A distinctive myoepithelial hamartoma of the pancreas histologically confirmed in the mother of a previously reported patient.

We encountered a 62-year-old female patient with a distinctive pancreatic myoepithelial hamartoma characterized by dilated loops formed by pancreatic branch ducts. The patient, who experienced recurrent acute pancreatitis caused by pancreatic juice stasis, underwent subtotal stomach-preserving pancreatoduodenectomy, achieving remission of pancreatitis. Computed tomography (CT) and magnetic resonance cholangiopancreatography (MRCP) demonstrated a honeycomb appearance of the pancreatic head, consisting largely of loop-forming dilated pancreatic branch ducts. Radiography of resected specimens demonstrated a tortuous main pancreatic duct that narrowed in the head, but communicated with the pancreatic branch ducts forming intricate loops within the lesion. Histologic examination showed dilated pancreatic ducts embedded in thick layers of smooth muscle, leading to a diagnosis of myoepithelial hamartoma. Her son shared a similar clinical course, radiologic findings, and histopathologic findings with his mother. MRCP demonstrated a honeycomb appearance of the pancreatic head in her daughter, who complained of persistent diarrhea. To our knowledge, this is the first English-language reports of such a myoepithelial hamartoma of the pancreas showing familial occurrence.