The Journal of Foot & Ankle Surgery xxx (2015) 1–4
Contents lists available at ScienceDirect
The Journal of Foot & Ankle Surgery journal homepage: www.jfas.org
Case Reports and Series
Limb Salvage in Secondary Chondrosarcoma of the Metatarsus Amit Sharma, MBBS 1, Lalit Maini, MS 2 1 2
Postgraduate Student in Orthopaedics, Department of Orthopaedics, Maulana Azad Medical College, Delhi, India Professor, Department of Orthopaedics, Maulana Azad Medical College, Delhi, India
a r t i c l e i n f o
a b s t r a c t
Level of Clinical Evidence: 4
Malignant transformation of metatarsal osteochondroma into chondrosarcoma is a rare entity. The most optimal line of treatment, salvage procedure or amputation, continues to be debated. Both of these treatments have varied in popularity. We report the case of a 24-year-old male with chondrosarcoma secondary to differentiation of an osteochondroma in the second and third metatarsals. Wide resection of the tumor mass and reconstruction of the second and third metatarsals was performed using a simple and biologic approach. This was accomplished with an autologous nonvascularized fibular graft to reconstruct the longitudinal arch of the foot. The foot was successfully salvaged, with good anatomic, functional, and aesthetic results. At 1 year postoperatively, the patient was able to walk without any orthotic devices and had returned to his routine work. No evidence of local recurrence or metastasis was observed. Ó 2015 by the American College of Foot and Ankle Surgeons. All rights reserved.
Keywords: chondrosarcoma fibular bone graft malignancy metatarsal oncologic surgery osteochondroma
Chondrosarcoma is the third most common primary malignant neoplasm of the bone. It has been observed that two thirds arise without any pre-existing benign lesion and have been referred to as primary chondrosarcomas (1,2). Primary chondrosarcoma is a tumor of adulthood and old age, with the peak incidence in the fifth to seventh decades of life. However, chondrosarcomas that are superimposed on preexisting benign cartilaginous neoplasms, such as enchondroma or osteochondroma, are referred to as secondary chondrosarcomas. They constitute about one third of all chondrosarcomas. Secondary chondrosarcoma arising from osteochondroma usually occurs in a relatively younger age group. The mean age at diagnosis has been reported to be 34.9 years (3,4). The incidence of secondary chondrosarcoma is rare in the foot. Instead, secondary chondrosarcoma has a predilection for flat bone, with pelvis being the most common site, followed by the femur and shoulder girdle (3,4). The present case was a 24-year-old male with chondrosarcoma of the second and third metatarsals secondary to differentiation of an osteochondroma that was treated with wide excision of the tumor and reconstruction of the foot.
Financial Disclosure: None reported. Conflict of Interest: None reported. Address correspondence to: Amit Sharma, MBBS, E–31, Radha Kunj, Brij Vihar, Ghaziabad, Uttar Pradesh 201011 India. E-mail address: [email protected] (A. Sharma).
Case Report A 24-year-old male was admitted in our hospital with complaints of swelling in the left foot for the previous 16 months (Fig. 1A) and associated with pain for the past 6 months. The patient described the nature of the pain as constant and mild that increased with weightbearing. The amount of swelling also increased during the 16-month period. He denied any history of trauma, erythema, or sensory changes to the foot. He also reported multiple nodular swellings in the leg and thigh that had been present since childhood. These swellings were, however, asymptomatic and had had a constant size for the previous 5 to 7 years. A review of the patient for any systemic illness was negative. On local physical examination, a bilobed swelling was palpated on the dorsum of the left foot with a size of around 4 cm 3 cm. The swelling was firm to hard in consistency and was firmly adherent to the underlying bone; the overlying skin was normal and was not adherent to the swelling, and no veins were dilated. Plain radiography revealed a lobulated outgrowth with considerable cortical destruction, endosteal scalloping, and punctate calcification involving the second and third metatarsals. It mainly involved the distal and dorsal aspects of the foot (Fig. 1B). Noncontrastenhanced computed tomography of the foot was suggestive of a large heterogeneous swelling arising from the second and third metatarsals showing punctate cartilaginous calcification with osteolytic areas (Fig. 2A). The extent of the swelling was up to the middiaphysis of the second and third metatarsal, excluding the base of the metatarsals (Fig. 2B). Magnetic resonance imaging was suggestive of an expansile destructive lesion involving the second and third
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Fig. 1. (A) Preoperative clinical photograph. (B) Preoperative anteroposterior radiograph.
metatarsal, measuring 5.4 4.3 4.0 cm, with a hypointense signal on the T1-weighted images (Fig. 2C) and a hyperintense signal on the T2-weighted images (Fig. 2D). The workup for metastasis, which included computed tomography of the chest and abdomen and a bone scan, was negative. The patient then underwent definitive operative management. En bloc tumor mass resection was performed by resecting the second and third metatarsals, leaving their base in place (Fig. 3A). The level of resection was decided in accordance with the criteria for wide resection. Thus, the bases of the metatarsals were left in place rather than resecting their full length. This served 2 benefits. It primarily preserved the midtarsal joint, avoiding the inevitable instability of the foot, which might have occurred after resecting the full metatarsals. Moreover, the chance of better uptake of the fibular graft when fixed to metatarsal base than to cuneiform bone (considering the anatomy) was also a consideration while operating. Intraoperatively, the tumor consisted of a well-encapsulated irregular mass of cartilaginous tissue measuring 5.5 cm 4.5 cm 3.5 cm. The specimen was white and firm, with intermittent soft gelatinous areas. After resection, evaluation of the resected tissue was done using immediate frozen section analysis, which showed clear tissue margins. Next, definitive management for the defect left in the foot was undertaken (Fig. 3B). The defect was reconstructed using a similar length of a nonvascularized fibular graft from the ipsilateral side (Fig. 3C). Proper care was taken while harvesting the fibular graft to avoid the slightest threat of tumor spread. The instruments used to resect the tumor mass were put aside, and a new set was used for fibular graft extraction. Fixation of the fibular graft in the foot was achieved with Kirschner wires (Fig. 4A). After fixation, the Kirschner wires were bent to minimize the risk of migration. The findings of the definitive histopathologic examination were suggestive of osteochondroma with secondary chondrosarcoma.
Postoperatively, marginal superficial necrosis of the skin flaps occurred that healed satisfactorily with antibiotics and wound dressings. A below-the-knee plaster cast with a window was applied. The patient was not allowed to bear weight for 2 months. After 2 months, the patient was allowed partial weightbearing using a foot orthosis. The progress was closely observed, with follow-up visits every 2 weeks. The Kirschner wires were removed at 4 months postoperatively after radiologic evidence of graft uptake was observed. During this period, the patient continued mobilizing with partial weightbearing using a foot brace. At 6 months postoperatively, full weightbearing was allowed using the brace. After 1 year of follow-up, radiographic evidence showed the graft had been incorporated (Fig. 4B), and the brace was no longer required. Clinically, the patient was maintaining a normal gait and his day-today activities without any orthotic support, and the foot appeared normal in contour (Fig. 4C). Discussion Osteochondroma is the most common “benign tumor” of the skeleton. They represent 35% of all benign tumors and 10% to 15% of all bone tumors (5). Most of these have been diagnosed in patients younger than 20 years old. A marked predilection for males exists, with a male-to-female ratio of 3:1 (6). It usually arises from the long bones, most commonly around the knee (40% of cases), with the distal femur (20% to 25%) more common than the proximal tibia (15% to 20%) (5,7). The risk of chondrosarcoma in a solitary osteochondroma has been reported to be 2% to 7% and 5% to 25% for multiple hereditary exostoses (8,9). Patients with Ollier’s disease and Maffucci syndrome have a 25% to 30% risk of developing chondrosarcoma (10–12). Chondrosarcoma is a malignant tumor with pure hyaline cartilage differentiation. It is the most common malignant tumor into which an
Fig. 2. (A) Preoperative computed tomography scan, sagittal view. (B) Preoperative computed tomography scan (3-dimensional reconstruction) demonstrating the tumor extent. (C) Preoperative sagittal T1-weighted magnetic resonance imaging scan. (D) Preoperative sagittal T2-weighted magnetic resonance imaging scan.
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Fig. 3. (A) Intraoperative photograph showing tumor excision. (B) Intraoperative photograph showing defect in foot after tumor excision. (C) Intraoperative photograph showing reconstruction of defect using fibular graft.
osteochondroma transforms, with the other being osteosarcoma, which has a rare occurrence (9). Malignant transformation is often associated with features of increased pain, a sudden increase in the size of the swelling, and an increase in the size of the cartilaginous cap (13). Chondrosarcoma is more commonly seen in the axial skeleton, with pelvis being the most common site. It is also commonly seen in the shoulder girdle, femur, and humerus, with the foot a rarer site. Its rarity of occurrence in the foot is shown in that of all the reported cases of chondrosarcoma, only 1% were seen in the pedal bones (14,15). In a study of >10,000 patients with malignant disease, in which 37 cases involved the foot, Schreiner and Wehr (16) found only 1 case of chondrosarcoma of the foot. Damron et al (17) reported only 3 cases involving the feet and hands among 2588 tumors. Henderson and Dahlin (18) reported 288 cases of chondrosarcoma, of which 6 (these were not subdivided further) involved either the foot or hand. McKenna et al (19) studied 151 cases of chondrosarcoma and found none in the foot. In a study of 83 foot tumor cases (both bone and soft tissue) by Kinoshita et al (20), chondrosarcoma was reported in only 1 case. In the series by Ogose et al (21), 75 cases of chondrosarcoma of the foot were reported. Of these, 16% originated from the metatarsal, 53% in the phalanges, 28% in the calcaneus, 13% in the talus, 2.6% in the cuboid, and 2.6% in the cuneiform bones (21).
Secondary chondrosarcoma is even rarer than primary chondrosarcoma of the foot. They constitute about 1% of all malignant bone tumors and 11.4% of all chondrosarcomas in the small bones of the hands and feet (4). In the study by Ahmed et al (4) of 107 patients with secondary chondrosarcoma, the 5- and 10-year local recurrence rate was 15.9% and 17.5%, and the 5- and 10-year mortality rate was 1.6% and 4.8%, respectively. Metastasis developed in 5 patients: in the lungs in 4 and in the groin in 1 patient. Local recurrence was the cause of death in most patients. Wide excision had the lowest local recurrence rate in their study (4). In a review of 627 patients with cartilageforming benign bone tumor, Altay et al (22) found only 32 patients (5%) with malignant transformation to chondrosarcoma. Of the 32 patients, 5 (15.6%) died of metastasis or local recurrence (22). Wuisman et al (23) reported a mortality rate of 11% in a review of 45 patients with secondary chondrosarcoma, with none of the patients developing local recurrence after primary wide resection. Considering the mortality associated with secondary chondrosarcoma and the risk of local recurrence, treatment using either wide excision or amputation is relevant. Other treatment options, such as intralesional excision, marginal excision, curettage with local adjuvant therapy such as cryosurgery, phenolization, or radiotherapy alone, have resulted in a considerable percentage of local recurrence, making these treatment modalities unsuitable (22–24).
Fig. 4. (A) Immediate postoperative radiograph showing fibular graft held in place with a Kirschner wire. (B) Postoperative radiograph at 1 year. (C) Postoperative clinical photograph at 1 year.
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A 2-stage procedure with reconstruction surgery at a later date could be another feasible option but has its own setbacks. First, a 2stage procedure unnecessarily exposes the patient to the side effects of a second anesthesia and surgery and also increases the chances of infection. Second, a 2-stage procedure could lead to difficulty in achieving adequate reconstruction with poor wound healing owing to the development of contracted scar tissue in the defect during the interval between the 2 surgeries. Hence, in our casedwith negative findings from intraoperative frozen section analysis, we opted for a single-stage procedure. Furthermore, different fixation devices such as plates and screws have been described to achieve fixation. Although, plates and screws are better fixation devices than Kirschner wires, with better strength, in our patient, we used Kirschner wires for 2 reasons. First, both plates and screws are cumbersome implants compared with a Kirschner wire, thus posing the threat of poor wound healing and skin coverage after foot surgery. Second, both plates and screws will hamper the vascularity of the bone more than will a Kirschner wire, increasing the chance of graft failure. Vascularized fibula could also be considered as an upcoming option but requires a lot of expertise. Although for all grades and subtypes of chondrosarcoma, only complete surgical treatment offers the chance for cure, the most optimal type of surgical management remains debated (25). Because the risk of local recurrence is minimal with wide surgical excision, the treatment of choice for chondrosarcoma is wide surgical excision (22–25). Moreover, in foot surgery, the wound margins could have healing problems owing to poor vascularity, such as occurred in our patient. Furthermore, en bloc removal of the tumor will leave a defect that requires reconstruction to restore the arch of the foot. In contrast, amputation would lead to significant loss of function and would also act as a major psychosocial trauma to the patient. Hence, in our patient, en bloc resection of tumor was done, followed by a simple approach of reconstruction using a fibular graft, which exhibited a good final outcome. In conclusion, orthopedic surgeons should be vigilant for signs of malignant transformation when monitoring patients with osteochondroma of an even rarer site such as the foot, although chondrosarcoma secondary to differentiation of an osteochondroma is a rarely encountered entity. Early recognition and appropriate surgical treatment are required to achieve successful outcomes. Adequate surgical treatment is the key to reduce the risk of local recurrence. Hence, wide resection, followed by a reconstructive procedure to maintain the functional and anatomic integrity of the limb, and adequate follow-up can be considered as the optimal first line of treatment, with least possibility of local recurrence. References 1. Henderson ED, Dahlin DC. Chondrosarcoma of bone: a study of two hundred and eighty-eight cases. J Bone Joint Surg Am 45:1450–1458, 1963.
2. Kenney PJ, Gilula LA, Murphy WA. The use of computed tomography to distinguish osteochondroma and chondrosarcoma. Radiology 139:129–137, 1981. 3. Garrison RC, Unni KK, McLeod RA, Pritchard DJ, Dahlin DC. Chondrosarcoma arising in osteochondroma. Cancer 49:1890–1897, 1982. 4. Ahmed AR, Tan TS, Unni KK, Collins MS, Wenger DE, Sim FH. Secondary chondrosarcoma in osteochondroma: report of 107 patients. Clin Orthop Relat Res 411:193–206, 2003. 5. Murphey MD, Choi JJ, Kransdorf MJ, Flemming DJ, Gannon FH. Imaging of osteochondroma: variants and complications with radiologic-pathologic correlation. Radiographics 20:1407–1434, 2000. 6. Kitsoulis P, Galani V, Stefanaki K, Paraskevas G, Karatzias G, Agnantis NJ, Bai M. Osteochondromas: review of the clinical, radiological and pathological features. In Vivo 22:633–646, 2008. 7. Blitz NM, Lopez KT. Giant solitary osteochondroma of the inferior medial calcaneal tubercle: a case report and review of the literature. J Foot Ankle Surg 47:206–212, 2008. 8. Nogier A, De Pinieux G, Hottya G, Anract P. Case reports: enlargement of a calcaneal osteochondroma after skeletal maturity. Clin Orthop Relat Res 447:260– 266, 2006. 9. Bertoni F, Bacchini P, Hogendoorn PCW. Chondrosarcoma. In: World Health Organization Classification of Tumors: Pathology and Genetics of Tumors of Soft Tissue and Bone, edited by CDM Fletcher, KK Unni, F Mertens, IARC Press, Lyon, France, 2002. 10. Brien EW, Mirra JM, Kerr R. Benign and malignant cartilage tumors of bone and joint: their anatomic and theoretical basis with an emphasis on radiology, pathology and clinical biology. I. The intramedullary cartilage tumors. Skeletal Radiol 26:325–353, 1997. 11. Liu J, Hudkins PG, Swee RG, Unni KK. Bone sarcomas associated with Ollier’s disease. Cancer 59:1376–1385, 1987. 12. Schwartz HS, Zimmerman NB, Simon MA, Wroble RR, Millar EA, Bonfiglio M. The malignant potential of enchondromatosis. J Bone Joint Surg Am 69:269–274, 1987. 13. Murphey MD, Walker EA, Wilson AJ, Kransdorf MJ, Temple HT, Gannon FH. From the archives of the AFIP imaging of primary chondrosarcoma: radiologicpathologic correlation. Radiographics 23:1245–1278, 2003. 14. Wiss DA. Chondrosarcoma of first metatarsal. J Surg Oncol 23:110–112, 1983. 15. Johnston JO. Chapter 24. In: Surgery of the Foot and Ankle, ed 6, pp. 1017–1018, edited by RA Mann, MJ Coughlin, Mosby-Year Book, St. Louis, 1993. 16. Schreiner BF, Wehr W. Primary malignant tumors of the foot: a report of 37 cases. Radiology 21:513–521, 1933. 17. Damron TA, Rock MG, Unni KK. Subcutaneous involvement after metacarpal chondrosarcoma: case report and review of literature. Clin Orthop Relat Res 316:189–194, 1995. 18. Jaffe HL. Tumors and Tumorous Conditions of the Bones and Joints, Lea & Febiger, Philadelphia, 1958. 19. McKenna RJ, Schwinn CP, Soong KY, Higinbotham NL. Sarcomata of the osteogenic series. J Bone Joint Surg Am 48:l–25, 1966. 20. Kinoshita G, Matsumoto M, Maruoka T, Shiraki T, Tsunemi K, Futani H, Maruo S. Bone and soft tissue tumors of the foot: review of 83 cases. J Orthop Surg (Hong Kong) 10:173–178, 2002. 21. Ogose A, Unni KK, Swee RG, May GK, Rowland CM, Sim FH. Chondrosarcoma of small bones of the hands and feet. Cancer 80:50–59, 1997. 22. Altay M, Bayrakci K, Yildiz Y, Erekul S, Saglik Y. Secondary chondrosarcoma in cartilage bone tumors: report of 32 patients. J Orthop Sci 12:415–423, 2007. 23. Wuisman PI, Jutte PC, Ozaki T. Secondary chondrosarcoma in osteochondromas: medullary extension in 15 of 45 cases. Acta Orthop Scand 68:396–400, 1997. 24. Mittermayr F, Dominkus M, Krepler P, Schwaneis E, Sloga M, Toma C, Long S, Grampp S, Kotz R. Chondrosarcoma of the hand. Clin Orthop Relat Res 424:211– 215, 2004. 25. Mondal SK. Chondrosarcoma of the distal phalanx of the right great toe: report of a rare malignancy and review of literature. J Cancer Res Ther 8:123–125, 2012.
Contents lists available at ScienceDirect
The Journal of Foot & Ankle Surgery journal homepage: www.jfas.org
Case Reports and Series
Limb Salvage in Secondary Chondrosarcoma of the Metatarsus Amit Sharma, MBBS 1, Lalit Maini, MS 2 1 2
Postgraduate Student in Orthopaedics, Department of Orthopaedics, Maulana Azad Medical College, Delhi, India Professor, Department of Orthopaedics, Maulana Azad Medical College, Delhi, India
a r t i c l e i n f o
a b s t r a c t
Level of Clinical Evidence: 4
Malignant transformation of metatarsal osteochondroma into chondrosarcoma is a rare entity. The most optimal line of treatment, salvage procedure or amputation, continues to be debated. Both of these treatments have varied in popularity. We report the case of a 24-year-old male with chondrosarcoma secondary to differentiation of an osteochondroma in the second and third metatarsals. Wide resection of the tumor mass and reconstruction of the second and third metatarsals was performed using a simple and biologic approach. This was accomplished with an autologous nonvascularized fibular graft to reconstruct the longitudinal arch of the foot. The foot was successfully salvaged, with good anatomic, functional, and aesthetic results. At 1 year postoperatively, the patient was able to walk without any orthotic devices and had returned to his routine work. No evidence of local recurrence or metastasis was observed. Ó 2015 by the American College of Foot and Ankle Surgeons. All rights reserved.
Keywords: chondrosarcoma fibular bone graft malignancy metatarsal oncologic surgery osteochondroma
Chondrosarcoma is the third most common primary malignant neoplasm of the bone. It has been observed that two thirds arise without any pre-existing benign lesion and have been referred to as primary chondrosarcomas (1,2). Primary chondrosarcoma is a tumor of adulthood and old age, with the peak incidence in the fifth to seventh decades of life. However, chondrosarcomas that are superimposed on preexisting benign cartilaginous neoplasms, such as enchondroma or osteochondroma, are referred to as secondary chondrosarcomas. They constitute about one third of all chondrosarcomas. Secondary chondrosarcoma arising from osteochondroma usually occurs in a relatively younger age group. The mean age at diagnosis has been reported to be 34.9 years (3,4). The incidence of secondary chondrosarcoma is rare in the foot. Instead, secondary chondrosarcoma has a predilection for flat bone, with pelvis being the most common site, followed by the femur and shoulder girdle (3,4). The present case was a 24-year-old male with chondrosarcoma of the second and third metatarsals secondary to differentiation of an osteochondroma that was treated with wide excision of the tumor and reconstruction of the foot.
Financial Disclosure: None reported. Conflict of Interest: None reported. Address correspondence to: Amit Sharma, MBBS, E–31, Radha Kunj, Brij Vihar, Ghaziabad, Uttar Pradesh 201011 India. E-mail address: [email protected] (A. Sharma).
Case Report A 24-year-old male was admitted in our hospital with complaints of swelling in the left foot for the previous 16 months (Fig. 1A) and associated with pain for the past 6 months. The patient described the nature of the pain as constant and mild that increased with weightbearing. The amount of swelling also increased during the 16-month period. He denied any history of trauma, erythema, or sensory changes to the foot. He also reported multiple nodular swellings in the leg and thigh that had been present since childhood. These swellings were, however, asymptomatic and had had a constant size for the previous 5 to 7 years. A review of the patient for any systemic illness was negative. On local physical examination, a bilobed swelling was palpated on the dorsum of the left foot with a size of around 4 cm 3 cm. The swelling was firm to hard in consistency and was firmly adherent to the underlying bone; the overlying skin was normal and was not adherent to the swelling, and no veins were dilated. Plain radiography revealed a lobulated outgrowth with considerable cortical destruction, endosteal scalloping, and punctate calcification involving the second and third metatarsals. It mainly involved the distal and dorsal aspects of the foot (Fig. 1B). Noncontrastenhanced computed tomography of the foot was suggestive of a large heterogeneous swelling arising from the second and third metatarsals showing punctate cartilaginous calcification with osteolytic areas (Fig. 2A). The extent of the swelling was up to the middiaphysis of the second and third metatarsal, excluding the base of the metatarsals (Fig. 2B). Magnetic resonance imaging was suggestive of an expansile destructive lesion involving the second and third
1067-2516/$ - see front matter Ó 2015 by the American College of Foot and Ankle Surgeons. All rights reserved. http://dx.doi.org/10.1053/j.jfas.2014.09.018
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Fig. 1. (A) Preoperative clinical photograph. (B) Preoperative anteroposterior radiograph.
metatarsal, measuring 5.4 4.3 4.0 cm, with a hypointense signal on the T1-weighted images (Fig. 2C) and a hyperintense signal on the T2-weighted images (Fig. 2D). The workup for metastasis, which included computed tomography of the chest and abdomen and a bone scan, was negative. The patient then underwent definitive operative management. En bloc tumor mass resection was performed by resecting the second and third metatarsals, leaving their base in place (Fig. 3A). The level of resection was decided in accordance with the criteria for wide resection. Thus, the bases of the metatarsals were left in place rather than resecting their full length. This served 2 benefits. It primarily preserved the midtarsal joint, avoiding the inevitable instability of the foot, which might have occurred after resecting the full metatarsals. Moreover, the chance of better uptake of the fibular graft when fixed to metatarsal base than to cuneiform bone (considering the anatomy) was also a consideration while operating. Intraoperatively, the tumor consisted of a well-encapsulated irregular mass of cartilaginous tissue measuring 5.5 cm 4.5 cm 3.5 cm. The specimen was white and firm, with intermittent soft gelatinous areas. After resection, evaluation of the resected tissue was done using immediate frozen section analysis, which showed clear tissue margins. Next, definitive management for the defect left in the foot was undertaken (Fig. 3B). The defect was reconstructed using a similar length of a nonvascularized fibular graft from the ipsilateral side (Fig. 3C). Proper care was taken while harvesting the fibular graft to avoid the slightest threat of tumor spread. The instruments used to resect the tumor mass were put aside, and a new set was used for fibular graft extraction. Fixation of the fibular graft in the foot was achieved with Kirschner wires (Fig. 4A). After fixation, the Kirschner wires were bent to minimize the risk of migration. The findings of the definitive histopathologic examination were suggestive of osteochondroma with secondary chondrosarcoma.
Postoperatively, marginal superficial necrosis of the skin flaps occurred that healed satisfactorily with antibiotics and wound dressings. A below-the-knee plaster cast with a window was applied. The patient was not allowed to bear weight for 2 months. After 2 months, the patient was allowed partial weightbearing using a foot orthosis. The progress was closely observed, with follow-up visits every 2 weeks. The Kirschner wires were removed at 4 months postoperatively after radiologic evidence of graft uptake was observed. During this period, the patient continued mobilizing with partial weightbearing using a foot brace. At 6 months postoperatively, full weightbearing was allowed using the brace. After 1 year of follow-up, radiographic evidence showed the graft had been incorporated (Fig. 4B), and the brace was no longer required. Clinically, the patient was maintaining a normal gait and his day-today activities without any orthotic support, and the foot appeared normal in contour (Fig. 4C). Discussion Osteochondroma is the most common “benign tumor” of the skeleton. They represent 35% of all benign tumors and 10% to 15% of all bone tumors (5). Most of these have been diagnosed in patients younger than 20 years old. A marked predilection for males exists, with a male-to-female ratio of 3:1 (6). It usually arises from the long bones, most commonly around the knee (40% of cases), with the distal femur (20% to 25%) more common than the proximal tibia (15% to 20%) (5,7). The risk of chondrosarcoma in a solitary osteochondroma has been reported to be 2% to 7% and 5% to 25% for multiple hereditary exostoses (8,9). Patients with Ollier’s disease and Maffucci syndrome have a 25% to 30% risk of developing chondrosarcoma (10–12). Chondrosarcoma is a malignant tumor with pure hyaline cartilage differentiation. It is the most common malignant tumor into which an
Fig. 2. (A) Preoperative computed tomography scan, sagittal view. (B) Preoperative computed tomography scan (3-dimensional reconstruction) demonstrating the tumor extent. (C) Preoperative sagittal T1-weighted magnetic resonance imaging scan. (D) Preoperative sagittal T2-weighted magnetic resonance imaging scan.
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Fig. 3. (A) Intraoperative photograph showing tumor excision. (B) Intraoperative photograph showing defect in foot after tumor excision. (C) Intraoperative photograph showing reconstruction of defect using fibular graft.
osteochondroma transforms, with the other being osteosarcoma, which has a rare occurrence (9). Malignant transformation is often associated with features of increased pain, a sudden increase in the size of the swelling, and an increase in the size of the cartilaginous cap (13). Chondrosarcoma is more commonly seen in the axial skeleton, with pelvis being the most common site. It is also commonly seen in the shoulder girdle, femur, and humerus, with the foot a rarer site. Its rarity of occurrence in the foot is shown in that of all the reported cases of chondrosarcoma, only 1% were seen in the pedal bones (14,15). In a study of >10,000 patients with malignant disease, in which 37 cases involved the foot, Schreiner and Wehr (16) found only 1 case of chondrosarcoma of the foot. Damron et al (17) reported only 3 cases involving the feet and hands among 2588 tumors. Henderson and Dahlin (18) reported 288 cases of chondrosarcoma, of which 6 (these were not subdivided further) involved either the foot or hand. McKenna et al (19) studied 151 cases of chondrosarcoma and found none in the foot. In a study of 83 foot tumor cases (both bone and soft tissue) by Kinoshita et al (20), chondrosarcoma was reported in only 1 case. In the series by Ogose et al (21), 75 cases of chondrosarcoma of the foot were reported. Of these, 16% originated from the metatarsal, 53% in the phalanges, 28% in the calcaneus, 13% in the talus, 2.6% in the cuboid, and 2.6% in the cuneiform bones (21).
Secondary chondrosarcoma is even rarer than primary chondrosarcoma of the foot. They constitute about 1% of all malignant bone tumors and 11.4% of all chondrosarcomas in the small bones of the hands and feet (4). In the study by Ahmed et al (4) of 107 patients with secondary chondrosarcoma, the 5- and 10-year local recurrence rate was 15.9% and 17.5%, and the 5- and 10-year mortality rate was 1.6% and 4.8%, respectively. Metastasis developed in 5 patients: in the lungs in 4 and in the groin in 1 patient. Local recurrence was the cause of death in most patients. Wide excision had the lowest local recurrence rate in their study (4). In a review of 627 patients with cartilageforming benign bone tumor, Altay et al (22) found only 32 patients (5%) with malignant transformation to chondrosarcoma. Of the 32 patients, 5 (15.6%) died of metastasis or local recurrence (22). Wuisman et al (23) reported a mortality rate of 11% in a review of 45 patients with secondary chondrosarcoma, with none of the patients developing local recurrence after primary wide resection. Considering the mortality associated with secondary chondrosarcoma and the risk of local recurrence, treatment using either wide excision or amputation is relevant. Other treatment options, such as intralesional excision, marginal excision, curettage with local adjuvant therapy such as cryosurgery, phenolization, or radiotherapy alone, have resulted in a considerable percentage of local recurrence, making these treatment modalities unsuitable (22–24).
Fig. 4. (A) Immediate postoperative radiograph showing fibular graft held in place with a Kirschner wire. (B) Postoperative radiograph at 1 year. (C) Postoperative clinical photograph at 1 year.
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A 2-stage procedure with reconstruction surgery at a later date could be another feasible option but has its own setbacks. First, a 2stage procedure unnecessarily exposes the patient to the side effects of a second anesthesia and surgery and also increases the chances of infection. Second, a 2-stage procedure could lead to difficulty in achieving adequate reconstruction with poor wound healing owing to the development of contracted scar tissue in the defect during the interval between the 2 surgeries. Hence, in our casedwith negative findings from intraoperative frozen section analysis, we opted for a single-stage procedure. Furthermore, different fixation devices such as plates and screws have been described to achieve fixation. Although, plates and screws are better fixation devices than Kirschner wires, with better strength, in our patient, we used Kirschner wires for 2 reasons. First, both plates and screws are cumbersome implants compared with a Kirschner wire, thus posing the threat of poor wound healing and skin coverage after foot surgery. Second, both plates and screws will hamper the vascularity of the bone more than will a Kirschner wire, increasing the chance of graft failure. Vascularized fibula could also be considered as an upcoming option but requires a lot of expertise. Although for all grades and subtypes of chondrosarcoma, only complete surgical treatment offers the chance for cure, the most optimal type of surgical management remains debated (25). Because the risk of local recurrence is minimal with wide surgical excision, the treatment of choice for chondrosarcoma is wide surgical excision (22–25). Moreover, in foot surgery, the wound margins could have healing problems owing to poor vascularity, such as occurred in our patient. Furthermore, en bloc removal of the tumor will leave a defect that requires reconstruction to restore the arch of the foot. In contrast, amputation would lead to significant loss of function and would also act as a major psychosocial trauma to the patient. Hence, in our patient, en bloc resection of tumor was done, followed by a simple approach of reconstruction using a fibular graft, which exhibited a good final outcome. In conclusion, orthopedic surgeons should be vigilant for signs of malignant transformation when monitoring patients with osteochondroma of an even rarer site such as the foot, although chondrosarcoma secondary to differentiation of an osteochondroma is a rarely encountered entity. Early recognition and appropriate surgical treatment are required to achieve successful outcomes. Adequate surgical treatment is the key to reduce the risk of local recurrence. Hence, wide resection, followed by a reconstructive procedure to maintain the functional and anatomic integrity of the limb, and adequate follow-up can be considered as the optimal first line of treatment, with least possibility of local recurrence. References 1. Henderson ED, Dahlin DC. Chondrosarcoma of bone: a study of two hundred and eighty-eight cases. J Bone Joint Surg Am 45:1450–1458, 1963.
2. Kenney PJ, Gilula LA, Murphy WA. The use of computed tomography to distinguish osteochondroma and chondrosarcoma. Radiology 139:129–137, 1981. 3. Garrison RC, Unni KK, McLeod RA, Pritchard DJ, Dahlin DC. Chondrosarcoma arising in osteochondroma. Cancer 49:1890–1897, 1982. 4. Ahmed AR, Tan TS, Unni KK, Collins MS, Wenger DE, Sim FH. Secondary chondrosarcoma in osteochondroma: report of 107 patients. Clin Orthop Relat Res 411:193–206, 2003. 5. Murphey MD, Choi JJ, Kransdorf MJ, Flemming DJ, Gannon FH. Imaging of osteochondroma: variants and complications with radiologic-pathologic correlation. Radiographics 20:1407–1434, 2000. 6. Kitsoulis P, Galani V, Stefanaki K, Paraskevas G, Karatzias G, Agnantis NJ, Bai M. Osteochondromas: review of the clinical, radiological and pathological features. In Vivo 22:633–646, 2008. 7. Blitz NM, Lopez KT. Giant solitary osteochondroma of the inferior medial calcaneal tubercle: a case report and review of the literature. J Foot Ankle Surg 47:206–212, 2008. 8. Nogier A, De Pinieux G, Hottya G, Anract P. Case reports: enlargement of a calcaneal osteochondroma after skeletal maturity. Clin Orthop Relat Res 447:260– 266, 2006. 9. Bertoni F, Bacchini P, Hogendoorn PCW. Chondrosarcoma. In: World Health Organization Classification of Tumors: Pathology and Genetics of Tumors of Soft Tissue and Bone, edited by CDM Fletcher, KK Unni, F Mertens, IARC Press, Lyon, France, 2002. 10. Brien EW, Mirra JM, Kerr R. Benign and malignant cartilage tumors of bone and joint: their anatomic and theoretical basis with an emphasis on radiology, pathology and clinical biology. I. The intramedullary cartilage tumors. Skeletal Radiol 26:325–353, 1997. 11. Liu J, Hudkins PG, Swee RG, Unni KK. Bone sarcomas associated with Ollier’s disease. Cancer 59:1376–1385, 1987. 12. Schwartz HS, Zimmerman NB, Simon MA, Wroble RR, Millar EA, Bonfiglio M. The malignant potential of enchondromatosis. J Bone Joint Surg Am 69:269–274, 1987. 13. Murphey MD, Walker EA, Wilson AJ, Kransdorf MJ, Temple HT, Gannon FH. From the archives of the AFIP imaging of primary chondrosarcoma: radiologicpathologic correlation. Radiographics 23:1245–1278, 2003. 14. Wiss DA. Chondrosarcoma of first metatarsal. J Surg Oncol 23:110–112, 1983. 15. Johnston JO. Chapter 24. In: Surgery of the Foot and Ankle, ed 6, pp. 1017–1018, edited by RA Mann, MJ Coughlin, Mosby-Year Book, St. Louis, 1993. 16. Schreiner BF, Wehr W. Primary malignant tumors of the foot: a report of 37 cases. Radiology 21:513–521, 1933. 17. Damron TA, Rock MG, Unni KK. Subcutaneous involvement after metacarpal chondrosarcoma: case report and review of literature. Clin Orthop Relat Res 316:189–194, 1995. 18. Jaffe HL. Tumors and Tumorous Conditions of the Bones and Joints, Lea & Febiger, Philadelphia, 1958. 19. McKenna RJ, Schwinn CP, Soong KY, Higinbotham NL. Sarcomata of the osteogenic series. J Bone Joint Surg Am 48:l–25, 1966. 20. Kinoshita G, Matsumoto M, Maruoka T, Shiraki T, Tsunemi K, Futani H, Maruo S. Bone and soft tissue tumors of the foot: review of 83 cases. J Orthop Surg (Hong Kong) 10:173–178, 2002. 21. Ogose A, Unni KK, Swee RG, May GK, Rowland CM, Sim FH. Chondrosarcoma of small bones of the hands and feet. Cancer 80:50–59, 1997. 22. Altay M, Bayrakci K, Yildiz Y, Erekul S, Saglik Y. Secondary chondrosarcoma in cartilage bone tumors: report of 32 patients. J Orthop Sci 12:415–423, 2007. 23. Wuisman PI, Jutte PC, Ozaki T. Secondary chondrosarcoma in osteochondromas: medullary extension in 15 of 45 cases. Acta Orthop Scand 68:396–400, 1997. 24. Mittermayr F, Dominkus M, Krepler P, Schwaneis E, Sloga M, Toma C, Long S, Grampp S, Kotz R. Chondrosarcoma of the hand. Clin Orthop Relat Res 424:211– 215, 2004. 25. Mondal SK. Chondrosarcoma of the distal phalanx of the right great toe: report of a rare malignancy and review of literature. J Cancer Res Ther 8:123–125, 2012.
