Primary epithelial‐myoepithelial carcinoma of the pituitary gland

Abstract

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.

Salivary gland tissues and derived primary and metastatic neoplasms: unusual pitfalls in the work-up of sellar lesions. A systematic review

“…All patients were operated on—half of them through a TC approach—and subsequently irradiated (20–54 Gy). Two patients also received chemotherapy (isofosfamide/BCNU or temozolomide) for an epithelial–myoepithelial carcinoma and an aggressive myoepithelioma, respectively [ 24 , 35 ], with a poor response. Except for one case of papillary mucinous carcinoma [ 33 ], all patients showed disease progression within 4 years (4/8, 50%) or died (6/8, 75%, 3 post-operative deaths).…”

“…Symptomatic enlargement of ectopic SG rests may be non-neoplastic (NNESG) or due to benign or malignant salivary tumours (ST) that mimic other non-functioning lesions, and the diagnosis relies on pathology where surgery is indicated. In addition, because malignant ST derived from major or minor eutopic SG may reach the sella through local invasion or blood spread [21][22][23], an extra-sellar origin should be excluded before concluding to a primary ectopic SG malignancy [24]. Sellar salivary neoplasms represent an unusual challenge for specialists involved in the management of pituitary neuroendocrine tumours (Pit-NETs) [25] and other sellar/parasellar lesions.…”

“…Primary malignant ectopic STPrimary malignant eST were reported in 8 cases[24,29,[33][34][35][36][37] and consisted of adenoid cystic carcinoma (2/8, 25%), myoepithelioma (2/8, 25%), epithelial-myoepithelial carcinoma (1/8, 12.5%), papillary mucinous adenocarcinoma (1/8, 12.5%), low-grade acinic cell carcinoma (1/8, 12.5%) and adenocarcinoma (1/8, 12.5%). All patients were adult (3M, 5F, 34-68 years, median 51.5) and had visual symptoms, with frequent oculomotor nerve palsy/diplopia (5/8, 62.5%).…”

Epithelial–myoepithelial carcinoma of the nasopharynx: A case report and review of the literature

“…Of all the types of nasopharyngeal malignancies treated at our center, the incidence of EMCa is 1/315 (0.3%) as of 2021. This tumor can occur in unusual sites, such as the breast (13)(14)(15), pituitary gland (16), paranasal sinus (9,17,18), lacrimal gland (19)(20)(21)(22), nasal cavity (1,3,6,(23)(24)(25), trachea and bronchus (26,27), lung (28)(29)(30)(31)(32)(33)(34), nasopharynx (4,5), and even the pleura mediastinalis (35). EMCa was first reported by Donath in 1972, and 8 patients have been reported with a salivary gland tumor that was termed EMCa (36).…”