Peripheral neuro-ectodermal tumors of the chest wall.

Peripheral neuroectodermal tumors, also called small round cell tumors, ari se from the neurons and develop outside the spinal cord and sympathetic str uctures, within the soft tissues of the chest wall, They include Askin's tu mor, Ewing's sarcoma, neuroblastoma, and, according to some authors, rhabdo myosarcoma and nonHodgkin's lymphoma, Although they are apparently accessib le, the diagnosis usually requires immunohistochemical, electron microscopy , and cytogenetic studies. A balanced t(11;22)(q24;q12) translocation is pr esent in all peripheral neuroectodermal tumors. Three patients, aged 30, 23 , and 14 years, respectively, were admitted for evaluation of a chest radio graph density apparently located within the chest wall. One patient had a p leural effusion and another vertebral involvement. The diagnosis was Askin' s tumor in two patients and Ewing's sarcoma in one. The treatment! which ha s not yet been standardized rests on surgery chemotherapy, and radiation th erapy. The place of autologous bone marrow transplantation remains to be de fined. Although the complete cure and complete remission rates are increasi ng, the overall prognosis is still poor. Of the three patients reported her ein, two received preoperative combination chemotherapy. Surgery was radica l in one case and palliative in two. Postoperative chemotherapy and externa l beam radiation therapy were used In one patient. Poor prognosis factors w ere vertebral involvement in one case and pleural involvement in another.