Peripheral neuroectodermal tumors
Springer Online Journal Archives 1860-2000
Abstract Two cases of Askin's tumor of the thoracopulmonary region in children (a 6-year-old female and a 5-year-old male) are presented. Primary surgery was unfeasible and despite aggressive chemotherapy both died within 304 and 300 days, respectively, from the onset of the disease. Although rare, Askin's tumor should be considered in the differential diagnosis of any thoracopulmonary mass in childhood and adolescence through appropriate histology and immunohistochemistry of incisional biopsies. Its prognosis is poor unless radical surgery is performed as soon as possible: unresectable tumors can be treated with chemotherapy, but prolonged chemotherapy yields potentially lethal complications. We conclude that only early diagnosis and cytogenetic recognition can give children with Askin's tumor a chance of curative surgery.
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