twenty-six patients, the proximal part of the humerus in eleven patients, and other locations in twenty-three patients. Of 126 patients, 119 (94.4%) were treated with curettage. Of these 119 patients, the defects were filled with allograft alone in forty-nine patients (41.2%), with autograft alone in thirtyone patients (26.1%), with cement alone in fourteen patients (11.8%), with a mixture of bone graft materials (allograft, autograft, or bone substitute) in sixteen patients (13.4%), or with bone cement with allograft in nine patients (7.6%). Ad-
Fig. 2
Anatomical distribution of extremity chondroblastoma (n = 199). The most frequent location was the proximal part of the tibia, followed by the proximal part of the femur and the distal part of the femur.
juvant application of phenol was used in eighty-six patients. An extra-articular surgical approach was performed in eightyone patients (68.1%) and an intra-articular approach was performed in thirty-eight patients (31.9%). Fifteen patients with femoral head chondroblastomas were treated with use of an intra-articular approach. Fourteen of these patients underwent curettage and the remaining patient underwent tumor resection. En bloc tumor resection was performed in a total of seven (5.6%) of the 126 patients; of these seven patients, six had prosthetic replacement and one had an allograft joint replacement after tumor resection. The overall local recurrence rate was 4.8% (six of 126 patients). All six patients with a recurrence underwent
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TABLE I Local Recurrences Age
Sex
Location
Stage
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Male
Distal part of the femur
2
Open
Epiphysis, 4 mm to the subchondral bone and 3 mm to the physis
11
Male
Proximal part of the humerus
2
Open
Epiphysis with physis invasion, 2 mm to the subchondral bone
12
Male
Proximal part of the humerus
3
Open
Epiphysis with metaphyseal involvement, 4 mm to the subchondral bone
12
Female
Proximal part of the humerus
3
Open
Epiphysis, 3 mm to the subchondral bone and 4 mm to the physis
15
Female
Proximal part of the humerus
3
Open
Epiphyseal with metaphyseal involvement, 4 mm to the subchondral bone
14
Female
Proximal part of the humerus
3
Closed
Epiphyseal with metaphyseal involvement, 3 mm to the subchondral bone and 5 mm to the physis
curettage and allograft reconstruction as the initial operation. Therefore, the local recurrence rate was 5.0% after curettage and 0% after resection. Of these six patients, five had a tumor in the proximal part of the humerus and one had a tumor in the distal part of the femur. The intervals between the time of the first operation and the time of recurrence were five, seven, ten, thirteen, twenty-two, and twenty-four months, with a mean interval of 13.5 months. Four patients underwent a repeat curettage and two patients underwent tumor resection. One patient developed a second recurrence and was treated with radiofrequency ablation at another hospital six months after her second recurrence. She had a follow-up at three months after this procedure and was doing well with no evidence of persistent or recurrent disease. The details of these six patients are shown in Table I. Sex, age, staging, physeal status, presence or absence of pathologic fracture, and type of surgery were not found to have a significant effect (p ‡ 0.05) on local recurrence. The only significant difference between recurrent and nonrecurrent tumors was related to the location of the lesion (p = 0.001). The recurrence of proximal humeral lesions was 45% (five of eleven patients). Complications Eleven patients had complications postoperatively. Four of eleven complications were related to prosthetic replacement, including a Type-2 failure (aseptic loosening) in three patients (sixty-five, eighty-four, and ninety-three months after the initial prosthetic replacement) and a Type-4 failure (infection) in one patient (sixty-five months after the initial prosthetic replacement)26. All four patients underwent a revision operation. One patient with a distal femoral lesion developed heterotopic ossification in the popliteal area nine years after the tumor curettage. One patient with a lesion in the proximal part of the femur had failure of allograft incorporation at six months after tumor curettage and allograft filling, and another patient with a lesion in the proximal part of the humerus had
Physis
Location Details
wound dehiscence at twelve months after tumor curettage and allograft filling. After removal of the allograft material, the wounds healed. One patient with a femoral head lesion developed osteonecrosis of the femoral head after curettage and bone-grafting. A second patient, whose tumor was primarily located in the greater trochanter, also developed osteonecrosis of the femoral head after curettage and bone-grafting. Both of these patients received prosthetic replacements. One patient had knee osteoarthritis; another patient had a focal area of femoral head collapse after curettage, but was asymptomatic and the remainder of the femoral head appeared viable on follow-up imaging. Neither patient underwent any further surgeries. Functional Outcomes In the group of 126 patients, 111 patients were evaluated for functional outcomes. Ninety-seven patients were nineteen years of age or older at the time of evaluation and were assessed with use of the MSTS functional scoring system. Of the fourteen patients who were eighteen years of age or younger at the time of evaluation, clinical outcome was investigated with use of the PODCI questionnaire developed by the Pediatric Orthopaedic Society of North America (POSNA)28. The mean MSTS score was 29.2 points (range, 22 to 30 points) for the ninety-seven patients evaluated with this system. For the eighty-nine patients in this group who underwent curettage, the mean MSTS score was 29.4 points (range, 22 to 30 points). For the resection group (eight patients), the mean MSTS score was 27.9 points (range, 24 to 30 points). There was a significant difference between these two groups (p = 0.003). The mean global PODCI score was 98 points (range, 94 to 100 points). All fourteen patients attained a score of 100 points for the upper extremity, transfer and basic mobility, pain or comfort, and happiness. Their sports and physical functioning domain mean score was 93 points (range, 76 to 100 points). In this group, thirteen patients underwent curettage and grafting, and one patient underwent a prosthetic replacement.
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TABLE I (continued) Initial Treatment
Treatment After Recurrence
Curettage, freeze-dried bone allograft grains
Resection and prosthetic replacement
Prosthesis revision 93 months postoperatively due to aseptic loosening, PODCI score of 94 points
Curettage, freeze-dried bone allograft grains
Repeat curettage
110 months without recurrence, PODCI score of 100 points
Curettage, freeze-dried bone allograft grains
Resection and osteoarticular allograft, joint replacement
25 months without recurrence, PODCI not available
Curettage, freeze-dried bone allograft grains
Repeat curettage, burring, phenol, allograft, radiofrequency ablation (outside facility)
16 months without recurrence
Curettage, freeze-dried bone allograft grains
Repeat curettage, phenol, allograft
15 months without recurrence
Curettage, freeze-dried bone allograft grains
Repeat curettage with burr, allograft
22 months without recurrence
Discussion hondroblastoma is a rare primary benign cartilaginous bone tumor. To our knowledge, no prospective studies and only a few retrospective series have been reported9,10,12,13,20,21,29. This multicenter report describes the largest case series, to our knowledge, focused on epidemiologic characteristics, clinical features, radiographic features, treatment, and functional outcomes. Most chondroblastoma series have shown a male predominance, with a male:female ratio of 1.56 to 2:110,13,30,31. In our series, the male:female ratio was 2.7:1. The peak age of occurrence was between fifteen and twenty years of age, with almost half of all patients falling in this range. The mean age of 18.0 years is consistent with the reports of others10,12,20,30. In our cohort, the most commonly involved locations were the proximal part of the tibia, the proximal part of the femur, the distal part of the femur, and the proximal part of the humerus, which is consistent with most other published series10,13,30,32. The symptoms and related clinical features are summarized in other reports13,29,30. Our findings indicate that pain is the most common symptom, as is the case in most bone tumors. The interval between the onset of symptoms and diagnosis was as long as three years (mean, 8.7 months), as longterm symptoms may be misdiagnosed as growth pain, sports injury, or some other non-tumor-related disease during this interval33-36. Although radiofrequency ablation has been reported as one option for the treatment of chondroblastoma in the literature15-17,37, the mainstay of treatment remains surgery. Specifically, meticulous curettage of the lesion followed by bone-grafting is the gold standard13,21,38. In our series, 94.4% (119 of 126 patients) received this method of treatment, and only seven patients had tumor en bloc resections as the initial treatment for this benign osseous neoplasm, similar to other reports12,13,21. The authors of several series on chondroblastoma have reported local recurrence rates ranging from 8.3% to 32%10,13,20,21. The total local recurrence in this series was 4.8%
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with a mean follow-up of 62.1 months. This is one of the lowest reported in the literature. Many factors related to local recurrence have been postulated. The presence of an aneurysmal bone cyst component is one of the factors thought to be associated with local recurrence2,39, but this was not confirmed in other series10,20,21. Tumor location was identified as one possible factor related to recurrence in some reports. Ramappa et al.21 reported that tumor locations near the hip, including the proximal part of the femur and the pelvis, are at greatest risk for recurrence, but others13 have suggested that locations in the proximal part of the femur and tarsal bones were more likely. However, in the current series, we identified the proximal part of the humerus as the most likely location for local recurrence. Although age and status of the physis failed to be identified as factors related to recurrence, all patients whose tumors recurred were fifteen years of age or younger and five had open physes. In a report of chondroblastomas in a pediatric population13, recurrence was as high as 32%. In other literature5,6,21, the presence of an open physis was considered to be a risk factor for local recurrence. All these might indicate that the presentation of chondroblastoma in skeletally immature patients is more likely to be associated with recurrence than in those with closed physes. Local recurrence generally implies residual tumor after initial removal by whatever method was used. Locations that are hard to access or close to the physis may lead to incomplete curettage and residual disease, thus increasing the risk of local recurrence. It is generally recognized that the most prognostic factor contributing to local recurrence is the surgical technique chosen to be used. Therefore, we believe that the first step in obtaining a successful outcome is careful preoperative planning, which includes appropriate access to perform a meticulous curettage of the entire tumor. Benign pulmonary metastasis of chondroblastoma is extremely rare10,21. Ozkoc et al. found fourteen cases in the English literature24. We did not identify any benign metastases in this series. Similarly, chondroblastoma undergoing malignant transformation with metastasis to the lung occurs rarely in
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recurrent disease10,21. No malignant transformation occurred in our series. The present study had a complication rate of 8.7% (eleven of 126 patients). Patients with tumors located in the proximal part of the femur, including the femoral head and greater trochanter, should be informed of the potential complication of osteonecrosis. The surgeon should be wary of the blood supply to the femoral head during the approach and curettage of these lesions. Although we demonstrated that the overall functional outcome after surgery was satisfactory, the patients treated with tumor resection had a higher complication rate. Because of the benign nature of chondroblastoma, the implications of resection with prosthetic replacement should be seriously considered before it is performed, even for patients with recurrent disease. Our study had at least three limitations. First, it was retrospective and had insufficient power to fully assess the outcomes of chondroblastoma treated surgically. Second, treatment other than surgery was not evaluated in this series. The benefits and disadvantages of nonsurgical treatment are still unknown. Third, not all of the patients had adequate follow-up to be included in our report when this study was performed. We demonstrate in this multicenter series that meticulous curettage and bone-grafting produce favorable local control and satisfactory functional outcomes in the treatment of chondroblastoma of the extremities. Appendix Radiographic Features adiographically, of the 171 patients with chondroblastomas in the long bones (femur, tibia, and humerus), 157 had tumors isolated to the epiphysis. The metaphysis was involved in fourteen patients. No lesion was exclusively located in the metaphysis or diaphysis. A review of radiographs and/or CT scans revealed that the physis was open in forty-four patients (25.7%), it was closing in thirty-eight patients (22.2%), and it was closed in eighty-nine patients (52.1%) at the time of diagnosis. A CT scan was available for review for 186 patients of the total group of 199 patients. The tumors demonstrated intralesional mineralization in eighty-three (44.6%) of these patients, but showed cortical destruction in thirty-six patients (19.4%) and revealed invasion of the physis in thirty-nine
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patients (21.0%). A CT scan with contrast was available for 106 patients. Ninety-one patients (85.8%) had lesions that demonstrated tumor enhancement. Of 112 patients with available MRI scans, seventy-two (64.3%) showed bone marrow edema, and thirty-one (27.7%) had edema of the adjacent joint. The aggressiveness of the lesion was classified, according to Enneking40, as latent in the tumors of seven patients (3.5%), active in the tumors of seventy-nine patients (39.7%), and aggressive in the tumors of 113 patients (56.8%). Of the 199 patients, thirteen (6.5%) presented with a pathologic fracture at the time of diagnosis. n NOTE: The authors thank Yang Sun, MD, Weifeng Liu, MD, Yongkun Yang, MD, Tao Jin, MD, Le Li, MD candidate, Bin Li, MD candidate, He Xiao, MD candidate, and Ming Gao, MD candidate, for their contributions to the data collection of this series.
Hairong Xu, MD Yi Ding, MD Xiaohui Niu, MD Departments of Orthopaedic Oncology Surgery (H.X. and X.N.) and Pathology (Y.D.), Beijing Ji Shui Tan Hospital, Peking University, 31 Xinjiekou East Street, Xicheng District, Beijing 100035, People’s Republic of China. E-mail address for X. Niu: [email protected] Dylan Nugent, MD Department of Orthopaedics and Rehabilitation, University of Florida College of Medicine, 3450 Hull Road, Gainesville, FL 32607 Hector L. Monforte, MD Department of Pathology, All Children’s Hospital, 501 6th Avenue South, St. Petersburg, FL 33701 Odion T. Binitie, MD G. Douglas Letson, MD David Cheong, MD Department of Sarcoma, H. Lee Moffitt Cancer Center & Research Institute, 12902 Magnolia Drive, Tampa, FL 33612
References 1. Kolodny A. Bone sarcoma: the primary malignant tumors of bone and the giant cell tumor. Surg Gynecol Obstet. 1927;44(Suppl 1):1-214. 2. Jaffe HL, Lichtenstein L. Benign chondroblastoma of bone: a reinterpretation of the so-called calcifying or chondromatous giant cell tumor. Am J Pathol. 1942 Nov;18(6):969-91. 3. Codman EA. Epiphyseal chondromatous giant cell tumors of the upper end of the humerus. Surg Gynecol Obstet. 1931;52:543-8. 4. Ewing J. The classification and treatment of bone sarcoma. Report of the International Conference on Cancer, London. Bristol: John Wright & Sons; 1928. p 365-76. 5. Dahlin DC, Ivins JC. Benign chondroblastoma. A study of 125 cases. Cancer. 1972 Aug;30(2):401-13. 6. Springfield DS, Capanna R, Gherlinzoni F, Picci P, Campanacci M. Chondroblastoma. A review of seventy cases. J Bone Joint Surg Am. 1985 Jun;67(5):748-55.
7. van der Eijken JW. Strategy in the treatment of benign bone tumors: an overview. J Pediatr Orthop B. 1998 Oct;7(4):249-52. 8. Campanacci M. Bone and soft tissue tumors: clinical features, imaging, pathology, and treatment. 2nd ed. New York: Springer; 1999. Chondroblastoma; p 247-64. 9. Strong DP, Grimer RJ, Carter SR, Tillman RM, Abudu A. Chondroblastoma of the femoral head: management and outcome. Int Orthop. 2010 Mar;34(3):413-7. Epub 2009 Apr 24. 10. Lin PP, Thenappan A, Deavers MT, Lewis VO, Yasko AW. Treatment and prognosis of chondroblastoma. Clin Orthop Relat Res. 2005 Sep;438:103-9. 11. de Silva MVC, Reid R. Chondroblastoma: varied histologic appearance, potential diagnostic pitfalls, and clinicopathologic features associated with local recurrence. Ann Diagn Pathol. 2003 Aug;7(4):205-13.
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12. Lehner B, Witte D, Weiss S. Clinical and radiological long-term results after operative treatment of chondroblastoma. Arch Orthop Trauma Surg. 2011 Jan;131 (1):45-52. Epub 2010 Apr 3. 13. Sailhan F, Chotel F, Parot R. SOFOP. Chondroblastoma of bone in a pediatric population. J Bone Joint Surg Am. 2009 Sep;91(9):2159-68. 14. Maheshwari AV, Jelinek JS, Song AJ, Nelson KJ, Murphey MD, Henshaw RM. Metaphyseal and diaphyseal chondroblastomas. Skeletal Radiol. 2011 Dec;40 (12):1563-73. Epub 2011 Jul 20. 15. Christie-Large M, Evans N, Davies AM, James SLJ. Radiofrequency ablation of chondroblastoma: procedure technique, clinical and MR imaging follow up of four cases. Skeletal Radiol. 2008 Nov;37(11):1011-7. Epub 2008 Jul 19. 16. Petsas T, Megas P, Papathanassiou Z. Radiofrequency ablation of two femoral head chondroblastomas. Eur J Radiol. 2007 Jul;63(1):63-7. Epub 2007 May 4. 17. Tins B, Cassar-Pullicino V, McCall I, Cool P, Williams D, Mangham D. Radiofrequency ablation of chondroblastoma using a multi-tined expandable electrode system: initial results. Eur Radiol. 2006 Apr;16(4):804-10. Epub 2005 Nov 3. 18. Harish K, Janaki MG, Alva NK. “Primary” aggressive chondroblastoma of the humerus: a case report. BMC Musculoskelet Disord. 2004 Mar 17;5:9. 19. Wirman JA, Crissman JD, Aron BF. Metastatic chondroblastoma: report of an unusual case treated with radiotherapy. Cancer. 1979 Jul;44(1):87-93. 20. Suneja R, Grimer RJ, Belthur M, Jeys L, Carter SR, Tillman RM, Davies AM. Chondroblastoma of bone: long-term results and functional outcome after intralesional curettage. J Bone Joint Surg Br. 2005 Jul;87(7):974-8. 21. Ramappa AJ, Lee FY, Tang P, Carlson JR, Gebhardt MC, Mankin HJ. Chondroblastoma of bone. J Bone Joint Surg Am. 2000 Aug;82(8):1140-5. 22. Tamura M, Oda M, Matsumoto I, Sawada-Kitamura S, Watanabe G. Chondroblastoma with pulmonary metastasis in a patient presenting with spontaneous bilateral pneumothorax: report of a case. Surg Today. 2011 Oct;41(10):1439-41. Epub 2011 Sep 16. 23. Ostrowski ML, Johnson ME, Truong LD, Hicks MJ, Smith FE, Spjut HJ. Malignant chondroblastoma presenting as a recurrent pelvic tumor with DNA aneuploidy and p53 mutation as supportive evidence of malignancy. Skeletal Radiol. 1999 Nov;28 (11):644-50. 24. Ozkoc G, Gonlusen G, Ozalay M, Kayaselcuk F, Pourbagher A, Tandogan RN. Giant chondroblastoma of the scapula with pulmonary metastases. Skeletal Radiol. 2006 Jan;35(1):42-8. Epub 2005 Jul 9. 25. McLaughlin RE, Sweet DE, Webster T, Merritt WM. Chondroblastoma of the pelvis suggestive of malignancy. J Bone Joint Surg Am. 1975 Jun;57(4):549-51.
26. Henderson ER, Groundland JS, Pala E, Dennis JA, Wooten R, Cheong D, Windhager R, Kotz RI, Mercuri M, Funovics PT, Hornicek FJ, Temple HT, Ruggieri P, Letson GD. Failure mode classification for tumor endoprostheses: retrospective review of five institutions and a literature review. J Bone Joint Surg Am. 2011 Mar 2;93(5):418-29. 27. Enneking WF, Dunham W, Gebhardt MC, Malawar M, Pritchard DJ. A system for the functional evaluation of reconstructive procedures after surgical treatment of tumors of the musculoskeletal system. Clin Orthop Relat Res. 1993 Jan;286: 241-6. 28. Haynes RJ, Sullivan E. The Pediatric Orthopaedic Society of North America pediatric orthopaedic functional health questionnaire: an analysis of normals. J Pediatr Orthop. 2001 Sep-Oct;21(5):619-21. 29. Hsu CC, Wang JW, Chen CE, Lin JW. Results of curettage and high-speed burring for chondroblastoma of the bone. Chang Gung Med J. 2003 Oct;26(10):761-7. 30. Turcotte RE, Kurt AM, Sim FH, Unni KK, McLeod RA. Chondroblastoma. Hum Pathol. 1993 Sep;24(9):944-9. 31. Kurt AM, Unni KK, Sim FH, McLeod RA. Chondroblastoma of bone. Hum Pathol. 1989 Oct;20(10):965-76. 32. Bloem JL, Mulder JD. Chondroblastoma: a clinical and radiological study of 104 cases. Skeletal Radiol. 1985;14(1):1-9. 33. Wolfe MW, Halvorson TL, Bennett JT, Martin PC. Chondroblastoma of the patella presenting as knee pain in an adolescent. Am J Orthop. 1995 Jan;24(1):61-4. 34. Endo H, Kawai A, Naito N, Sugihara S, Inoue H. Knee pain in a 16-year-old girl. Clin Orthop Relat Res. 2001 Dec;393:345-9. 35. Diwanji SR, Cho SG, Kong IK, Yoon TR. Hip pain in a 24-year-old woman. Clin Orthop Relat Res. 2007 Aug;461:262-8. 36. Chatha DS, Rybak LD, Wittig JC, Desai P. Painful tibial lesion in a 16-year-old girl. Clin Orthop Relat Res. 2007 Nov;464:247-52. 37. Rajalakshmi P, Srivastava DN, Rastogi S, Julka PK, Bhatnagar S, Gamanagatti S. Bipolar radiofrequency ablation of tibialchondroblastomas: a report of three cases. World J Radiol. 2012 Jul 28;4(7):335-40. 38. Atalar H, Basarir K, Yildiz Y, Erekul S, Saglik Y. Management of chondroblastoma: retrospective review of 28 patients. J Orthop Sci. 2007 Jul;12(4):334-40. Epub 2007 Aug 2. 39. Huvos AG, Marcove RC. Chondroblastoma of bone. A critical review. Clin Orthop Relat Res. 1973 Sep;95:300-12. 40. Enneking WF. A system of staging musculoskeletal neoplasms. Clin Orthop Relat Res. 1986 Mar;204:9-24.
BY
T HE J OURNAL
OF
B ONE
AND J OINT
S URGERY, I NCORPORATED
Chondroblastoma of Bone in the Extremities A Multicenter Retrospective Study Hairong Xu, MD, Dylan Nugent, MD, Hector L. Monforte, MD, Odion T. Binitie, MD, Yi Ding, MD, G. Douglas Letson, MD, David Cheong, MD, and Xiaohui Niu, MD Investigation performed at the Department of Orthopaedic Oncology Surgery, Beijing Ji Shui Tan Hospital, Peking University, Beijing, People’s Republic of China, and the Department of Sarcoma, H. Lee Moffitt Cancer Center & Research Institute, Tampa, Florida, United States
Background: Chondroblastoma is a rare benign cartilage tumor that commonly occurs in children and adolescents. This study was designed to review the epidemiologic characteristics and outcomes of surgical management in a large series of patients with extremity chondroblastoma. Methods: We performed a multicenter retrospective analysis of 199 patients with extremity chondroblastoma. Clinical data, radiographic images, histological findings, treatment, and outcome were analyzed. Results: There were 145 male patients and fifty-four female patients with a mean age of 18.0 years. The most commonly involved bone was the proximal part of the tibia (fifty-five patients [27.6%]), followed by the proximal part of the femur (fifty-two patients [26.1%]) and the distal part of the femur (thirty-eight patients [19.1%]). Prior to presentation, 73.4% (146 of 199 patients) experienced pain. The mean duration of pain and other symptoms was 8.7 months. The physis was open in 25.7%, it was closing in 22.2%, and it was closed in 52.1% of the patients at the time of presentation. One hundred and twenty-six patients had at least twenty-four months of follow-up; their mean follow-up duration was 62.1 months (range, twenty-four to 190 months). Initial treatment was curettage for 119 patients (94.4%) and en bloc resection for seven patients (5.6%). The local recurrence rate was 5.0% after curettage and 0% after resection. The only significant factor related to recurrence was the location of the lesion in the proximal part of the humerus (p = 0.001). Conclusions: Chondroblastoma occurs most frequently in the proximal part of the tibia and the proximal part of the femur with significant male predilection. In this series, recurrence was most frequent in the proximal part of the humerus. Our results suggest that curettage and bone-grafting provide favorable local control and satisfactory functional outcome for patients with this disease. Level of Evidence: Therapeutic Level IV. See Instructions for Authors for a complete description of levels of evidence.
Peer Review: This article was reviewed by the Editor-in-Chief and one Deputy Editor, and it underwent blinded review by two or more outside experts. The Deputy Editor reviewed each revision of the article, and it underwent a final review by the Editor-in-Chief prior to publication. Final corrections and clarifications occurred during one or more exchanges between the author(s) and copyeditors.
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hondroblastoma was first recognized by Kolodny, who described it as a “giant cell variant” in 19271. Before the term “benign chondroblastoma” was coined by Jaffe and Lichtenstein2, “epiphyseal chondromatous giant cell tumor”3 and “calcifying giant cell tumor”4 were also used to describe this entity. As noted in some literature5-9, this tumor represents approximately 1% to 2% of all primary bone tu-
mors and 9% of all benign bone tumors. It is frequently discovered in adolescents with a mean age of fifteen years. Male patients are affected almost twice as frequently as female patients10-12. Clinical manifestations include pain in the majority of patients, local swelling, effusion, and limited motion of the adjacent joint13. Radiographically, chondroblastoma typically
Disclosure: None of the authors received payments or services, either directly or indirectly (i.e., via his or her institution), from a third party in support of any aspect of this work. None of the authors, or their institution(s), have had any financial relationship, in the thirty-six months prior to submission of this work, with any entity in the biomedical arena that could be perceived to influence or have the potential to influence what is written in this work. Also, no author has had any other relationships, or has engaged in any other activities, that could be perceived to influence or have the potential to influence what is written in this work. The complete Disclosures of Potential Conflicts of Interest submitted by authors are always provided with the online version of the article.
J Bone Joint Surg Am. 2015;97:925-31
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http://dx.doi.org/10.2106/JBJS.N.00992
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presents in the epiphysis of long bones of the extremities. Although the tumor may secondarily involve the metaphysis and even the diaphysis (probably by extension across the physis), primary metaphyseal or diaphyseal occurrences of this disease without epiphyseal or apophyseal involvement are exceptionally rare14. Surgical management is the mainstay of treatment for chondroblastomas that threaten the physis or joint surface. Meticulous curettage, alone or in combination with chemical or mechanical adjuvants, and bone-grafting or cementation are the gold standard of treatment13. However, resection is still an option for selected patients. There are also reports of the use of radiofrequency ablation15-17 or radiation, alone or in combination with surgery, for the treatment of chondroblastomas18,19. Reported local recurrence rates vary from 30%10,13,20,21. Malignant transformation of recurrent tumors and cases with benign pulmonary metastasis have been described in the literature, but are extremely rare22-25. Because of the rarity of chondroblastoma, the variation in treatments provided over time, and the smaller number of patients in other retrospective reports, it is difficult to draw conclusions about the clinical, radiographic, and treatment outcomes of this disease. We therefore performed a multicenter retrospective review of extremity chondroblastoma with a cohort of surgically treated patients. The objectives of this study were to describe the clinical, radiographic, and epidemiologic characteristics of chondroblastoma; to analyze the recurrences and complications associated with surgical treatment; and to report functional outcomes after treatments, which have been rarely documented in a large series20.
Patients underwent follow-up with clinical examination and radiographs at three-month intervals for the first year and then at six-month intervals until five years. For patients with more than twenty-four months of follow-up or early recurrence (less than twenty-four months), outcomes related to treatment were analyzed. Initial treatment included curettage or resection. Intralesional curettage was performed with a series of curets and with a high-speed burr. The operative field was copiously washed out with an irrigation gun for three to five minutes. Adjuvant application of phenol was used in 154 patients. In those 154 patients, the resultant cavity was filled with allograft, autogenous bone graft, bone substitute, cement, or a combination of these. En bloc resection was performed in seven patients whose defects would have been unreconstructible after curettage. Endoprostheses or segmental allografts were used for reconstruction after resection. The failure modes of prosthetic replacements in the six patients who received this management for reconstruction were classified with use of the system described 26 by Henderson et al. . Local recurrences and complications were recorded. Recurrence was confirmed by histological analysis in all patients. In patients with recurrent disease, clinical and pathologic features were analyzed to identify possible factors contributing to recurrence. Functional outcome was evaluated with the Musculoskeletal Tumor 27 Society (MSTS) scoring system when patients were nineteen years of age or older and with the Pediatric Outcomes Data Collection Instrument 28 (PODCI) questionnaire when patients were younger than nineteen years of age.
Materials and Methods
No external funding was received in support of this research.
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his was a multicenter retrospective study based on a review of patients’ medical records, imaging, and pathology. All patients who were identified with a chondroblastoma of an extremity, from 1980 to 2012 at the Beijing Ji Shui Tan Hospital (Beijing, People’s Republic of China), from 1993 to 2012 at the Moffitt Cancer Center (Tampa, Florida), and from 1993 to 2012 at the All Children’s Hospital (St. Petersburg, Florida), were reviewed. Of the 227 patients identified, 199 were included in the final analysis after excluding twenty-eight patients whose pathology was reviewed only for consultation. Pathology in all patients was independently reviewed by two pathologists (H.L.M. and Y.D.) experienced in musculoskeletal tumors. Medical records were reviewed for each patient. The age at the time of presentation and sex were identified. The clinical symptoms and the findings of the initial examination, including joint effusion, limitation of movement, flexion deformity, and muscle atrophy, were documented. The intervals between the first symptom and the time of diagnosis were also recorded. Imaging studies at the time of presentation, including radiographs, computed tomography (CT), and magnetic resonance imaging (MRI) (where available), were reviewed for each patient (see Appendix). The status of the 20 physis at the adjacent joint was categorized as open, closing, or closed . The location of each lesion was classified as epiphyseal, metaphyseal, or diaphyseal. The presence of physeal destruction was determined by review of radiographs, CT, and MRI. Mineralization and enhancement were identified on CT scans when that study was available. For patients with MRI scans, the presence of bone marrow edema and adjacent joint involvement were recorded.
Statistical Methods Statistical analysis was performed with use of SPSS software, version 20 (IBM, Armonk, New York). Continuous variables such as age and time of follow-up were expressed as the mean, the median, and the standard deviation. Categorical variables such as recurrence and complications were described as a percentage rate. One-way analysis of variance (ANOVA) was used to determine the differences in functional score. Chi-square analysis was performed to evaluate the difference in various percentages.
Source of Funding
Fig. 1
Age and sex distribution of extremity chondroblastoma (n = 199). The male: female ratio was 2.7:1. The mean patient age (and standard deviation) at the time of diagnosis was 18.02 ± 5.28 years.
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Results Epidemiologic Characteristics his series included 145 male patients and fifty-four female patients, a ratio of 2.7:1 (Fig. 1). The mean age at the time of diagnosis was 18.0 years (range, nine to thirty-eight years). The locations of the lesions were the lower limb (175 patients [87.9%]) and the upper limb (twenty-four patients [12.1%]). The predominant locations in the former were the femur (ninety patients [45.2%], with 57.8% of the lesions occurring proximally and 42.2% of the lesions occurring distally) and the tibia (fifty-seven patients [28.6%], with 96.5% of the lesions occurring proximally and 3.5% of the lesions occurring distally), and the predominant location in the latter was the humerus (twenty-four patients [12.1%], with 95.8% of the lesions occurring proximally and 4.2% of the lesions occurring distally), but other locations were also recorded (twenty-eight patients [14.1%]) (Fig. 2). Overall, the most commonly involved bone was the proximal part of the tibia (fifty-five patients [27.6%]), followed by the proximal part of the femur and the distal part of the femur.
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Clinical Features Pain was the primary presenting symptom in 73.4% (146 of 199 patients). The mean interval between the onset of symptoms and the time of diagnosis was 8.7 months (range, one to thirty-six months). The interval was one year or more in fiftyseven patients (28.6%) and two years or more in twenty patients (10.1%). At the time of diagnosis, sixty-five patients had a joint effusion, forty-five patients had limitation of movement, and twenty-six patients had a flexion deformity of the involved joint. Treatment and Recurrence One hundred and twenty-six patients had at least twenty-four months of follow-up postoperatively or developed early recurrence and were selected for analysis of treatment and outcome. The mean follow-up duration in this group was 62.1 months (range, twenty-four to 190 months). Among these, eighty-nine patients had at least three years of follow-up. Of them, fifty patients had at least five years of follow-up. This group included eighty-eight male patients and thirty-eight female patients with a mean age of 18.8 years (range, nine to thirty-five years). Tumors were located in the proximal part of the femur in thirty-eight patients (fifteen tumors in the femoral head and twenty-three tumors in the greater trochanter), followed by the proximal part of the tibia in twenty-eight patients, the distal part of the femur in twenty-six patients, the proximal part of the humerus in eleven patients, and other locations in twenty-three patients. Of 126 patients, 119 (94.4%) were treated with curettage. Of these 119 patients, the defects were filled with allograft alone in forty-nine patients (41.2%), with autograft alone in thirtyone patients (26.1%), with cement alone in fourteen patients (11.8%), with a mixture of bone graft materials (allograft, autograft, or bone substitute) in sixteen patients (13.4%), or with bone cement with allograft in nine patients (7.6%). Ad-
Fig. 2
Anatomical distribution of extremity chondroblastoma (n = 199). The most frequent location was the proximal part of the tibia, followed by the proximal part of the femur and the distal part of the femur.
juvant application of phenol was used in eighty-six patients. An extra-articular surgical approach was performed in eightyone patients (68.1%) and an intra-articular approach was performed in thirty-eight patients (31.9%). Fifteen patients with femoral head chondroblastomas were treated with use of an intra-articular approach. Fourteen of these patients underwent curettage and the remaining patient underwent tumor resection. En bloc tumor resection was performed in a total of seven (5.6%) of the 126 patients; of these seven patients, six had prosthetic replacement and one had an allograft joint replacement after tumor resection. The overall local recurrence rate was 4.8% (six of 126 patients). All six patients with a recurrence underwent
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ä
TABLE I Local Recurrences Age
Sex
Location
Stage
13
Male
Distal part of the femur
2
Open
Epiphysis, 4 mm to the subchondral bone and 3 mm to the physis
11
Male
Proximal part of the humerus
2
Open
Epiphysis with physis invasion, 2 mm to the subchondral bone
12
Male
Proximal part of the humerus
3
Open
Epiphysis with metaphyseal involvement, 4 mm to the subchondral bone
12
Female
Proximal part of the humerus
3
Open
Epiphysis, 3 mm to the subchondral bone and 4 mm to the physis
15
Female
Proximal part of the humerus
3
Open
Epiphyseal with metaphyseal involvement, 4 mm to the subchondral bone
14
Female
Proximal part of the humerus
3
Closed
Epiphyseal with metaphyseal involvement, 3 mm to the subchondral bone and 5 mm to the physis
curettage and allograft reconstruction as the initial operation. Therefore, the local recurrence rate was 5.0% after curettage and 0% after resection. Of these six patients, five had a tumor in the proximal part of the humerus and one had a tumor in the distal part of the femur. The intervals between the time of the first operation and the time of recurrence were five, seven, ten, thirteen, twenty-two, and twenty-four months, with a mean interval of 13.5 months. Four patients underwent a repeat curettage and two patients underwent tumor resection. One patient developed a second recurrence and was treated with radiofrequency ablation at another hospital six months after her second recurrence. She had a follow-up at three months after this procedure and was doing well with no evidence of persistent or recurrent disease. The details of these six patients are shown in Table I. Sex, age, staging, physeal status, presence or absence of pathologic fracture, and type of surgery were not found to have a significant effect (p ‡ 0.05) on local recurrence. The only significant difference between recurrent and nonrecurrent tumors was related to the location of the lesion (p = 0.001). The recurrence of proximal humeral lesions was 45% (five of eleven patients). Complications Eleven patients had complications postoperatively. Four of eleven complications were related to prosthetic replacement, including a Type-2 failure (aseptic loosening) in three patients (sixty-five, eighty-four, and ninety-three months after the initial prosthetic replacement) and a Type-4 failure (infection) in one patient (sixty-five months after the initial prosthetic replacement)26. All four patients underwent a revision operation. One patient with a distal femoral lesion developed heterotopic ossification in the popliteal area nine years after the tumor curettage. One patient with a lesion in the proximal part of the femur had failure of allograft incorporation at six months after tumor curettage and allograft filling, and another patient with a lesion in the proximal part of the humerus had
Physis
Location Details
wound dehiscence at twelve months after tumor curettage and allograft filling. After removal of the allograft material, the wounds healed. One patient with a femoral head lesion developed osteonecrosis of the femoral head after curettage and bone-grafting. A second patient, whose tumor was primarily located in the greater trochanter, also developed osteonecrosis of the femoral head after curettage and bone-grafting. Both of these patients received prosthetic replacements. One patient had knee osteoarthritis; another patient had a focal area of femoral head collapse after curettage, but was asymptomatic and the remainder of the femoral head appeared viable on follow-up imaging. Neither patient underwent any further surgeries. Functional Outcomes In the group of 126 patients, 111 patients were evaluated for functional outcomes. Ninety-seven patients were nineteen years of age or older at the time of evaluation and were assessed with use of the MSTS functional scoring system. Of the fourteen patients who were eighteen years of age or younger at the time of evaluation, clinical outcome was investigated with use of the PODCI questionnaire developed by the Pediatric Orthopaedic Society of North America (POSNA)28. The mean MSTS score was 29.2 points (range, 22 to 30 points) for the ninety-seven patients evaluated with this system. For the eighty-nine patients in this group who underwent curettage, the mean MSTS score was 29.4 points (range, 22 to 30 points). For the resection group (eight patients), the mean MSTS score was 27.9 points (range, 24 to 30 points). There was a significant difference between these two groups (p = 0.003). The mean global PODCI score was 98 points (range, 94 to 100 points). All fourteen patients attained a score of 100 points for the upper extremity, transfer and basic mobility, pain or comfort, and happiness. Their sports and physical functioning domain mean score was 93 points (range, 76 to 100 points). In this group, thirteen patients underwent curettage and grafting, and one patient underwent a prosthetic replacement.
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TABLE I (continued) Initial Treatment
Treatment After Recurrence
Curettage, freeze-dried bone allograft grains
Resection and prosthetic replacement
Prosthesis revision 93 months postoperatively due to aseptic loosening, PODCI score of 94 points
Curettage, freeze-dried bone allograft grains
Repeat curettage
110 months without recurrence, PODCI score of 100 points
Curettage, freeze-dried bone allograft grains
Resection and osteoarticular allograft, joint replacement
25 months without recurrence, PODCI not available
Curettage, freeze-dried bone allograft grains
Repeat curettage, burring, phenol, allograft, radiofrequency ablation (outside facility)
16 months without recurrence
Curettage, freeze-dried bone allograft grains
Repeat curettage, phenol, allograft
15 months without recurrence
Curettage, freeze-dried bone allograft grains
Repeat curettage with burr, allograft
22 months without recurrence
Discussion hondroblastoma is a rare primary benign cartilaginous bone tumor. To our knowledge, no prospective studies and only a few retrospective series have been reported9,10,12,13,20,21,29. This multicenter report describes the largest case series, to our knowledge, focused on epidemiologic characteristics, clinical features, radiographic features, treatment, and functional outcomes. Most chondroblastoma series have shown a male predominance, with a male:female ratio of 1.56 to 2:110,13,30,31. In our series, the male:female ratio was 2.7:1. The peak age of occurrence was between fifteen and twenty years of age, with almost half of all patients falling in this range. The mean age of 18.0 years is consistent with the reports of others10,12,20,30. In our cohort, the most commonly involved locations were the proximal part of the tibia, the proximal part of the femur, the distal part of the femur, and the proximal part of the humerus, which is consistent with most other published series10,13,30,32. The symptoms and related clinical features are summarized in other reports13,29,30. Our findings indicate that pain is the most common symptom, as is the case in most bone tumors. The interval between the onset of symptoms and diagnosis was as long as three years (mean, 8.7 months), as longterm symptoms may be misdiagnosed as growth pain, sports injury, or some other non-tumor-related disease during this interval33-36. Although radiofrequency ablation has been reported as one option for the treatment of chondroblastoma in the literature15-17,37, the mainstay of treatment remains surgery. Specifically, meticulous curettage of the lesion followed by bone-grafting is the gold standard13,21,38. In our series, 94.4% (119 of 126 patients) received this method of treatment, and only seven patients had tumor en bloc resections as the initial treatment for this benign osseous neoplasm, similar to other reports12,13,21. The authors of several series on chondroblastoma have reported local recurrence rates ranging from 8.3% to 32%10,13,20,21. The total local recurrence in this series was 4.8%
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Outcome
with a mean follow-up of 62.1 months. This is one of the lowest reported in the literature. Many factors related to local recurrence have been postulated. The presence of an aneurysmal bone cyst component is one of the factors thought to be associated with local recurrence2,39, but this was not confirmed in other series10,20,21. Tumor location was identified as one possible factor related to recurrence in some reports. Ramappa et al.21 reported that tumor locations near the hip, including the proximal part of the femur and the pelvis, are at greatest risk for recurrence, but others13 have suggested that locations in the proximal part of the femur and tarsal bones were more likely. However, in the current series, we identified the proximal part of the humerus as the most likely location for local recurrence. Although age and status of the physis failed to be identified as factors related to recurrence, all patients whose tumors recurred were fifteen years of age or younger and five had open physes. In a report of chondroblastomas in a pediatric population13, recurrence was as high as 32%. In other literature5,6,21, the presence of an open physis was considered to be a risk factor for local recurrence. All these might indicate that the presentation of chondroblastoma in skeletally immature patients is more likely to be associated with recurrence than in those with closed physes. Local recurrence generally implies residual tumor after initial removal by whatever method was used. Locations that are hard to access or close to the physis may lead to incomplete curettage and residual disease, thus increasing the risk of local recurrence. It is generally recognized that the most prognostic factor contributing to local recurrence is the surgical technique chosen to be used. Therefore, we believe that the first step in obtaining a successful outcome is careful preoperative planning, which includes appropriate access to perform a meticulous curettage of the entire tumor. Benign pulmonary metastasis of chondroblastoma is extremely rare10,21. Ozkoc et al. found fourteen cases in the English literature24. We did not identify any benign metastases in this series. Similarly, chondroblastoma undergoing malignant transformation with metastasis to the lung occurs rarely in
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recurrent disease10,21. No malignant transformation occurred in our series. The present study had a complication rate of 8.7% (eleven of 126 patients). Patients with tumors located in the proximal part of the femur, including the femoral head and greater trochanter, should be informed of the potential complication of osteonecrosis. The surgeon should be wary of the blood supply to the femoral head during the approach and curettage of these lesions. Although we demonstrated that the overall functional outcome after surgery was satisfactory, the patients treated with tumor resection had a higher complication rate. Because of the benign nature of chondroblastoma, the implications of resection with prosthetic replacement should be seriously considered before it is performed, even for patients with recurrent disease. Our study had at least three limitations. First, it was retrospective and had insufficient power to fully assess the outcomes of chondroblastoma treated surgically. Second, treatment other than surgery was not evaluated in this series. The benefits and disadvantages of nonsurgical treatment are still unknown. Third, not all of the patients had adequate follow-up to be included in our report when this study was performed. We demonstrate in this multicenter series that meticulous curettage and bone-grafting produce favorable local control and satisfactory functional outcomes in the treatment of chondroblastoma of the extremities. Appendix Radiographic Features adiographically, of the 171 patients with chondroblastomas in the long bones (femur, tibia, and humerus), 157 had tumors isolated to the epiphysis. The metaphysis was involved in fourteen patients. No lesion was exclusively located in the metaphysis or diaphysis. A review of radiographs and/or CT scans revealed that the physis was open in forty-four patients (25.7%), it was closing in thirty-eight patients (22.2%), and it was closed in eighty-nine patients (52.1%) at the time of diagnosis. A CT scan was available for review for 186 patients of the total group of 199 patients. The tumors demonstrated intralesional mineralization in eighty-three (44.6%) of these patients, but showed cortical destruction in thirty-six patients (19.4%) and revealed invasion of the physis in thirty-nine
R
patients (21.0%). A CT scan with contrast was available for 106 patients. Ninety-one patients (85.8%) had lesions that demonstrated tumor enhancement. Of 112 patients with available MRI scans, seventy-two (64.3%) showed bone marrow edema, and thirty-one (27.7%) had edema of the adjacent joint. The aggressiveness of the lesion was classified, according to Enneking40, as latent in the tumors of seven patients (3.5%), active in the tumors of seventy-nine patients (39.7%), and aggressive in the tumors of 113 patients (56.8%). Of the 199 patients, thirteen (6.5%) presented with a pathologic fracture at the time of diagnosis. n NOTE: The authors thank Yang Sun, MD, Weifeng Liu, MD, Yongkun Yang, MD, Tao Jin, MD, Le Li, MD candidate, Bin Li, MD candidate, He Xiao, MD candidate, and Ming Gao, MD candidate, for their contributions to the data collection of this series.
Hairong Xu, MD Yi Ding, MD Xiaohui Niu, MD Departments of Orthopaedic Oncology Surgery (H.X. and X.N.) and Pathology (Y.D.), Beijing Ji Shui Tan Hospital, Peking University, 31 Xinjiekou East Street, Xicheng District, Beijing 100035, People’s Republic of China. E-mail address for X. Niu: [email protected] Dylan Nugent, MD Department of Orthopaedics and Rehabilitation, University of Florida College of Medicine, 3450 Hull Road, Gainesville, FL 32607 Hector L. Monforte, MD Department of Pathology, All Children’s Hospital, 501 6th Avenue South, St. Petersburg, FL 33701 Odion T. Binitie, MD G. Douglas Letson, MD David Cheong, MD Department of Sarcoma, H. Lee Moffitt Cancer Center & Research Institute, 12902 Magnolia Drive, Tampa, FL 33612
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