Rhabdomyosarcoma of thumb: A case report with review of literature

ABSTRACT

rhabdomyosarcoma is a rare malignant tumour occurring in the hand. though the prognosis for this tumour is poor, when it occurs in the hand, early diagnosis is possible, since the swelling becomes clinically obvious. radical surgical excision even at the cost of significant functional loss is recommended with possible reconstruction once the disease is under control. advances in chemotherapy protocols and radiotherapy in selected cases has improved the prognosis. Rhabdomyosarcoma arising in the thumb of a four-year-old child is presented. he was treated by amputation of the thumb at the carpometacarpal joint level followed by chemotherapy. when he was disease-free at 18 months, thumb was reconstructed by pollicisation of the index finger. the child has remained recurrence-free at 30 months from diagnosis. the patient details are presented with review of the relevant literature.

KEY WORDS

hand tumour - pollicisation - rhabdomyosarcoma

• 1 Young Jr JL, Ries LG, Silverberg E, Horm JW, Miller RW. Cancer incidence, survival and mortality for children younger than age 15 years. Cancer 1986; 58: 598-602
• 2 Potenza AD, Winslow DJ. Rhabdomyosarcoma of the hand. J Bone Joint Surg Am 1961; 43: 700-8
• 3 Murray PM. Soft tissue sarcoma of the upper extremity. Hand Clin 2004; 20: 325-33
• 4 Lawrence Jr W, Anderson JR, Gehan EA, Maurer H. Pretreatment TNM staging of childhood rhabdomyosarcoma: A report of intergroup rhabdomyosarcoma study group. Cancer 1997; 80: 1165-70
• 5 Hare HF, Cerny Jr MJ. Soft tissue sarcoma: A review of 200 . Cancer 1963; 16: 1332-7
• 6 Linscheid RL, Soule EH, Henderson ED. Pleomorphic Rhabdomyosarcomata of the extremities and limb girdles: A clinicopathological study. J Bone Joint Surg Am 1965; 47: 715-26
• 7 Xarchas K, Papavassiliou N, Tsoutseos N, Burke FD. Rhabdomyosarcoma of the hand: Two case reports and a review of the literature. J Hand Surg Br 1996; 21: 325-9
• 8 Cohen M, Ghosh L, Schafer ME. Congenital embryonal rhabdomyosarcoma of the hand and Apert’s syndrome. J Hand Surg Am 1987; 12: 614-7
• 9 Siegel HJ, Connor GS, Lee D, Lopez-Ben R, Kelly DR. Synchronous bifocal alveolar rhabdomyosarcoma: A case report. J Bone Joint Surg Br 2006; 88: 955-8
• 10 Sorenson PH, Lynch JC, Qualman SJ, Tirabosco R, Lim JF, Maurer HM. et al. PAX3-FKHR and PAX7-FKHR gene fusions are prognostic indicators in alveolar rhabdomyosarcoma: A report from the children’s oncology group. J Clin Oncol 2002; 20: 2672-9
• 11 Fleegler EJ. An approach to soft tissue sarcomas of the hand and upper limb. J Hand Surg Br 1994; 19: 411-9
• 12 Pappo AS, Shapiro DN, Crist WM. Rhabdomyosarcoma. Biology and treatment. Pediatr Clin North Am 1997; 44: 953-72