The Journal of Foot & Ankle Surgery xxx (2015) 1–4

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Case Reports and Series

Giant Cell Tumor of the Distal Phalanx of the Fourth Toe: A Case Report Masahiro Yokouchi, MD, PhD 1, Yoshiya Arishima, MD, PhD 2, Satoshi Nagano, MD, PhD 3, Hirofumi Shimada, MD 2, Shunsuke Nakamura, MD, PhD 2, Takao Setoguchi, MD, PhD 4, Ichiro Kawamura, MD, PhD 2, Yasuhiro Ishidou, MD, PhD 5, Setsuro Komiya, MD, PhD 6 1

Associate Professor, Department of Orthopaedic Surgery, Graduate School of Medical and Dental Sciences, Kagoshima University, Kagoshima, Japan Orthopedist, Department of Orthopaedic Surgery, Graduate School of Medical and Dental Sciences, Kagoshima University, Kagoshima, Japan Lecturer, Department of Orthopaedic Surgery, Graduate School of Medical and Dental Sciences, Kagoshima University, Kagoshima, Japan 4 Associate Professor, The Near-Future Locomotor Organ Medicine Creation Course, Graduate School of Medical and Dental Sciences, Kagoshima University, Kagoshima, Japan 5 Associate Professor, Department of Medical Joint Materials, Graduate School of Medical and Dental Sciences, Kagoshima University, Kagoshima, Japan 6 Professor, Department of Orthopaedic Surgery, Graduate School of Medical and Dental Sciences, Kagoshima University, Kagoshima, Japan 2 3

a r t i c l e i n f o

a b s t r a c t

Level of Clinical Evidence: 4

Giant cell tumor of the bone is a benign, but locally aggressive, primary bone tumor of unknown origin. It most commonly occurs in the long bones and is only rarely found in the phalangeal bones, such as the distal phalanx of the foot. In our review of English-language published studies, only 4 other cases of giant cell tumor involving the distal phalangeal bone of the foot had been reported to date. We report a case of giant cell tumor arising in the distal phalanx of the fourth toe in a 28-year-old female. Although bisphosphonate therapy was administered, the tumor showed highly aggressive behavior with ulceration of the overlying skin, and the patient underwent phalangeal amputation 1.5 months after diagnosis. Ó 2015 by the American College of Foot and Ankle Surgeons. All rights reserved.

Keywords: amputation bisphosphonate bone tumor foot skin ulceration

Giant cell tumor (GCT) is a relatively common benign bone tumor that accounts for 5% of all primary bone tumors and almost 20% of benign primary bone tumors (1). The tumor most frequently occurs in the metaphyseal region of the long bones, in particular, the distal femur, proximal tibia, distal radius, and proximal humerus. Its occurrence in the small bones is very rare (1,2). GCT of the small bones is characterized by extensive bony destruction and displays more aggressive behavior, with a greater recurrence rate, than that of GCT involving the ends of the long bones (2). Although the mainstay of treatment of GCT of the long bones is surgery, including en bloc or wide resection or curettage with adjuvant therapy, no consensus has been reached regarding the ideal treatment of GCT located in the small bones owing to the rarity of this condition (2). Among the cases of small bone GCT, GCT of the distal phalanx of the foot is particularly rare. Our review of published English-language studies to date yielded only 4 reported cases of GCTs involving the distal

Financial Disclosure: M. Yokouchi was supported by a Grant-in-Aid for Scientific Research (grant 25462343). Conflict of Interest: None reported. Address correspondence to: Masahiro Yokouchi, MD, PhD, Department of Orthopaedic Surgery, Graduate School of Medical and Dental Sciences, Kagoshima University, 8-35-1 Sakuragaoka, Kagoshima 890-8520, Japan. E-mail address: [email protected] (M. Yokouchi).

phalanx of the foot (3–6). In the present report, we describe a case of GCT located in the distal phalanx of the foot in a patient who was initially treated with conservative bisphosphonate therapy. To the best of our knowledge, no isolated case reports of phalangeal bone GCT treated with bisphosphonates have been previously reported. Although several reports have demonstrated the efficacy of bisphosphonate therapy in the treatment of GCT (7,8), no response to bisphosphonate therapy was observed in our patient. The tumor grew rapidly, completely displacing the distal phalanx, and causing ulceration of the overlying skin; therefore, the patient underwent phalangeal amputation. The present case report illustrates a pathologic rarity and incorporates a brief review of the published data relevant to surgeons. Case Report A previously healthy 28-year-old female visited a local hospital because of increasing pain and swelling in the distal part of her fourth toe that she had first experienced 1 month previously. She had no history of trauma to this area. The patient was diagnosed with a bone tumor of the distal phalanx of the fourth toe from the radiographic findings and was subsequently referred to our hospital. At her first visit to our hospital, an enlarged fourth toe and toenail were observed, with severe tenderness in the distal part of

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.2014.09.023

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Fig. 1. (A) Clinical photograph showing enlargement of the toe and toenail in the distal part of the patient’s fourth toe. (B) Anteroposterior radiograph of the distal phalanx of the fourth toe at initial presentation showing osteolysis with cortical destruction.

the fourth toe (Fig. 1A). The overlying skin was smooth and nonadherent, and no redness or warmth was present in the area. The laboratory test results were all within the normal range. Radiographs showed osteolysis with cortical destruction in the region of the distal phalanx of the fourth toe (Fig. 1B). No calcification or periosteal reaction was observed. A tissue biopsy was performed, revealing round to oval mononuclear cells with numerous osteoclast-like giant cells, leading to the diagnosis of GCT of the bone (1). The patient did not agree to radical surgical treatment

such as en bloc resection; thus, conservative bisphosphonate therapy was initially administered (1 cycle of 4 mg of zoledronic acid). However, the tumor did not show any response to the chemotherapy and began to show highly aggressive behavior with skin ulcerations forming (Fig. 2A). Radiographs and magnetic resonance imaging revealed complete displacement of the distal phalanx by the tumor (Figs. 2B and 3A). The patient underwent phalangeal amputation only 1.5 months after the diagnosis (Fig. 3B). Histopathologic analysis of the resected specimen (Fig. 3C) confirmed the

Fig. 2. (A) Clinical photograph showing skin ulceration associated with the tumor (arrow). (B) Anteroposterior radiographs of the distal phalanx of the fourth toe 1 month after bisphosphonate therapy showing rapid progression of the tumor with cortical destruction.

M. Yokouchi et al. / The Journal of Foot & Ankle Surgery xxx (2015) 1–4

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Discussion

Fig. 3. (A) Sagittal T1-weighted magnetic resonance imaging scan revealing complete displacement of the distal phalanx by the tumor. (B) Gross appearance of the resected tissue showing a gelatinous, red-yellowish tumor with bleeding. (C) Microscopic examination revealing round to oval mononuclear cells, with numerous osteoclast-like giant cells and without fibrosis. Neither spindle-shaped stromal cell proliferation around the hemorrhagic area nor phagocytosed hemosiderin was observed in the lesions. The nuclei of the giant cells were similar to those of the mononuclear cells (hematoxylin-eosin stain, original magnification 400).

diagnosis of GCT suggested by the previous biopsy. The patient’s postoperative course was uneventful, and no recurrence or metastasis was observed during a 7-year follow-up period.

GCT of the small bones is rare. It has been demonstrated to occur in younger patients, and these GCTs have been associated with a greater risk of local recurrence compared with those located at the ends of the long bones (2). Therefore, when this disease involves the small bones in young patients, surgery will be required for good long-term functional outcomes and to avoid local recurrence after treatment. However, the surgical experience has been limited, owing to the rarity of the disease (2). GCT of the phalangeal bone of the foot is especially rare, with only approximately 10 cases reported to date. Detailed clinical information for the reported cases of GCT involving the distal phalanx of the foot is provided in the Table. Because of the high recurrence rate associated with GCT of the small bones, Fujisawa et al (3) recommended amputation or complete block resection of the tumor as the primary surgical treatment of GCT of the phalanx. Alvarez Ramos et al (9) also recommended en bloc resection of the tumor, but not curettage, for the treatment of GCT located in the phalangeal bone. However, Oliveira et al (2) analyzed 91 cases of GCT of the small bones and reported that the recurrence rate after curettage alone, curettage with adjuvant therapy, resection, and amputation was 72%, 13%, 15%, and 10%, respectively. From that analysis, the investigators identified curettage with adjuvant therapy as the recommended treatment of both primary and recurrent GCT of the small bone. Among the 4 reported cases of GCT involving the distal phalanx of the foot, 2 underwent curettage, 1 underwent resection, and 1 underwent amputation (Table). However, no recurrence or metastasis was observed in any of these cases. Thus, the surgical management of this rare condition remains controversial. Recently, several reports have described the successful treatment of GCT of the bone with bisphosphonate therapy (7,8). In the present case, we first recommended en bloc resection of the tumor and distal phalanx, because we judged the cortex of the lesion to be very thin and believed that curettage would not have afforded complete tumor removal. However, the patient did not consent to radical surgical treatment. Therefore, bisphosphonate was administered to the patient as conservative medical management, with the goal of preserving the phalanx and preventing local spread. The tumor did not show any response to the chemotherapy but rather grew rapidly. No other isolated case reports describing bisphosphonate therapy for GCT of the phalangeal bone have been previously reported. Although medical therapy with bisphosphonates and also with denosumab can alter the course of GCTs of the long bones (7), the efficacy of bisphosphonate therapy for GCT of the small bones requires clarification. Although GCT of the small bones characteristically behaves more aggressively than that of the long bones, it is very rare for a benign tumor to cause skin disintegration. In general, skin ulceration by a tumor is presumed to occur from enlargement of the neoplasm, trauma to the skin, and other factors such as infection or a poor nutritional condition (10). In our patient, the distal phalangeal bone was completely displaced by the tumor, and the tumor grew rapidly under the skin. Because the patient kept

Table Reports of giant cell tumor occurring in distal phalanx of foot Year

Investigator

Patient age (y)

Location

Treatment

Recurrence

Metastasis

Follow-up Period

2006 2011 2011 2014 2014

Fujisawa et al (3) Kiatisevi et al (4) Bachhal et al (5) Dellenbaugh et al (6) Present study

40 13 27 39 28

Second toe Fifth toe Great toe Great toe Fourth toe

Amputation Curettage and bone graft Resection Curettage and ablation Bisphosphonate followed by amputation

No No No No No

No No No No No

d 25 mo 24 mo 6 mo 7y

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walking (without weightbearing) during the conservative therapy, the tumor might have broken through the skin owing to microtrauma and/or repeated trauma. Although GCT is a benign tumor, we must undertake proper precautions and perform careful observation of such tumors arising in the distal phalanx to protect against ulceration. The differential diagnosis for this rare condition includes all diseases that cause expansion or distraction in the cortex, along with areas of bone destruction. In particular, it is important to differentiate giant cell reparative granuloma from GCT in terms of the clinical, radiologic, and histologic appearance (3–6). Histologically, giant cell reparative granuloma will be characterized by clustered giant cells separated by fibrous stromal tissue around the hemorrhagic area and phagocytosed hemosiderin (3,4). In our patient, microscopic examination revealed round to oval mononuclear cells with numerous osteoclast-like giant cells and without fibrosis. We did not observe spindle-shaped stromal cell proliferation around the hemorrhagic area or phagocytosed hemosiderin in the lesions. The nuclei of the giant cells were similar to those of the mononuclear cells. From these findings, we diagnosed the lesion as GCT of the bone. In conclusion, we have reported a rare case of a patient with GCT of the distal phalanx who initially received conservative medical therapy with bisphosphonate before undergoing phalangeal amputation. No standard treatment of GCT of the distal phalanx has been determined.

Therefore, treatment should be guided by consideration of an individual patient’s overall condition.

References 1. Fletcher CDM, Unni KK, Mertens F, eds. World Health Organization Classification of Tumours: Pathology and Genetics of Tumours of Soft Tissue and Bone. IARC Press, Lyon, France, 2002. 2. Oliveira VC, van der Heijden L, van der Geest IC, Campanacci DA, Gibbons CL, van de Sande MA, Dijkstra PD. Giant cell tumours of the small bones of the hands and feet: long-term results of 30 patients and a systematic literature review. Bone Joint J 95:838–845, 2013. 3. Fujisawa Y, Takahashi T, Kawachi Y, Otsuka F. Giant cell tumor of the distal phalanx of the foot. Eur J Dermatol 16:204–205, 2006. 4. 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 50:598–602, 2011. 5. Bachhal V, Rangdal S, Saini U, Sament R. Giant cell tumor of distal phalanx of great toe: a case report. Foot 21:198–200, 2011. 6. Dellenbaugh SG, Wilkinson L, Aydogan U. Giant cell tumor of the distal phalanx of the great toe: a case report. Foot Ankle Spec 7:237–241, 2014. 7. Raskin KA, Schwab JH, Mankin HJ, Springfield DS, Hornicek FJ. Giant cell tumor of bone. J Am Acad Orthop Surg 21:118–126, 2013. 8. Cornelis F, Truchetet ME, Amoretti N, Verdier D, Fournier C, Pillet O, Gille O, Hauger O. Bisphosphonate therapy for unresectable symptomatic benign bone tumors: a long-term prospective study of tolerance and efficacy. Bone 58:11–16, 2014. 9. Alvarez Ramos A, Valverde Garcıa JA, Garcıa Alonso MG. Giant cell tumor of the foot phalanges in children: a case report. J Pediatr Orthop B 8:132–135, 1999. 10. Adderley UJ, Holt IG. Topical agents and dressings for fungating wounds. Cochrane Database Syst Rev (5):CD003948, 2014.

Giant Cell Tumor of the Distal Phalanx of the Fourth Toe: A Case Report.

Giant cell tumor of the bone is a benign, but locally aggressive, primary bone tumor of unknown origin. It most commonly occurs in the long bones and ...
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