Atypical teratoid/rhabdoid tumor arising from the trigeminal nerve in an adult

Abstract

A 30-year-old male presented with left facial pain and numbness. Initial MRI demonstrated an enhancing mass involving the left trigeminal nerve. Follow-up imaging showed interval growth with erosion of the sphenoid body. Surgical resection was performed and immunohistochemistry staining was consistent with an atypical teratoid/rhabdoid tumor. Awareness of this entity and its imaging features such as diffusion restriction, intratumoral hemorrhage, and bony destruction, can help guide confirmatory diagnostic testing and appropriate therapy.

Atypical Teratoid/Rhabdoid Tumor Originated From the Trigeminal Nerve in a Young Male Adult: Case Report and Review of the Literature

“…To date, a total of nearly 55 adult AT/RTs have been reported in the literature (12). Only one of them originated from the trigeminal nerve (9), and this young adult reported here belongs to another one. In the present report, multiple brain nerves were involved successively due to the expansion of the tumor, which then manifested as third to eighth cranial nerve dysfunctions.…”

“…AT/RT has a similar incidence rate in young pediatric patients as medulloblastoma and primitive neuroectodermal tumor (PNET), at approximately 2%, but it is very rare in adults (9,10). AT/RT occurs more frequently in men than in women, with a ratio of ∼2: 1 (8).…”

“…Adult-onset AT/RT can occur anywhere in the central nervous system, such as the cerebellar hemispheres, sellar region, spinal cord, pineal region, and cranial nerves (9,(13)(14)(15)(16), even secondarily developing from other primary tumors (17,18). In addition, the clinical manifestations and neuroimaging characteristics of patients often lacked specificity (19).…”

“…In addition, the clinical manifestations and neuroimaging characteristics of patients often lacked specificity (19). Although mixed intensity in T2-weighted image, heterogeneous contrast enhancement, mild peritumoral edema, high density, and bone destruction on computed tomography have been reported frequently in adult-onset AT/RT (9,20), there are still great challenges in accurate diagnosis of AT/RT preoperatively. In clinical practice, AT/RT is easily misdiagnosed as other types of intracranial tumor before pathological confirmation.…”

“…The clinical manifestations of AT/RT mainly depend on the tumor location. Patients with AT/RT deriving from the cranial nerves have been described in few literature to date (6,7), but these cases are still rarely reported in adults (8,9). Here, we report a case of adultonset AT/RT suspected to be derived from the trigeminal nerve followed by the involvement of multiple cranial nerves in a short period of time.…”