Cystic lymphangioma of the pancreas

We read with interest the recent case report entitled ‘Giant cystic lymphangioma of pancreas’ in the August edition of the Journal. We recently saw a case in pathology consultation involving a 46-year-old woman who presented with left-sided abdominal pain, bloating and firmness. Physical examination indicated a large, leftsided, mildly tender intra-abdominal mass. Computed tomography (CT) demonstrated a well-circumscribed, 15-cm, predominantly cystic multiloculated mass within the tail of the pancreas. No enlarged intra-abdominal lymph nodes were seen. Exploratory laparotomy indicated a large cystic mass in the tail of the pancreas, displacing intra-abdominal organs and producing illformed adhesions to the surrounding structures. A distal pancreatectomy and splenectomy were performed. Gross examination of the distal pancreatectomy specimen showed a lobulated cystic mass with hemorrhagic contents. Microscopic sections demonstrated a multilocular cyst, lined by flattened lymphatic endothelial cells, without cytological atypia (Fig. 1). Scattered small lymphocytic aggregates were seen closely associated with the cyst wall. The multiple cysts were separated by a spindled stroma, without cytological atypia. Cyst contents included serous fluid and blood. Background pancreatic parenchyma was without significant alterations. A pathologic diagnosis of cystic lymphangioma of the pancreas was made. The patient had an uneventful recovery and was discharged on postoperative day 8, eating and ambulating well. As the authors mention, this is a rare benign neoplasm of the lymphatic system that may rarely involve the pancreas, with <100 reported cases in almost 100 years. Histologic features can be divided into three subtypes: cystic, cavernous or capillary. The cystic and cavernous types have been reported in the pancreas. A rare case has been reported to extend into the duodenal wall, causing gastrointestinal bleeding. The histopathologic differential diagnosis includes other, more common cystic lesions of the pancreas, such as simple cysts, pseudocysts, serous cystic neoplasms, mucinous cystic neoplasms, including adenocarcinoma, intraductal papillary mucinous neoplasms and cystic islet cell tumors. Although various imaging features can help, definitive diagnosis requires pathologic examination of the excised lesion.