Temporal lobe atypical teratoid/ rhabdoid tumor in a 24-year old adult female

Abstract

The name atypical teratoid/rhabdoid tumor (AT/RT), a histological variant of Wilm's tumor exemplifies the tumor's disparate mixtures of rhabdoid, primitive neuroepithelial, mesenchymal and epithelial components. AT/RT is much more frequently seen in infants and young children and is rare in adults. Prognosis of AT/RT is poor with most of the patients dying shortly after the diagnosis. [1] The literature suggests that the use of more aggressive therapies (including surgery, chemotherapy with or without stem-cell support, intrathecal chemotherapy, and early radiotherapy), may lead to some prolongation of the natural history of this tumor. [2,3] A 24-year-old female was admitted in the neurosurgery ward of Institute of Post Graduate Medical Education and Research and S.S.K.M Hospital, Kolkata with complaints of repeated headache for 2 months, few episodes of vomiting and unable to open her right eye for 1 month. On general physical examination patient was conscious, afebrile, with stable vital signs and good hydration. There was mild pallor, with no cyanosis, edema, and icterus. On central nervous system examination, there was right sided third cranial nerve palsy. No other neurological deficit was found. Other systemic examinations were within normal limits. Magnetic resonance imaging brain showed a mass lesion in the medial aspect of right temporal lobe and in suprasellar region [Figure 1]. After 10 days, right temporal craniotomy with gross excision of the tumor was done under general anesthesia. Operative specimen was sent for histopathological examination and immuno-histochemistry. Section showed cerebral tissue and a tumor composed of rhabdoid cells in abundant myxoid matrix, cells were round to spindle with a moderate amount of eosinophilic granular cytoplasm with hyper chromatic nuclei [Figure 2] suggestive of AT/RT (World Health Organization Grade four). Immuno-histochemistry depicted that tumor cells were positive for vimentin, epithelial membrane antigen, S-100, and glial fibrillary acidic protein (GFAP) and immune-negativity for CD-31; CD-34 and cytokeratin with loss of expression of nuclear INI1 protein. The Mib-1 labeling index was 12-15%. After 2 weeks of surgery, a computed tomography scan brain was done and showed ill-defined residual mass in right medial temporal lobe and post-operative changes in right temporal region [Figure 3]. However, after surgery general condition of the patient did not improve satisfactorily and the patient was referred for adjuvant therapy. A post-operative chest X-ray, ultra-sonography abdomen and routine blood parameters were all within normal limits. The patient underwent adjuvant craniospinal irradiation (CSI) to 36 Gy, followed

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

“…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).…”