Suboccipital Extraspinal Extracranial Atypical Teratoid Rhabdoid Tumor

• Published in: Journal of neurosciences in rural practice 2017-07, Vol.8 (3), p.494-495
• Main Authors: Karthikeyan, Y R, Gupta, Ashok, Varshney, Tarun, Deora, Amit Pratap Singh
• Format: Article
• Language: English
• Subjects: Age; Brain cancer; Chemotherapy; Letters to the Editor; Medical prognosis; Nervous system; Neurosurgery; Patients; Pediatrics; Proteins; Radiation therapy; Tumors
• ISSN: 0976-3147; 0976-3155
• DOI: 10.4103/jnrp.jnrp_388_16

There was no obvious intracranial or spinal extension or compression of the cervical spinal cord. Computed tomography cervical spine showed erosion of the squamous occipital bone, left occipital condyle, left half of arch of the atlas, and involvement of left atlanto-occipital joint. With a provisional diagnosis of soft tissue sarcoma or peripheral nerve sheath tumor, surgical resection of the tumor was planned. Histopathologically, ATRT is characterized by rhabdoid tumor cells, which have vesicular nuclei, large nucleoli, and cytoplasmic filamentous inclusions. Besides the presence of rhabdoid cells, the lack of nuclear INI1 expression is the most important prerequisite to secure the diagnosis and is usually used as an important marker to distinguish from other central nervous system tumors. The majority of ATRT cases display genetic alterations of SMARCB1 (INI1/hSNF5), a tumor suppressor gene located on 22q11.2, and resulting in loss of INI1 protein. There is no accepted standard chemotherapeutic regimen, but intensive alkylator-based regimens with stem cell rescue may be effective in these patients. Atypical teratoid rhabdoid tumors: A population-based clinical outcomes study involving 174 patients from the surveillance, epidemiology, and end results database (1973-2010).