Primary hemangioblastoma of the pancreas: a case report and review of the literature

Pancreatic hemangioblastoma is an exceedingly rare neoplasm, with only a handful of cases reported in the English literature to date. We present a unique case of pancreatic hemangioblastoma in a 21-year-old Chinese male, aiming to provide deeper insights into this exceptionally rare disease and its diagnostic challenges. A 21-year-old Chinese male was incidentally found to have a pancreatic mass on ultrasonography during a physical examination. An initial contrast-enhanced abdominal computed tomography (CT) scan revealed a solid mass in the pancreatic tail, suggestive of a neuroendocrine tumor, along with multiple pancreatic cysts, bilateral renal cysts, and multiple nodules in the right kidney. The patient underwent distal pancreatectomy and splenectomy. Histopathological and immunohistochemical analyses confirmed pancreatic hemangioblastoma with an atypical immunoprofile, co-expressing neuroendocrine markers and cytokeratin. At 7-month postoperative follow-up, the patient remained asymptomatic with no evidence of recurrence. Hemangioblastomas have a complex immunohistochemical profile that can mimic neuroendocrine tumors, posing diagnostic challenges. Pancreatic hemangioblastoma is rare and, in the presence of bilateral renal cysts and renal nodules, strongly suggests an underlying von Hippel-Lindau (VHL) syndrome.