Pancreatic lymphangioma: two case reports from an institutional experience with a rare entity and review of literature

Abstract

Pancreatic lymphangiomas are very rare benign cystic lesions that pose a diagnostic dilemma due to their resemblance to other non-neoplastic and neoplastic pancreatic cystic lesions. As such, pancreatic lymphangiomas are frequently diagnosed only after histological examination of the excised lesion. We present 2 cases of pancreatic lymphangioma, determine its prevalence at our institution and perform a detailed review of published literature since 2010. Case 1 is a 36-year-old male and case 2 is a 35-year-old female, both of which presented with abdominal pain. These were the only cases of pancreatic lymphangiomas reported at our institution since 2010. We reviewed 69 cases of pancreatic lymphangiomas from 52 publications. It affects predominantly females with a median age of 43 and such patients typically present with abdominal pain (58.8%) or are asymptomatic (27.9%). The median size is 8.6 cm, the most common location is the head of pancreas and the most common imaging finding is that of a multilocular cyst. Majority of patients underwent surgical resection (69.6%). Endoscopic ultrasound-guided fine-needle aspiration features of pancreatic lymphangiomas include chylous cyst fluid, elevated fluid triglyceride levels (15/16 cases) and numerous lymphocytes on cytology. The majority of patients with elevated fluid triglyceride levels (13/15 cases) were managed conservatively. Pancreatic lymphangiomas are rare pancreatic cystic lesions that may be diagnosed preoperatively using a multidisciplinary and multimodal approach involving clinical, radiological, biochemical and cytological features, allowing greater confidence in the selection of patients who can be managed conservatively.