Outcome analysis of patients with transformed teratoma to primitive neuroectodermal tumor

Y. Ehrlich, S. D W Beck, Thomas Ulbright, Liang Cheng, M. J. Brames, M. Andreoiu, Richard Foster, Lawrence Einhorn

Research output: Contribution to journalArticle

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Abstract

Background: The emergence of a primitive neuroectodermal tumor (PNET) within a germ-cell tumor (GCT) is rare. We assess the prognosis and response to treatment. Patients and methods: Eighty-one patients were identified. Selected patients were treated with cyclophosphamide 1200 mg/m2, doxorubicin 75 mg/m2, and vincristine 2 mg i.v. alternating with ifosfamide 1.8 g/m2 × 5 days plus etoposide 100 mg/m2 × 5 days (CAV/IE). Ewing's sarcoma (EWS) translocation was assessed using a FISH-based method. Results: Median follow-up was 41 months. Seventy-six patients had PNET in the primary tumor or in initial metastasis. Five harbored PNET only at relapse. Twenty-six of 76 underwent primary retroperitoneal lymph node dissection, 13 of whom had retroperitoneal PNET and four are dead of disease (DOD). Fifty of 76 were initially treated with GCT chemotherapy (n = 49) or CAV/IE (n = 1). Twenty-seven of these 50 underwent complete postchemotherapy resection of residual PNET and 17 are DOD. Ten patients received CAV/IE. Eight achieved an objective response, and five are currently alive. One of the 14 specimens examined carried the EWS translocation. Conclusions: PNET of GCT origin is associated with an adverse outcome. For low-volume disease, surgery is the optimal initial therapy. CAV/IE may have a role in patients with unresectable disease.

Original languageEnglish
Pages (from-to)1846-1850
Number of pages5
JournalAnnals of Oncology
Volume21
Issue number9
DOIs
StatePublished - Mar 15 2010

Fingerprint

Primitive Neuroectodermal Tumors
Teratoma
Germ Cell and Embryonal Neoplasms
Ewing's Sarcoma
Ifosfamide
Vincristine
Etoposide
Lymph Node Excision
Doxorubicin
Cyclophosphamide
Neoplasm Metastasis
Recurrence
Drug Therapy
Therapeutics
Neoplasms

Keywords

  • Primitive neuroectodermal tumor
  • Teratoma
  • Testicular neoplasia

ASJC Scopus subject areas

  • Oncology
  • Hematology

Cite this

Outcome analysis of patients with transformed teratoma to primitive neuroectodermal tumor. / Ehrlich, Y.; Beck, S. D W; Ulbright, Thomas; Cheng, Liang; Brames, M. J.; Andreoiu, M.; Foster, Richard; Einhorn, Lawrence.

In: Annals of Oncology, Vol. 21, No. 9, 15.03.2010, p. 1846-1850.

Research output: Contribution to journalArticle

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