Extracranial primitive neuroectodermal tumors. The memorial sloan-kettering cancer center experience

✍ Scribed by Brian H. Kushner; Steven I. Hajdu; Subhash C. Gulati; Robert A. Erlandson; Philip R. Exelby; Philip H. Lieberman

• Publisher: John Wiley and Sons
• Year: 1991
• Tongue: English
• Weight: 492 KB
• Volume: 67
• Category: Article
• ISSN: 0008-543X
• DOI: 10.1002/1097-0142(19910401)67:7<1825::aid-cncr2820670702>3.0.co;2-3

✦ Synopsis

The clinical data of 54 patients (57% males) with extracranial primitive neuroectodermal tumors (PNET) seen over a 20-year period at Memorial Sloan-Kettering Cancer Center were reviewed. The age at diagnosis ranged from 1 month to 81 years (median, 17 years). One PNET arose in a previously irradiated site. One patient had an unaffected identical twin. Primary sites were thoracopulmonary (n = 251, pelvis (n = 12), retroperitoneum or abdomen (n = lo), limbs (n = 5), neck (n = 11, and unknown (n = 1). At diagnosis, epidural extension was present in 13 patients with truncal primaries, and 11 patients had distant metastases. All of the latter died with disease. Progression-free survival (PFS) among the 43 patients with localized tumors (all >5 cm) was 25% at 24 months. Two of 13 patients who relapsed after more than 12 months without therapy were long-term survivors. Patients with localized PNET who had resection of all gross disease within 3 months of diagnosis had a significantly longer PFS (P = 0.0003). Radiation therapy caused tumor shrinkage but was not curative of measurable disease. A doseresponse effect was evident with the most commonly used drug, cyclophosphamide. Myeloablative regimens using melphalan (n = 8) or thiotepa (n = 1) with autologous bone marrow rescue were not clearly beneficial. The treatment results favor: (1) early surgical removal, (2) dose-intensive use of drugs active against PNET (especially cyclophosphamide), and (3) radiation therapy to ablate residual microscopic disease. Cancer 67:1825-1829,1991. XTRACRANIAL PRIMITIVE NEUROECTODERMAL TU-E MORS (PNET) are small round cell malignancies of presumed neural crest origin arising outside the central and sympathetic nervous systems. I Other names for PNET are peripheral neuroepithelioma, Askin tumor, and peripheral or adult neuroblastoma. Histopathology may be