Abstract Primary pancreatic leiomyosarcoma (PPL) is an exceptionally rare malignant mesenchymal neoplasm, accounting for a minute fraction of pancreatic tumors. Owing to its nonspecific clinical presentation, diagnosis is frequently delayed and often established at an advanced stage. We report the case of a 52-year-old woman who presented with progressive abdominal distention and reduced oral intake. Computed tomography revealed a large pancreatic head mass associated with extensive ascites and omental caking. Diagnostic paracentesis demonstrated malignant cells. Endoscopic ultrasound–guided fine-needle biopsy (EUS-FNB) established the diagnosis of primary pancreatic leiomyosarcoma, confirmed by histopathology and immunohistochemical positivity for smooth muscle markers. The presence of malignant ascites indicated advanced, unresectable disease. The patient was managed with systemic chemotherapy. Her course was complicated by spontaneous bacterial peritonitis and multiorgan failure requiring intensive care support. This case underscores an unusual presentation of PPL with malignant ascites at diagnosis, highlights the diagnostic utility of EUS-guided core biopsy, and emphasizes the importance of considering rare pancreatic mesenchymal malignancies in atypical clinical scenarios.
Omar et al. (Thu,) studied this question.
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