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Ewing’s Sarcoma of the Distal Phalanx of the Little FingerSt stephen’s hospital, delhi, india, E-mail: terrencejose{at}gmail.com Dear Sir, A 13 year-old girl presented with pain and a diffusely tender, warm, cystic swelling of the tip of the right little finger of 1-month duration. The skin over the swelling was red and indurated. Plain X-rays showed a lytic lesion in the middle phalanx (Fig 1). Chest X-ray was normal. Fine needle aspiration cytology was inconclusive, so curettage biopsy was carried out. The histology reported features characteristic of Ewing’s sarcoma. Stage AJCC grouping was IA (G1T1N0M0). Amputation at the metacarpophalangeal level was carried out to obtain 2 cm of clearance. Cyclophosphamide, Vincristine, Actinomycin and Doxorubicin were given postoperatively. Two years after surgery, there has been no local or distant recurrence.
Ewing’s sarcoma most commonly occurs in the first or second decade of life, with the youngest recorded case occurring in the distal phalanx of a 5 month-old infant, although this tumour rarely occurs in the bones of the hand and feet. Euler et al. (1990) reviewed the literature up to 1990 and found 20 cases of Ewing’s sarcoma of the hand. Three of the twenty cases reported were located in the distal phalanx. Later, Jones (1993) reported a case in the distal phalanx of the thumb and reviewed the literature and found a further nine cases involving the phalanges of the hand. Bernstein et al. (2006), reporting on 1426 patients from the European Intergroup Cooperative Ewing Sarcoma Study, recorded only 1% of primary Ewing’s sarcomas in the hand. Pain and swelling of the affected fingers were the most frequent complaints at diagnosis. Osteomyelitis may present a pattern similar to Ewing’s sarcoma on plain X-ray. Diaphyseal location suggests a Ewing’s sarcoma, as compared with the metaphyseal location more common in osteosarcoma. The European Intergroup Study identified only two histopathology parameters with prognostic significance, viz. topographical pattern and mitotic rate (Bernstein et al., 2006). Our patient had a predominant diffuse pattern, which carries a better prognosis. Management, preferably at a specialist centre by a multi-disciplinary team, has included both local control, by either surgery, radiation or a combination of these, and systemic chemotherapy. Chemotherapy has included cyclical combinations of vincristine, doxorubicin, cyclophosphamide, etoposide, ifosfamide and, occasionally, actinomycin D. Topotecan in combination with cyclophosphamide produced responses in approximately 35% of patients with recurrent Ewing’s sarcoma (Bernstein et al., 2006). Modern combinations of treatment are possibly curative due to the tumour’s surgical accessibility and apparent restricted involvement of only tubular bone (Euler et al., 1990). With small soft tissue and bone sarcomas in the extremities, local control by surgery has been better than with primary radiotherapy (Euler et al., 1990; Jones, 1993). Therefore, for these distal phalangeal lesions, surgery would seem appropriate and adequate proximal amputation should always achieve wide clearance. Whether only marginal or wide resection is possible, even if the later is preferable, surgery should be performed. Patients with hand lesions are recorded to have survived for more than 41 months and European Intergroup Study data showed a 68% overall 3-year survival rates in patients with distal extremity lesions. At initial diagnosis, approximately 25% of Ewing’s sarcoma patients present with clinically detectable metastases in the lung and/or in bone and/or in bone marrow. Solitary or circumscribed bony metastases should be irradiated to doses of 40 to 50 Gy, in addition to local therapy to the primary site and Ewing’s sarcoma-directed chemotherapy. Bilateral pulmonary irradiation has been reported to improve the outcome of patients with pulmonary disease. However, the survival rates of patients with multiple bony metastases are reported to be below 20% (Bernstein et al., 2006).
Euler E, Wilhelm K, Permanetter W et al Ewing’s-sarcoma of the hand—localization and treatment Journal of Hand Surgery A 1990 15 659 662.[Medline] [Order article via Infotrieve]Jones MW Ewing’s sarcoma of the thumb Journal of Hand Surgery B 1993 18 356 357.Bernstein M, Kovar H, Paulussen M et al Ewing’s sarcoma family of tumors- current management Oncologist 2006 11 503 519.
Journal of Hand Surgery (European Volume), Vol. 33, No. 1,
81-82 (2008)
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