Large-cell medulloblastomas. A distinct variant with highly aggressive behavior.

TitleLarge-cell medulloblastomas. A distinct variant with highly aggressive behavior.
Publication TypeJournal Article
Year of Publication1992
AuthorsGiangaspero F, Rigobello L, Badiali M, Loda M, Andreini L, Basso G, Zorzi F, Montaldi A
JournalAm J Surg Pathol
Date Published1992 Jul
KeywordsBlotting, Southern, Cerebellar Neoplasms, Cerebellum, Gene Amplification, Genes, myc, Humans, Immunohistochemistry, Infant, Infant, Newborn, Karyotyping, Male, Medulloblastoma, Prognosis

We present four cases of infantile cerebellar neoplasms composed of cells with large vesicular nuclei with prominent nucleoli. All four cases were strongly immunoreactive for synaptophysin, and one case showed immunoreactivity for neurofilaments. Filter hybridization for N-myc and c-myc oncogenes showed a 27-fold c-myc amplification in one case. The cytogenetic analysis in this case showed Double-Minutes and isochromosome 17q. An intracerebral xenograft in nude mice obtained from one such tumor showed a similar morphology to that of the original tumor as well as strong immunoreactivity for synaptophysin and neurofilaments. All the neoplasms were characterized by highly aggressive behavior leading to early cerebrospinal fluid dissemination despite radiotherapy and chemotherapy. We conclude that large-cell medulloblastoma represents a distinct and more aggressive variant of medulloblastoma that requires more aggressive therapy.

Alternate JournalAm J Surg Pathol
PubMed ID1530108
Related Faculty: 
Massimo Loda, M.D.

Pathology & Laboratory Medicine 1300 York Avenue New York, NY 10065 Phone: (212) 746-6464
Surgical Pathology: (212) 746-2700