Atypical teratoid/rhabdoid tumors: imaging findings of two cases and review of the literature.
نویسندگان
چکیده
Atypical teratoid/rhabdoid tumor (AT/RT) is a malignant embryonal central nervous system (CNS) tumor, manifesting in children, and composed of rhabdoid cells, with or without fields resembling a classical primitive neuroectodermal tumor (PNET), epithelial tissue and neoplastic mesenchyme. Around 200 cases of CNS AT/RT have been documented in the literature. Although the clinical and pathological findings have been defined in large series previously, and AT/RT has become increasingly recognized, awareness of typical AT/RT is important in making the correct diagnosis of this uncommon but probably underdiagnosed entity. Neuroradiologists rarely mention AT/RT in their differential diagnosis and this paper presents two additional cases in which clinical and pathological findings are combined with neuroradiological presentation.
منابع مشابه
Imaging findings of CNS atypical teratoid/rhabdoid tumors.
Four patients with brain tumors were diagnosed with atypical teratoid/rhabdoid tumors and underwent CT and MR imaging. For all tumors, aggressive features were shown by imaging studies and included hydrocephalus, apparent invasion of the adjacent brain and dura, and marked mass effect. The striking heterogeneity of the atypical teratoid/rhabdoid tumor shown by imaging studies reflects the histo...
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SUMMARY Destruction of the bony structures of the skull is rare in primary tumors of the CNS. In low-grade gliomas, modeling of the skull is caused by slow growth and chronic pressure. Bony destruction is exceptional even in highly malignant gliomas. Atypical teratoid/rhabdoid tumors of the CNS are highly malignant neoplasms diagnosed with an increasing frequency, mainly in young children. On i...
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K.K. Moeller S. Coventry S. Jernigan T.M. Moriarty SUMMARY: Atypical teratoid/rhabdoid tumor (AT/RT) is a highly malignant central nervous system neoplasm usually seen in young children and infants. Prognosis for AT/RT is poor, with most patients dying within 1 year of presentation. AT/RT most commonly occurs intracranially. Location in the spine, though previously reported, is rare, and imagin...
متن کاملGerm-line and acquired mutations of INI1 in atypical teratoid and rhabdoid tumors.
We examined 18 atypical teratoid and rhabdoid tumors of the brain and 7 renal and 4 extrarenal rhabdoid tumors for mutations in the candidate rhabdoid tumor suppressor gene, INI1. Fifteen tumors had homozygous deletions of one or more exons of the INI1 gene, and the other 14 tumors demonstrated mutations. Germ-line mutations of INI1 were identified in four children, one with an atypical teratoi...
متن کاملTwo-stage surgical resection of an atypical teratoid rhabdoid tumor occupying the infratentorial and supratentorial compartment in children under two years: Report of two cases
INTRODUCTION Atypical teratoid rhabdoid tumors are highly malignant neoplasms that present in young children and can grow to a large size. Maximal safe surgical resection is a mainstay of treatment. PRESENTATION OF CASES Two cases of children under the age of two with large tumors involving the supratentorial and infratentorial compartments are presented. A two-staged operative approach combi...
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عنوان ژورنال:
- Turkish neurosurgery
دوره 18 1 شماره
صفحات -
تاریخ انتشار 2008