Intracranial calcifications in childhood medulloblastoma: Relation to nevoid basal cell carcinoma syndrome

BACKGROUND AND PURPOSE: Medulloblastoma is one of the most common posterior fossa tumors to occur in children, Our purpose was to document the frequen cy, location, and time of occurrence of intracranial calcifications in cran ial CT studies of children with medulloblastoma, METHODS: We retrospectively reviewed cranial CT studies of 56 patients diag nosed with medulloblastoma from 1983 through 1997 for the presence of intra cranial calcifications, The findings were compared with 159 cranial CT stud ies of patients who were evaluated in the emergency department (control gro up), Thirty-two patients with medulloblastoma without shunts were compared with 118 patients from the control group without shunts, Similarly, 24 pati ents with medulloblastoma with shunts were compared with 41 patients from t he control group with shunts, RESULTS: Overall, three (9%) patients with medulloblastoma without shunts, four (16%) patients with medulloblastoma with shunts, and four (10%) patien ts from the control group with shunts had fair calcification, Only the two children carrying the diagnoses of medulloblastoma and nevoid basal cell ca rcinoma syndrome, however, had calcification of the fair cerebri shown on t he cranial CT scans obtained during the peridiagnostic period, Both were di agnosed with medulloblastoma before the age of 3 years and later developed jaw cysts and multiple basal cell carcinomas in the radiation field, CONCLUSION: Previous studies have shown that fair calcification is a major component of nevoid basal cell carcinoma syndrome, Our two cases illustrate the importance of considering the diagnosis of nevoid basal cell carcinoma syndrome when fair calcification is present in young patients with medullo blastoma. if the concomitant diagnosis of nevoid basal cell carcinoma syndr ome is made, alternative types of therapy should be sought to minimize radi ation therapy sequelae.