MEDULLOBLASTOMA WITH MYOGENIC DIFFERENTIATION; CASE REPORT AND REVIEW OF LITERATURE.

Rodolfo Franco Marquez; Marco Antonio Ponce Camacho; Hersilia Aide Hernandez Zamonsett; Estefania Boland Rodriguez; Oralia Barboza Quintana

Volume : VII, Issue : III, March - 2018

MEDULLOBLASTOMA WITH MYOGENIC DIFFERENTIATION; CASE REPORT AND REVIEW OF LITERATURE.

Rodolfo Franco Marquez, Marco Antonio Ponce Camacho, Hersilia Aide Hernandez Zamonsett, Estefania Boland Rodriguez, Oralia Barboza Quintana

Abstract :

Medulloblastoma with myogenic differentiation (Medullomyoblastoma) its an entity first described in 1933, consisting in a medulloblastoma conformed by primitive neuroectodermal cells intermixed with cells featuring myogenic differentiation, where the two tumoral populations share the same genetic alterations and occurs almost exclusively in children. Historically, patients with medullomyoblastoma have been considered to have a poor prognosis. We present the case of a 3 year old patient with history of gait disturbances, emesis, headache, generalized weakness and loss of sphincter control. Cranial CT scan revealed a 5 cm. tumor located in the posterior fossa compressing the fourth ventricle with an heterogeneous appearance that enhances with contrast administration. Histological exam showed a tumor with biphasic configuration, consisting of poorly differentiated small cells with abundant mitosis and marked pleomorphism, with areas that had an evident myogenic differentiation . Immunohistochemistry revealed positivity for INI–1, desmin and synaptophysin.