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Squamous-Lined Cyst of Pancreas with unusual Plexiform Neuronal Hyperplasia: A Case Report and Review of the Literature

Aoife J McCarthy, Runjan Chetty

A 61-year old female presented with a unilocular cyst in the tail of the pancreas. The cyst was lined partly by squamous and lowcuboidal epithelium and did not communicate with the pancreatic ductal system. Much of the lining was denuded with underlying mixed inflammation and extensive fibrosis. No ovarian stroma was identified despite extensive sampling and immunohistochemical staining. An additional curious finding was that of extensive neuronal hyperplasia surrounding the cyst wall. It consisted of nerves bundles of varying size and had a distinct plexiform pattern. The patient did not have a history or the clinical stigmata of neurofibromatosis. This is an unusual case of a primary squamous or epidermoid cyst of the pancreas with associated reactive plexiform neuronal hyperplasia.