Spinal Atypical Rhabdoid Teratoid Tumor in an Adult Woman: Case Report and Review of the Literature.

Affiliation

Department of Neurosurgery, Evangelismos Hospital, Athens, Greece; Department of Neurosurgery, National Kapodistrian University of Athens, Athens, Greece; Athens Microneurosurgery Laboratory, Athens, Greece. Electronic address: [Email]

Abstract

BACKGROUND : Atypical rhabdoid teratoid tumors are very rare embryonal tumors that typically affect children younger than 3 years old and are encountered intracranially.
METHODS : Here, we describe the case of a 19-year-old woman who presented with gait disturbances and coccydynia. Imaging revealed a cauda equina mass. The tumor was partially resected. Histology reported loss of SMARCB1/INI1 expression and therefore the diagnosis of atypical rhabdoid teratoid was established. The patient underwent radiation treatment, but within 3 months 2 relapses were manifested.
CONCLUSIONS : Atypical rhabdoid teratoids are exceptionally rare in adults and are seldom found in spine; only 8 such cases have been reported in the medical literature. They are invariantly characterized by multiple relapses and dismal prognosis. The clinician must be attentive of leptomeningeal disseminations and 22q11 deletion-associated comorbidities.

Keywords

ATRT,Adult,Atypical rhabdoid teratoid tumors,Spinal,