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Primitive Neuroectodermal Tumor of the Stomach: A Case Report
Min Jeong Song1, Soyeon An1, Seung Soo Lee1
1University of Ulsan Collage of Medicine, Asan Medical Center, Seoul, Republic of Korea.
International Journal of Surgical Pathology
|March 24, 2016
Summary
Ewing sarcoma/primitive neuroectodermal tumor (ES/PNET) is a rare gastric tumor. This case highlights the importance of considering ES/PNET in stomach small round cell tumor diagnoses.
Area of Science:
- Oncology
- Pathology
Background:
- Ewing sarcoma/primitive neuroectodermal tumor (ES/PNET) is a rare, aggressive small round cell neoplasm typically affecting bone and soft tissues in younger individuals.
- Gastric ES/PNET is exceptionally uncommon, posing diagnostic challenges due to its rarity and potential resemblance to other small round cell malignancies.
Observation:
- A 55-year-old male presented with gastrointestinal bleeding (melena) and anemia, necessitating endoscopic evaluation.
- Endoscopy revealed an ulcerofungating mass in the upper stomach, leading to a total gastrectomy for definitive treatment.
Findings:
- Histopathological examination identified a small round cell tumor with features including scanty cytoplasm, inconspicuous nucleoli, perivascular pseudorosettes, and frequent multinucleated giant cells.
- Immunohistochemistry showed strong expression of CD99, FLI1, and chromogranin, with weak synaptophysin and CD56 positivity.
- Molecular analysis confirmed the presence of the EWS-FLI1 fusion transcript via reverse transcription-polymerase chain reaction.
Implications:
- The distinct immunophenotype and genetic profile confirmed the diagnosis of gastric ES/PNET.
- This case underscores the necessity of including ES/PNET in the differential diagnosis of gastric small round cell tumors to avoid misdiagnosis, particularly with small cell carcinoma.
- Accurate diagnosis is crucial for appropriate management and prognosis of this rare gastric malignancy.