522094 research-articleXXXX

FASXXX10.1177/1938640014522094Foot & Ankle SpecialistFoot & Ankle Specialist

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〈 Case Report 〉 Giant Cell Tumor of the Distal Phalanx of the Great Toe

Samuel G. Dellenbaugh, MD, Lee Wilkinson, MD, and Umur Aydogan, MD

A Case Report

Abstract: Giant cell tumors are rarely seen in the foot. They can cause a significant amount of pain and deformity due to their aggressive and recurrent nature. We present the unusual case of a giant cell tumor of the distal phalanx of the hallux in a 39-year-old man. Levels of Evidence: Therapeutic Level IV, Case Report Keywords: benign and malignant tumors; foot surgery techniques; big toe joint pain

G

iant cell tumor (GCT) of bone is a moderately common tumor of bone, accounting for approximately 6% of all bone tumors and 20% of benign bone tumors.1,2 There is a higher prevalence noted in southern India and China, where 20% of all primary bone tumors are GCT.3,4 First described by Cooper and Travers in 1818,5 it is typically seen in the metaphyseal and epiphyseal regions of the long bones and rarely seen in the foot.6 Typically, this tumor presents as a well-circumscribed, eccentric lytic lesion that lacks a sclerotic border and is juxtaarticular. Histologically it is normally

which showed abnormal signal intensity described as a background of involving the entire distal phalanx of the mononuclear stromal cells with many hallux, which was thought to be of multinucleated giant cells, which appear indeterminate etiology—most likely an similar to osteoclasts, leading to the enchondroma—but other processes such initial description of the tumor as an as osteomyelitis or other malignancies osteoclastoma.7 could not be excluded (Figures 1-3). Incidences in the foot noted in the X-rays taken on the date of presentation literature range from 1.2% noted by revealed an osteolytic lesion involving Unni2 to 1.8% as described by Huvos.8 the distal phalanx of the great toe with One 50-year case series of 900 cases of associated endosteal scalloping and focal GCT had a 2.4% incidence in the foot, cortical destruction of the dorsal aspect none distal to the base of the metatarsals.9 We present an unusual case of GCT Giant cell tumor (GCT) of bone is a of the distal phalanx of the hallux of a middlemoderately common tumor of bone, aged male.

Case Report



accounting for approximately 6% of all bone tumors and 20% of benign bone

A 39-year-old man presented to our tumors.” outpatient clinic as a referral from an outside facility. He had been having pain in his left great toe for approximately 6 months. He denied any trauma to the area. This pain was worse with walking or standing. He was experiencing no constitutional symptoms. The patient brought with him a magnetic resonance imaging (MRI) scan from a month earlier,

of the distal phalanx (Figures 4-6). The differential diagnosis at this time was giant cell reparative granuloma, enchondroma, or epidermal inclusion cyst. An excisional biopsy was planned at that time. The patient was taken to the operating room and an L-shaped incision was

DOI: 10.1177/1938640014522094. From the Penn State Hershey Bone and Joint Institute, Hershey, Pennsylvania. Address correspondence to Samuel G. Dellenbaugh, MD, OrthoNY, 121 Everett Road, Albany NY 12208; e-mail: [email protected]. For reprints and permissions queries, please visit SAGE’s Web site at http://www.sagepub.com/journalsPermissions.nav. Copyright © 2014 The Author(s)

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Figure 1.

Figure 4.

MRI T2 axial.

X-ray AP foot.

Figure 2. MRI T2 sagittal. Figure 5. X-ray oblique foot.

Figure 3. MRI T2 coronal.

made, with the horizontal limb along the interphalangeal joint of the hallux and the vertical limb along the lateral aspect of the great toe, as described by Horst and Nunley10 (Figure 7). The subcutaneous tissues were dissected and the extensor tendons as well as the neurovascular structures were identified and protected. On sharply dissecting the nail matrix from the bone, we came upon a section of the dorsomedial cortex

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that had been eroded by the lesion. We did a curettage of the lesion, which had a reddish, vascular appearance, and sent it for a frozen section, which was reported to be a GCT. We then did a complete curettage of the lesion, followed by burring the walls of the cavity until normal bone was visible. After the burring, we filled the cavity with hydrogen peroxide as a chemical ablative agent. The wound was then copiously irrigated and the void was filled with bone putty and the incision was closed. Pathologic evaluation of the interoperative tissue sample via frozen section described a tissue diagnosis of a giant cell lesion. The lesion evidenced a uniform distribution of multinucleated giant cells within a spindled stroma without excessive hemorrhage. The diagnosis of giant cell reparative granuloma was considered, but was not the favored diagnosis, as the specimen demonstrated a uniform distribution of giant cells, whereas giant cell reparative granuloma classically demonstrates nodular clusters of giant cells with fibrosis and stromal hemorrhages (Figure 8). Additionally, the giant cells present included numerous very large cells with large numbers of nuclei. This feature also favors the diagnosis of GCT over giant cell reparative granuloma, as the later tends to have smaller giant cells with fewer numbers of nuclei. The patient was doing well at his 6-month postoperative appointment. His incisions were well healed, and he had excellent range of motion of the distal interphalangeal joint (Figures 9 and 10). The graft had been incorporated by his body, and new bone had filled the site of the lesion (Figures 11 and 12). He was asymptomatic and very satisfied with the result of his surgery.

Discussion Giant cell tumor is typically seen in patients 20 to 40 years of age. They are most commonly seen in the metaphyseal and epiphyseal regions of the distal femur, the proximal tibia, the distal radius, and the proximal humerus.1,8,11,12

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Figure 6.

Figure 11.

X-ray lateral foot.

X-ray AP foot 6 months postoperative.

Figure 7.

Figure 9.

Intraoperative photograph showing surgical approach to lesion.

Lateral of toe, 6 months postoperative.

Figure 8.

Figure 10.

Larger image (100×) demonstrating the even distribution of giant cells and cellular background, with inset (200×) demonstrating the larger multinucleated giant cells.

Lateral of toe, showing IP range of motion.

Approximately half of all lesions present around the knee. Rarely seen are lesions

in the phalanx, metatarsal, or metacarpal. The Hamburg Bone Tumor Registry had only 1 lesion in the metatarsals and 1 lesion in the phalanges of the toes noted

on a total of 189 patients. The lesions that present in these areas tend to be in younger patients and are more aggressive than GCTs in the long bones.4 Review of the recent published literature yields 6 published articles describing GCT involving the phalanges of the foot.9,13-16,26 The vast majority of the lesions described were located in the metatarsals or proximally. Only 3 were located within the phalanges and only 1 lesion was located within the distal phalanx of the hallux. It is important to discriminate between GCTs of bone and other giant cell containing lesions seen in the foot, such as aneurysmal bone cysts and giant cell reparative granulomas. These all can appear similar on physical exam and radiographic evaluation. Pathologic examination is the main method of differentiation between these entities, which have some degree of histologic similarity. Each lesion shares the presence of giant cells, varying degrees of hemorrhage, and a fibrous and generally cellular background. The GCT of bone has the most uniform distribution of giant cells with a cellular background, whereas the giant cell

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Figure 12. X-ray lateral foot 6 months postoperative.

reparative granuloma has a more fibrotic stroma with clustered giant cells often centered on the stromal hemorrhage. Other lesions can imitate GCTs of bone, in both their location and radiologic appearance. Secondary aneurysmal bone cysts (ABCs) form in approximately 14% of cases of GCT of bone25; and therefore primary ABC can mimic GCT. However, primary ABC do not have an enhancing soft tissue component to the tumor, whereas ABCs secondary to a GCT have a soft tissue component.18 Chondroblastomas are usually also included in the differential diagnosis for a suspected GCT lesion. It is epiphyseal and can form secondary ABCs similar to GCTs of bone. However, chondroblastoma typically has a sclerotic border on radiographs and histologically shows central calcification with a chicken wire appearance of chondroid matrix. Some subtypes of osteosarcoma, such as telangiectatic, giant cell-rich, and fibroblastic osteosarcoma, can be expansive in nature and lack the large amounts of osteoid matrix typically seen in osteosarcomas. Clear cell chondrosarcoma needs also to be in the differential diagnosis, as it does occur in the epiphysis and can appear similar to GCT.18 Other disease processes in the differential of any radiolucent lesion seen in the phalanges of the foot can include brown tumors of hyperparathyroidism,

enchondromas, nonossifying fibroma, and infections. More severe consequences of GCTs of bone include metastasis and malignant transformation. Metastasis occurs in approximately 1% to 6% of cases, and these lesions are believed to originate via hematogenous seeding of GCT of bone, typically after treatment.19 These lesions are benign appearing on histology, appearing similar to the primary tumor, and are typically resected. Unresectable tumors can be treated nonoperatively, as they typically do not grow aggressively.19-21 Malignant transformation can occur rarely, either from de-differentiation of the primary tumor or secondary to radiation therapy.18 This occurs less than 1% of the time.22-24 However, when malignant transformation occurs, a high-grade sarcoma is usually diagnosed, with the commensurate poor prognosis. This transformation occurs approximately 9 years after initial GCT diagnosis in patients treated with radiation and after 19 years in cases of spontaneous transformation.22 Traditional surgical treatment options for GCTs of bone include curettage or en-bloc excision. Therefore, intraoperative frozen section should be performed in every case as the lesion is usually destructive in nature and could be misidentified as malignant in nature. The aggressiveness of the tumor and the site of the tumor will drive the choice of

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treatment. Lesions that are perceived to be less aggressive are typically treated with curettage. However, this has a higher rate of recurrence than excision.1,2 More aggressive lesions can be treated with en-bloc excision. Recently, the drug denosumab has been used to treat GCT of bone. This is a monoclonal antibody that targets the activator of the NF κ-β receptor and thus decreasing the osteoclastic activity of cells in GCT. This has been shown in one article, which described both laboratory studies and phase 2 trials, to cause 90% tumor necrosis.17 Studies in laboratories at the University of Southern California have shown reconstitution of cortical bone and sclerosis after treatment with denosumab.18 Current thinking is that this drug can be used as a preoperative treatment to make surgical resection of aggressive tumors easier and possibly as a nonsurgical treatment in patients with inoperable tumors. Further research is being done currently on this topic.

Conclusion Giant cell tumors are most often seen within the long bones of the lower extremity and are rarely seen in the small bones of the foot. However, they are a significant percentage of the tumors seen in this location. Although these lesions are rare, it is important to accurately diagnose the disease process and use the treatment for the location and aggressiveness of the tumor. The L-incision technique is very appropriate for lesions involving the distal phalanx in order to protect the nail matrix and get a good surgical exposure of the lesion in order to decide between treatment options. Some new nonsurgical treatments are in development, but are not usable at this time.

References 1. Turcotte RE. Giant cell tumor of bone. Orthop Clin North Am. 2006;37:35-51. 2. Unni KK, ed. Dahlins Bone Tumors: General Aspects and Data on 10165 Cases. 6th ed. Philadelphia, PA: Lippincott-Raven; 2010:225-242.

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3. Arnold RT, van Holsbeeck MT, Mayer TG, Mott MP, Koch SR. Necrotic giant cell tumor of bone manifesting with pathologic fracture. Radiographics. 2011;31:35-51.

12. Wülling M, Engels C, Jesse N, Werner M, Delling G, Kaiser E. The nature of giant cell tumor of bone. J Cancer Res Clin Oncol. 2001;127:467-474.

4. Dhillon MS, Prasad P. Multicentric giant cell tumour of bone. Acta Orthop Belg. 2007;73:289-299.

13. Alvarez Ramos A, Valverde Garcia JA, Garcia Alonso MG. Giant cell tumor of the foot phalanges in children: a case report. J Pediatr Orthop B. 1999;8:132-135.

5. Cooper AS, Travers B. Surgical Essays. London, England: Cox Longman; 1818: 178-179. 6. Raskin KA, Schwab JH, Mankin HJ, Springfield DS, Hornicek FJ. Giant cell tumor of bone. J Am Acad Orthop Surg. 2013;21:118-126. 7. Balke M, Schremper L, Gebert C, et al. Giant cell tumor of bone, treatment and outcome of 214 cases. J Cancer Res Clin Oncol. 2008;134:969-978. 8. Huvos AG,ed. Bone Tumors: Diagnosis, Treatment, and Prognosis. 2nd ed. Philadelphia, PA: WB Saunders; 1998: 429-467. 9. Boscaglia R, Bacchini P, Bertoni F. Giant cell tumor of the bones of the hand and feet. Cancer. 2000;88:2022-2032. 10. Horst F, Nunley JA. Technique tip: Glomus tumors in the foot: a new surgical technique for removal. Foot Ankle Int. 2003;24:949-951. 11. Eckardt JJ, Grogan TJ. Giant cell tumor of bone. Clin Orthop Relat Res. 1986;(204): 45-58.

14. Bachhal V, Rangdal S, Saini U, Sament R. Giant cell tumor of distal phalanx of great toe. A case report. Foot (Edinb). 2011;21:198-200. 15. Bibbo C. Metatarsal giant cell tumor in adolescents. Foot Ankle Int. 2010;31: 717-724. 16. Fugjisawa Y, Takahashi T, Kawachi Y, Otsuka F. Giant cell tumor of the distal phalanx of the foot. Eur J Dermatol. 2006;16:204-205. 17. Thomas D, Henshaw R, Skubitz K, et al. Denosumab in patients with giant-cell tumor of bone: an open-label, phase 2 study. Lancet Oncol. 2010;11:275-280. 18. Chakarun CJ, Forrester DM, Gottsegen CJ, Patel DB, White EA, Matcuk GR Jr. Giant cell tumor of bone: review, mimics and new developments in treatment. Radiographics. 2013;33:197-211. 19. Okamoto Y, Mathew S, Daw NG, et al. Giant cell tumor of bone with pulmonary metastases. Med Pediatr Oncol. 2003;41:454-459.

20. Dominkus M, Ruggieri P, Bertoni F, et al. Histologically verified lung metastases in benign giant cell tumors: 14 cases from a single institution. Int Orthop. 2006;30: 499-504. 21. Miller IJ, Blank A, Yin SM, McNickle A, Gray R, Gitelis S. A case of recurrent giant cell tumor of bone with malignant transformation and benign pulmonary metastases. Diagn Pathol. 2010;5:62. 22. Bertoni F, Bacchini P, Staals EL. Malignancy in giant cell tumor of bone. Cancer. 2003;97:2520-2529. 23. Mendenhall WM, Zlotecki RA, Scarborough MT, Gibbs CP, Mendenhall NP. Giant cell tumor of bone. Skeletal Radiol. 2007;36:973-978. 24. Nahal A, Ajlan A, Alcindor T, Turcotte R. Dedifferentiated giant cell tumour of bone in the form of low-grade fibroblastic osteogenic sarcoma case report of a unique presentation with follow-up. Curr Oncol. 2010;17(4):71-76. 25. Anchan C. Giant cell tumor of bone with secondary aneurysmal bone cyst. Int J Shoulder Surg. 2008;2(3):68. 26. Kiatisevi P, Thanakit V, Boonthathip M, Sukanthanak B, Witoonchart K. Giant cell tumor of the distal phalanx of the biphalangeal fifth toe: a case report and review of the literature. J Foot Ankle Surg. 2011;50:598-602.

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Giant cell tumor of the distal phalanx of the great toe: a case report.

Giant cell tumors are rarely seen in the foot. They can cause a significant amount of pain and deformity due to their aggressive and recurrent nature...
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