Primary epithelial-myoepithelial carcinoma of the pituitary gland [Neurosurgery Wiki]

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====== Primary epithelial-myoepithelial carcinoma of the pituitary gland ======

Primary [[salivary gland-like tumor]]s of the [[sella]] are rare and often challenging to diagnose. They reportedly derive from [[serous]] and [[mucinous]] glands that remain trapped in the [[infundibulum]] during [[embryogenesis]]. 

Lavin et al. reported a 68-year-old man who presented with partial left [[third cranial nerve palsy]], visual loss in the left eye without visual field defects, headache, weight loss and reduced muscle bulk. Neuroimaging studies demonstrated a solid and cystic, avidly enhancing lesion expanding the pituitary fossa and extending to the left cavernous sinus. The patient underwent craniotomy and the tissue removed showed features of epithelial-myoepithelial carcinoma similar to the salivary gland, skin and breast counterpart. No primary tumor was found outside the sella. The lesion behaved aggressively despite radio-chemotherapy and the patient died 22 months from the onset. The tumor showed a novel TP53 in-frame deletion (Gly154del) while no variants were found in H-RAS hotspot regions (codons 12, 13 and 61). Our report expands the spectrum of salivary gland-like tumors primarily occurring in the sella and emphasizes the need for specialist review of rare, non-neuroendocrine tumors of the pituitary and sella regions
((Lavin V, Callipo F, Donofrio CA, Ellwood-Thompson R, Metcalf R, Djoukhadar I, 
Higham CE, Kearney T, Colaco R, Gnanalingham K, Roncaroli F. Primary
epithelial-myoepithelial carcinoma of the pituitary gland. Neuropathology. 2020
Jan 3. doi: 10.1111/neup.12628. [Epub ahead of print] PubMed PMID: 31900996.
)).