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Gastric Ewing sarcoma/primitive neuroectodermal tumor: A case report.

Makoto Inoue¹, Toshifumi Wakai, Pavel V Korita

¹Division of Digestive and General Surgery, Niigata University Graduate School of Medical and Dental Sciences, Chuo-ku, Niigata 951-8510, Japan.

Oncology Letters

|August 7, 2012

Summary

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Gastric Ewing sarcoma/primitive neuroectodermal tumors (ES/PNETs) are rare, often presenting aggressively. This case highlights the poor prognosis and need for multimodal treatment in gastric ES/PNET patients.

Area of Science:

Oncology
Gastroenterology
Pathology

Background:

Ewing sarcoma/primitive neuroectodermal tumors (ES/PNETs) typically arise in bone or soft tissue.
Gastric origin of ES/PNETs is exceptionally rare, with only four prior English-language reports.
This study details a unique case of gastric ES/PNET in a 41-year-old woman.

Purpose of the Study:

To report a rare case of gastric ES/PNET.
To characterize the tumor's immunophenotype and genetic profile.
To discuss treatment strategies and prognosis for gastric ES/PNET.

Main Methods:

Gastrectomy for primary tumor removal.
Immunohistochemistry (CD99, vimentin, CD117, S100, chromogranin A, synaptophysin).
Chromosomal karyotype and molecular analysis (RT-PCR) for EWS-FLI1 translocation t(11;22).

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Main Results:

The gastric tumor exhibited positive immunoreactivity for markers including CD99, CD117, and neuroendocrine markers.
The characteristic EWS-FLI1 fusion gene translocation t(11;22)(q24;q12) was identified.
The patient experienced disease recurrence and succumbed to progressive disease 110 months post-surgery.

Conclusions:

Gastric ES/PNET is an aggressive malignancy with a high metastatic potential and unfavorable prognosis post-resection.
Multimodal treatment, including surgery, chemotherapy, and radiotherapy, may improve survival outcomes.
Further research is needed to optimize management for this rare tumor type.