Atypical teratoid and rhabdoid tumor. Report of a congenital case

The atypical teratoid rhabdoid tumor is a rare brain tumor of childhood. We report a congenital case, revealed by peripheral facial palsy. This polymorphous tumor consisted of rhabdoid cells associated with areas of primitive neuroectodermal tumor. The immunoreactivity for the three proteins vimenti...

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Bibliographic Details
Published inAnnales de pathologie Vol. 21; no. 3; p. 259
Main Authors Fernandez, C, Sévenet, N, Bouvier-Labit, C, Lena, G, Figarella-Branger, D
Format Journal Article
LanguageFrench
Published France 01.06.2001
Subjects
Actins - analysis
Brain Neoplasms - congenital
Brain Neoplasms - genetics
Brain Neoplasms - pathology
Child
DNA, Neoplasm - genetics
Humans
Immunohistochemistry
Male
Mucin-1 - analysis
Mutation
Neoplasms, Multiple Primary - congenital
Neoplasms, Multiple Primary - genetics
Neoplasms, Multiple Primary - pathology
Neuroectodermal Tumors, Primitive - pathology
Rhabdoid Tumor - congenital
Rhabdoid Tumor - genetics
Rhabdoid Tumor - pathology
Teratoma - congenital
Teratoma - genetics
Teratoma - pathology
Vimentin - analysis
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Summary:The atypical teratoid rhabdoid tumor is a rare brain tumor of childhood. We report a congenital case, revealed by peripheral facial palsy. This polymorphous tumor consisted of rhabdoid cells associated with areas of primitive neuroectodermal tumor. The immunoreactivity for the three proteins vimentin, epithelial membrane antigen and smooth-muscle actin was suggestive of rhabdoid tumor. Genetic study showed a homozygous mutation in the tumoral DNA and a constitutional heterozygous mutation, as it was demonstrated in atypical teratoid rhabdoid tumors and in renal and extra-renal rhabdoid tumors.
ISSN:0242-6498