Content » Vol 94, Issue 4

Clinical Report

Cutaneous Location of Atypical Teratoid/Rhabdoid Tumour

Nathalia Bellon, Sylvie Fraitag, Catherine Miquel, Laurent J. Salomon, Franck Bourdeaut, Christine Bodemer, Thomas Roujeau, Michel Zerah, Smail Hadj-Rabia
DOI: 10.2340/00015555-1716


Atypical teratoid/rhabdoid tumour is a rare and highly malignant tumour of the posterior fossae nervous system that occurs in children especially in the first few years of life. Cutaneous location is not previously reported. A newborn boy was referred for both aqueductal stenosis detected antenatally and skin tags mimicking hamartoma. The cerebral tumour increased in size during a few months leading to both skin and cerebral biopsies. Integrase Interactor-1 (INI-1) immunostaining and tumoural and leukocytes INI-1 gene sequencing confirmed the atypical teratoid/rhabdoid tumour nature of the cerebral tumour. INI-1 immunostaining in skin biopsy confirmed the dermal location of rhabdoid tumour. Thus, unusual cutaneous lesions may be part of atypical teratoid/rhabdoid tumour. The loss of Integrase INI-1 on immunohistochemical staining is characteristic.


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