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Genet Couns. 1990;1(3-4):273-7.

Medulloblastoma in the nevoid basal-cell carcinoma syndrome: case reports and review of the literature.

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Clinique de Pédiatrie et Génétique Médicale, Hôpital des Enfants, Bordeaux.


The nevoid basal-cell carcinoma syndrome (NBCCS) is a rare autosomal-dominant inherited disorder. Its clinical manifestations are multiple basal-cell nevi and cysts of the jaw along with skeletal anomalies and various combinations of numerous other defects. NBCCS is characterized by a marked propensity for developing cancers. One of the most frequently reported tumour is brain medulloblastoma. We are reporting two cases of NBCCS and medulloblastoma. A review of the case reports demonstrates certain prominent features of medulloblastoma associated with NBCCS. The patients generally are males, presenting at an unusually young age, under 5 or 2 years and show a longer survival rate. Its lay down to search for NBCCS in early medulloblastoma's, especially under 2 years.

[Indexed for MEDLINE]

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