Multifocal Small Bowel Gastrointestinal Stromal Tumors and Concurrent Neuroendocrine Tumor in Neurofibromatosis Type...

Neurofibromatosis type I (NF1) is associated with a distinct subset of gastrointestinal stromal tumors (GISTs) but is only rarely linked to neuroendocrine tumors. We describe a 61-year-old woman with NF1 who developed multifocal jejunal GISTs with a synchronous duodenal neuroendocrine tumor (an exceptionally uncommon coexistence). NF1-associated GISTs are typically multifocal, small-bowel predominant, and poorly responsive to targeted medical therapy, rendering surgical resection the primary treatment strategy. This case highlights the unique tumor biology of NF1 and emphasizes the importance of meticulous anatomic evaluation and coordinated multidisciplinary surgical planning to determine the optimal extent and approach to resection.