High-grade adenoid cystic carcinoma originating from the lacrimal gland

Abstract

Among primary lacrimal gland tumors, adenoid cystic carcinoma (ACC) is the most common malignant epithelial neoplasm; it is characterized by local intracranial invasion. A case with unusual dumbbell-type intracranial extension representing cavernous sinus syndrome is described. A 49-year-old woman was admitted to our hospital with right cavernous sinus syndrome. Computerized tomographic (CT) scans and magnetic resonance (MR) imaging demonstrated well-enhanced intraorbital and middle fossa tumors mimicking multifocal mass lesions. Operative findings revealed an ACC originating from the lacrimal gland and extending into the right cavernous sinus and middle fossa along the nerve sheath in the superior orbital fissure. Although MR image findings of intracranial ACC often resemble the image findings for meningiomas, intracranial ACC is very aggressive in comparison with meningioma. It is best treated surgically and aggressively.

Adenoid Cystic Carcinoma of the Cavernous Region-Case Report-

“…[1][2][3]6) In addition, three routes have been reported for the intracranial invasion of salivary gland ACCs: the eustachian tube (in the peritubal space), 1,[5][6][7] the mandibular and maxillary nerves, and the internal carotid artery. 3,5) In the present case, the tumor was located in the right Meckel's cave and cavernous sinus with invasion of the foramen rotundum, foramen ovale, and superior orbital fissure. These findings suggested that the intracranial invasion occurred through perineural infiltration along the mandibular or maxillary nerves.…”

“…Two major regions of intracranial invasion are the optic chiasm and the gasserian ganglion. 5,6) We report a case of ACC that mimicked a trigeminal schwannoma on neuroimaging. The diagnosis was established by histological examination, but no evidence of a primary lesion was detected, which is very unusual.…”

“…1,6) Most ACCs arise in the major and minor salivary glands, nasopharynx, and lacrimal glands, but also arise from the upper respiratory tract, the lungs, the mammary gland, the skin, the mastoid air cell, and the genital tract. [1][2][3][5][6][7] ACCs account for 11% 5) or 25-30% 4) of epithelial neoplasms in the lacrimal gland. The incidence of intracranial invasion varies from 4% [1][2][3]5,6) to 22%.…”

“…[1][2][3][5][6][7] ACCs account for 11% 5) or 25-30% 4) of epithelial neoplasms in the lacrimal gland. The incidence of intracranial invasion varies from 4% [1][2][3]5,6) to 22%. 7) Most reported intracranial ACCs are located at the base of the skull.…”

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“…9 A maioria dos CAC origina-se das glândulas salivares maiores e menores, nasofaringe e glându-las lacrimais. 4,6 É reconhecido pelo seu crescimento lento, padrão de recorrência e metástases a distância.…”