Uncommon presentation of medulloblastoma

Abstract

Introduction: Medulloblastoma commonly occurs in children as a midline posterior fossa mass arising from the vermis, which appears as a hyperdense, homogeneously enhancing mass on CT scan and is associated with the clinical profile of posterior fossa syndrome. This unique clinico-radiological pattern is considered 'typical', but then medulloblastomas do not follow the typical clinico-radiological pattern in a significant number of cases.

Patients: Out of the 42 cases of medulloblastoma operated on at SGPGIMS from 1988 to 1998, 29 cases were retrospectively and 13 cases were prospectively studied to detect the atypical clinico-radiological features. The typical radiological feature of a hyperdense homogeneously enhancing mass was seen in only 23 of the 42 patients, while 5 patients had hypodense nonenhancing masses, 13 had cystic changes, and 6 patients had calcifications in their tumours. Three patients presented with tumours in a very unusual location, i.e. the cerebellopontine angle cistern.

Results: During follow-up, which ranged from a minimum period of 1 year to a maximum of 9 years, patients came back with metastases at very unusual sites. There were 5 cases of metastases in the frontal and subfrontal area (developed between 5 months and 5 years following surgery), and 1 patient developed a cervical intramedullary metastasis. Two patients developed abdominal metastases and ascites 2 years after definitive surgery and ventriculo-peritoneal shunting. Each of these 2 patients, however, had received a full course of craniospinal irradiation following surgery. Thus, we had a number of cases with an unusual clinical, radiological and metastatic pattern.