Primary Adenoid Cystic Carcinoma of the Orbital Apex

Abstract

Adenoid cystic carcinoma is an uncommon malignant tumor of epithelial origin typically arising from salivary glands. Orbital involvement may occur via direct or perineural spread from a lacrimal gland or sinonasal source. Primary orbital adenoid cystic carcinoma without involvement of the lacrimal gland is rare. The authors report a 53-year-old woman who was examined for insidious monocular vision loss and was found to have a primary adenoid cystic carcinoma of the orbital apex and cavernous sinus. Systemic workup for a primary source, including ipsilateral lacrimal gland biopsy, was negative. One must maintain a high index of suspicion for adenoid cystic carcinoma when evaluating orbital tumors.

Bilateral Adenoid Cystic Carcinoma of the Orbit

“…5,7 Arsene et al 8 described the only documented case of bilateral extension of ACC across the cavernous sinus. This case was unique in that the primary mass in the left cavernous sinus gradually extended in the right cavernous sinus, sphenoid jugulum, and right orbit across the suprasellar space without macroscopic involvement of either lacrimal gland.…”

Orbital apex disorders: Imaging findings and management

“…Most common neoplastic involvement of the OA is by head and neck squamous cell carcinoma (SCC), adenocystic carcinoma, mucoepidermoid carcinoma and nasopharyngeal carcinoma either via perineural spread, local invasion, or metastasis. [16][17][18][19] Perineural tumor spread (PNTS) is most commonly seen with SCC, adenoid cystic carcinoma, as well as lymphomas and melanoma. Trigeminal nerve (CN V) is the most commonly affected at the skull base with involvement of maxillary (V2), mandibular (V3), and ophthalmic (V1) divisions in that order.…”

Adenoid cystic carcinoma presenting as an orbital apex mass with intracranial extension

“…1,2 ACC is an aggressive malignancy with high rate of recurrence, distant metastasis, and intracranial extension. 3 Within the orbit, ACC typically presents as a superotemporal orbital mass of lacrimal gland origin, as the lacrimal gland is the only source of epithelial cells in the orbits. 4 Rare cases of tumours developing in the lacrimal sac or other areas of the orbit from ectopic lacrimal gland tissue have been reported.…”

“…[4][5][6][7][8] To our knowledge, there have been only 3 reported cases of ACC arising as a primary orbital tumour without lacrimal gland involvement. 3,9,10 The authors would like to report another case of primary orbital apex tumour with a subsequent histopathologic diagnosis of ACC without pathological evidence of lacrimal gland involvement.…”

“…Lin et al 9 presented a case of ACC in a 60-year-old female in the inferior orbit involving the inferior rectus muscle and without evidence of lacrimal tissue involvement. Walsh et al 3 reported a case of ACC invading the orbital apex and cavernous sinus without evidence of lacrimal tissue involvement in a 53-year-old female.…”