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Extracranial primitive neuroectodermal tumors. The Memorial Sloan-Kettering Cancer Center experience.

Abstract
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 = 25), pelvis (n = 12), retroperitoneum or abdomen (n = 10), limbs (n = 5), neck (n = 1), 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 greater than 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 dose-response 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.
AuthorsB H Kushner, S I Hajdu, S C Gulati, R A Erlandson, P R Exelby, P H Lieberman
JournalCancer (Cancer) Vol. 67 Issue 7 Pg. 1825-9 (Apr 01 1991) ISSN: 0008-543X [Print] United States
PMID1848468 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't, Research Support, U.S. Gov't, P.H.S.)
Chemical References
  • Doxorubicin
  • Cyclophosphamide
Topics
  • Adolescent
  • Adult
  • Aged
  • Aged, 80 and over
  • Antineoplastic Combined Chemotherapy Protocols (therapeutic use)
  • Child
  • Child, Preschool
  • Combined Modality Therapy
  • Cyclophosphamide (administration & dosage)
  • Doxorubicin (administration & dosage)
  • Female
  • Humans
  • Infant
  • Male
  • Middle Aged
  • Neoplasms, Germ Cell and Embryonal (diagnosis, mortality, secondary, therapy)
  • Radiotherapy Dosage
  • Remission Induction
  • Survival Rate

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