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Primitive neuroectodermal tumors of the chest wall
Author(s) -
Stefanko Jerome,
Turnbull Alan D.,
Helson Larry,
Lieberman Philip,
Martini Nael
Publication year - 1988
Publication title -
journal of surgical oncology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.201
H-Index - 111
eISSN - 1096-9098
pISSN - 0022-4790
DOI - 10.1002/jso.2930370110
Subject(s) - medicine , radiation therapy , chemotherapy , bone marrow transplant , primitive neuroectodermal tumor , neuroectodermal tumor , surgery , neoplasm , resection , radiology , bone marrow transplantation , sarcoma , transplantation , pathology
Primitive neuroectodermal tumor (PNET) is a rare, highly malignant small‐cell neoplasm that most often arises from the chest wall or paravertebral region. A patient is described from whom resection, intensive chemotherapy, and bone marrow transplant were unsuccessful. Eleven patients were treated between 1975 and 1985; there was only one long term survivor (48 months) despite intensive multidisciplinary treatment. The median survival of 49 published cases was 9 months. Conservative resection minimizing disability, radiotherapy, and intensive chemotherapy remain valid options, but use of experimental agents as part of intial therapy seems justified. The role of autologous marrow transplant remains unclear.