Medulloblastoma with extensive nodularity: a variant occurring in the very young-clinicopathological and immunohistochemical study of four cases.
METHODS
Four cases of medulloblastoma with extensive nodularity (MBEN) are described. The patients were 3 years of age or younger at diagnosis. Cranial CT scan disclosed multiple coalescing nodules with peculiar 'grape-like' architecture in three patients. A near total excision of the tumour was performed in all four patients. The patients are currently receiving radiation and chemotherapy. Histologically, the tumours were characterized by extreme nodularity with intranodular uniformity and low proliferative index. The internodular zones were extremely cellular, composed of undifferentiated, mitotically active cells. Bcl-2 protein expression was observed in the cellular zones but was distinctly absent within nodules.


CONCLUSIONS
MBEN represents a variant that occurs in very young children but has a good prognosis. The favourable outcome is probably related to its spontaneous neurocytic differentiation. The pattern of Bcl-2 immunoreactivity in MBENs indicates that this protein could be a key player in the regulation of neuronal differentiation in medulloblastomas.
Suresh
T N
TN
Department of Neuropathology, National Institute of Mental Health and Neurosciences, 560 029, Bangalore, India.
Santosh
V
V
Yasha
T C
TC
Anandh
B
B
Mohanty
A
A
Indiradevi
B
B
Sampath
S
S
Shankar
S K
SK
D002528:Cerebellar Neoplasms / Q000378:metabolism / Q000473:pathology*; D002675:Child, Preschool; D005260:Female; D005904:Glial Fibrillary Acidic Protein / Q000378:metabolism; D006801:Humans; D007150:Immunohistochemistry / Q000379:methods; D007223:Infant; D019394:Ki-67 Antigen / Q000378:metabolism; D008279:Magnetic Resonance Imaging; D008297:Male; D008527:Medulloblastoma / Q000378:metabolism / Q000473:pathology*; D010751:Phosphopyruvate Hydratase / Q000378:metabolism; D019253:Proto-Oncogene Proteins c-bcl-2 / Q000378:metabolism; D016708:Synaptophysin / Q000378:metabolism; D014057:Tomography, X-Ray Computed
D005904:Glial Fibrillary Acidic Protein; D019394:Ki-67 Antigen; D019253:Proto-Oncogene Proteins c-bcl-2; D016708:Synaptophysin; D010751:Phosphopyruvate Hydratase