Medulloblastoma in the nevoid basal-cell carcinoma syndrome: case reports and review of the literature.
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.
Lacombe
D
D
Clinique de Pédiatrie et Génétique Médicale, Hôpital des Enfants, Bordeaux.
Chateil
J F
JF
Fontan
D
D
Battin
J
J
D000293:Adolescent; D001478:Basal Cell Nevus Syndrome / Q000175:diagnosis / Q000235:genetics*; D001845:Bone Cysts / Q000175:diagnosis / Q000235:genetics; D002528:Cerebellar Neoplasms / Q000175:diagnosis / Q000235:genetics*; D002648:Child; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D008297:Male; D008339:Mandibular Neoplasms / Q000175:diagnosis / Q000235:genetics; D008527:Medulloblastoma / Q000175:diagnosis / Q000235:genetics*; D009386:Neoplastic Syndromes, Hereditary / Q000175:diagnosis / Q000235:genetics*