Primary epithelial-myoepithelial carcinoma of the pituitary gland.
epithelial-myoepithelial carcinoma
pituitary gland
salivary gland-like tumors
Journal
Neuropathology : official journal of the Japanese Society of Neuropathology
ISSN: 1440-1789
Titre abrégé: Neuropathology
Pays: Australia
ID NLM: 9606526
Informations de publication
Date de publication:
Jun 2020
Jun 2020
Historique:
received:
29
10
2019
revised:
10
11
2019
accepted:
10
11
2019
pubmed:
5
1
2020
medline:
2
4
2021
entrez:
5
1
2020
Statut:
ppublish
Résumé
Primary salivary gland-like tumors 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. We report 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.
Types de publication
Case Reports
Langues
eng
Sous-ensembles de citation
IM
Pagination
261-267Informations de copyright
© 2020 Japanese Society of Neuropathology.
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