Malignant Gastrointestinal Neuroectodermal Tumor Presenting With Recurrent Ascites

Abstract Gastrointestinal neuroectodermal tumors (GNET) are rare and few cases exist in literature. Most common amongst middle-aged and young adults, this tumor metastasizes rapidly. Due to its rarity and poor prognosis, there is limited data on staging and treatment. However, there are common histological and morphological patterns amongst these tumors that can help diagnose them earlier and lead to prompt therapy options. GNET mimics several other tumors and can present as a diagnostic challenge to clinicians and pathologists alike. We present a case of a 39-year-old female who developed new onset ascites and an unusual presentation of a highly aggressive malignant gastrointestinal neuroectodermal tumor with widespread metastasis. Our patient deteriorated rapidly during her admission with multi-organ failure and passed shortly after presentation despite treatment with chemotherapy. This case emphasizes the manifestations of an uncommon malignancy and the diagnostic difficulty it can cause especially in a metastatic setting.