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Malignant Gastrointestinal Neuroectodermal Tumor, a Rare Neoplasm, Presenting With Hemoperitoneum and Malena: A Case
Sampada Wankhede1, Debiprasad Sahoo1, Aishwarya A Meshram1
1General Surgery, Topiwala National Medical College and Bai Yamunabai Laxman Nair Charitable Hospital, Mumbai, IND.
Cureus
|November 4, 2024
Summary
Malignant gastrointestinal neuroectodermal tumors (GNET) are rare. This case highlights a GNET presenting with gastrointestinal bleeding and hemoperitoneum, initially misdiagnosed as Crohn
Area of Science:
- Gastroenterology
- Surgical Oncology
- Pathology
Background:
- Malignant gastrointestinal neuroectodermal tumors (GNET) are rare mesenchymal neoplasms.
- GNET can manifest with diverse symptoms, including abdominal pain and bowel obstruction.
- Early diagnosis and treatment are crucial for managing GNET.
Observation:
- A patient presented with gastrointestinal bleeding and hemoperitoneum, a rare GNET manifestation.
- Initial misdiagnosis as Crohn's disease complicated the diagnostic process.
- Development of melena and ascites prompted further investigation.
Findings:
- Exploratory laparotomy revealed a small intestinal mass with hemoperitoneum.
- Histopathology and immunohistochemistry confirmed GNET (S100+, synaptophysin+).
- Molecular FISH analysis identified an EWSR1 gene translocation, confirming GNET.
Implications:
- This case underscores the importance of considering rare diagnoses in non-responsive cases.
- Accurate diagnosis of GNET is essential for appropriate treatment.
- Surgical resection is a viable treatment option for localized GNET.
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