Malignant Peripheral Neuroectodermal Tumor

Schmidt, Dietmar

doi:10.1007/978-3-642-77289-4_15

Cited by 24 publications

(1 citation statement)

References 43 publications

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“…The primitive neuroectodermal tumor (peripheral neuroepithelioma, or PNET) is a prototypic small round cell tumor of childhood that is characterized by a translocation resulting in fusion of the Ewing's sarcoma gene (EWS) to the FLI1 gene in over 90% of cases [1][2][3]. Many, but not all, of the remaining cases of PNET are associated with translocations resulting in the fusion of the EWS gene to other members of the ETS family [4 -9].…”

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confidence: 99%

Primitive Neuroectodermal Tumors of the Biliary and Gastrointestinal Tracts: Clinicopathologic and Molecular Diagnostic Study of Two Cases

et al. 2001

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Primitive neuroectodermal tumor (PNET) is a prototypic malignant small round cell tumor of childhood that is characterized in most cases by t(11;22) resulting in an EWS-FLI1 gene fusion. Once thought to be uncommon, PNET now accounts for almost 20% of malignant soft tissue tumors in children. Increased recognition of PNET is partly due to advances in immunohistochemistry and molecular diagnostics, which have led to the identification of the tumor in non-classical sites. We report the clinical, histologic, immunohistochemical, and molecular findings of two visceral PNETs of the digestive system--one involving the small intestine and the other involving the hepatic duct. Histologically, each tumor was composed of malignant small cells growing in sheets, nests, and lobules; the tumor cells of both cases showed characteristic immunoreactivity for vimentin and O13 (CD99). Reverse transcription-polymerase chain reaction (RT-PCR) analysis for t(11;22) using nested primers was performed with RNA extracted from paraffin-embedded, formalin-fixed tissue and demonstrated an EWS exon 7 to FLI1 exon 5 fusion in both cases, confirmed by Southern blot hybridization and DNA sequence analysis. These results illustrate the expanded clinicopathologic profile of PNET, and demonstrate that visceral PNETs, despite their unusual sites of presentation, maintain the characteristic immunohistochemical and genetic features of PNETs at more conventional sites.

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Section: Introductionmentioning

confidence: 99%

Primitive Neuroectodermal Tumors of the Biliary and Gastrointestinal Tracts: Clinicopathologic and Molecular Diagnostic Study of Two Cases

et al. 2001

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Solide Tumoren des Kindes- und Adoleszentenalters

Harms¹,

Schmidt²

1997

Pathologie 4

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Der primitive neuroektodermale Tumor

Zimmermann

Blütters-Sawatzki

Berghäuser

et al. 1997

Chirurg

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The primitive neuroectodermal tumor is a rare soft tissue neoplasm occurring in children and young adults. It derives from a carcinogeneic alteration of pluripotent neural crest cells, caused by a balanced reciprocal translocation t(11;22) (q24;q12). Treatment of this undifferentiated, extremely malignant small cell tumor is carried out in compliance with the soft tissue trail (CWS) from the German Society of Pediatric Oncology. Biopsy-proven diagnosis is followed by primary chemotherapy, which in 95% of cases leads to remission, allowing excision of the remainder of the tumor without mutilation and avoidance of intraoperative tumor cell dissemination. After excision, irradiation of the tumor site and two further sequences of chemotherapy are performed. If PNETs of the paravertebral region cause symptoms of paralysis and immediate surgery is required, postoperative chemotherapy, a second-look operation and irradiation are mandatory. Between 1986 and 1994, in cooperation with our pediatric and radiotherapy colleagues, we treated ten patients. In four patients (median age, 14 years) the PNET originated from the chest wall, in six patients from the paravertebral and retroperitoneal region. Five patients died after 20 months on average, while the remaining five patients are in full remission after 31, 46, 50, 51 and 91 months, respectively.

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Malignant Peripheral Neuroectodermal Tumor

Cited by 24 publications

References 43 publications

Primitive Neuroectodermal Tumors of the Biliary and Gastrointestinal Tracts: Clinicopathologic and Molecular Diagnostic Study of Two Cases

Primitive Neuroectodermal Tumors of the Biliary and Gastrointestinal Tracts: Clinicopathologic and Molecular Diagnostic Study of Two Cases

Solide Tumoren des Kindes- und Adoleszentenalters

Der primitive neuroektodermale Tumor

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