January 2021: An incidento-cyst in the pancreas

Clinical history: A 50-year-old female was found to have an incidental solitary multi-septated cyst in the pancreatic neck. There was enhancement of the septa of the cyst on CT imaging. Endoscopic ultrasound showed an echogenic central area. The cyst does not communicate with the pancreatic ductal system. Fine needle aspiration of the lesion was performed and 1ml of clear watery fluid was aspirated.

  • Smears were paucicellular  
  • Rare epithelial aggregates in close association with fibrous stromal tissue fragments (see picture below) 
    • Small round uniform nuclei
    • Fine and evenly distributed chromatin
    • Clear cytoplasm with distinct cell borders
    • No nuclear atypia or mitoses

DDx for cystic pancreatic lesions on imaging:

(Here are the more commonly seen cystic pancreatic lesions)

  • Pseudocyst (usually no epithelium; some inflammatory cells; history of pancreatitis)
  • Serous microcystic adenoma (scant watery fluid aspirated; usually hypocellular smears)
  • Neoplastic mucinous cyst (e.g. Intraductal papillary mucinous neoplasm; Mucinous cystic neoplasm – thick viscous mucin; IPMN connected to pancreatic ductal system)
  • Neuroendocrine tumour with cystic change 

A concurrent core biopsy was also performed.

  • Cystic spaces of vacious sizes separated by relatively hypocellular fibrovascular septa
  • Lined by a single layer of cuboidal epithelium with clear cytoplasm
  • No significant nuclear atypia

The presence of intracytoplasmic glycogen was confirmed by a positive PAS stain and negative D-PAS stain:

The lesional cells were positive for inhibin-A:

Diagnosis: Serous microcystic adenoma

  • A benign cystic epithelial neoplasm, presumed to originate from a centroacinar cell / intercalated duct system
  • It has a female predominance, often an incidental finding and can be situated anywhere in the pancreas
  • An associated somatic VHL gene mutation is often detected in sporadic cases; Von Hippel-Lindau syndrome (germline VHL gene mutation) is associated with multifocality
  • On EUS imaging, there may be a 'honeycomb' appearance, and sometimes a central scar +/- calcifications. There is no connection with the pancreatic ductal system. 
  • The diagnosis of serous microcystic adenoma is tricky on fine needle aspiration cytology; the presence of a paucicellular, non-mucinous background should trigger a search for lesional epithelium with associated fibrous tissue fragments.
  • The epithelial cells are arranged in honeycombed sheets and contain bland, round, central nuclei with distinct cell borders and clear cytoplasm.
    • The lesional cells may be confused with
      • Benign gastrointestinal epithelium
        • Large fragments of epithelium with associated mucin
      • Mucinous epithelium
        • More abundant, mucin-containing cytoplasm
        • Variable nuclear atypia
  • Cyst fluid analysis shows low CEA levels (typically <5ng/ml); and wild-type KRAS   

Case writer: Dr Noel Chia

 

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