Invasion of cranial nerves by salivary cylindroma: four cases treated by radiotherapy

A Case of Adenoid Cystic Carcinoma Presenting as Garcin's Syndrome without Mass Formation

“…In the present case, ACC manifested unilateral, markedly extensive, third to seventh and ninth to twelfth cranial nerve palsies without intracranial hypertension or long tract sign, indicative of Garcin's syndrome. A review mentioned that the disease duration of intracranial ACC cases varies from To our knowledge, the symptomatic duration of Garcin's syndrome cases ranges from two months to seven years (6,(14)(15)(16). Thus, the total clinical course period (14 months), from onset to death, of the present case is in keeping with the previous above-mentioned descriptions.…”

“…Swash (6) reported four cases of ACC of the submaxillary gland, readily confirmed by biopsy at the primary site as evidenced by skull CT, that exhibited mass lesions which spread to the posterior fossa, and invaded the dura matter. Soprani et al (21) reported a case of ACC of the nasopharynx, readily confirmed by biopsy of the surrounding soft tissues evidenced by skull MRI findings such as an expansive submucosal lesion of the unilateral parapharyngeal space, reaching the adjacent petrous bone.…”

“…It is known that ACC can be responsible for several neurological features, including olfactory hypoesthesia (2), visual disturbance (18), neurogenic ophthalmoplegia (2, 5, 6), trigeminal neuralgia and numbness (2,5,6), unilateral facial, glossopharyngeal, vagal, accessory and hypoglossal nerve palsies (6), cavernous sinus syndrome (18,19), Horner's syndrome (6,(19)(20)(21), and pituitary dysfunction (18). In the present case, ACC manifested unilateral, markedly extensive, third to seventh and ninth to twelfth cranial nerve palsies without intracranial hypertension or long tract sign, indicative of Garcin's syndrome.…”

“…There has been a subset of intracranial ACC cases with Garcin's syndrome (5,6,10,21,24). Swash (6) reported four cases of ACC of the submaxillary gland, readily confirmed by biopsy at the primary site as evidenced by skull CT, that exhibited mass lesions which spread to the posterior fossa, and invaded the dura matter.…”

“…ACC is characterized by slow growth, high tendency of mass formation and local recurrence, and distant metastasis typically to the lungs, liver, and bones. It has been shown that intracranial involvement of ACC can spread via the following means: direct invasion as well as hematogenous, lymphatic and perineural invasion (5,6).…”