A case of primitive neuroectodermal tumor in the distal phalanx

Abstract

Primitive neuroectodermal tumor of the hand is extremely rare. To our knowledge, only four cases have been reported. A 13-year-old girl presented with pain, tenderness, and swelling at the distal phalanx of her right middle finger. On physical examination, there was a tender, swollen, erythematous nodular mass, 2x1 cm in size, at the distal volar tip of the middle finger. A radiogram of the finger demonstrated a soft tissue swelling over the phalanx. Magnetic resonance imaging revealed an extra-osseous soft tissue mass without infiltration to bone. Bone scintigraphy showed increased uptake only in the distal phalanx. Histological examination of an incisional biopsy showed cellular tumoral tissue. The lesion was diagnosed as primitive neuroectodermal tumor based on immunohistochemical studies. Surgical treatment was performed with marginal resection of the tumor site and preservation of the digital artery and nerve. Two weeks after surgery, combined chemotherapy was administered at eight cycles over a time period of six months. Forty-two months after surgery, the patient was free of tumor. She had normal function of the hand with full strength.