Medullomyoblastoma in an adult

Abstract

Medullomyoblastoma is a rare histologic variant of medulloblastoma. Of the 20 cases reported in the literature, 19 were in children ages 2.5 to 10.5 years and one was in a 26-year-old woman. In the reported adult case the myogenic component of the tumor was leiomyosarcomatous. The authors report a case of medullomyoblastoma with a rhabdomyosarcomatous component in a 40-year-old man with light microscopic, immunohistochemical, and ultrastructural findings. The histogenetic theories regarding this tumor include that it is a teratoma, or that the myogenic component arises from the perivascular or leptomeningeal ectomesenchyme, or pluripotential neuroectodermal cells, or endothelial cells. The authors' findings do not elucidate the histogenesis but argue against an endothelial origin of the rhabdomyoblastic component.

Medulloblastoma with myogenic differentiation: long-term survival in a patient treated with aggressive combination therapy and autologous stem cell transplantation

“…MMD presents exceptionally rarely in adulthood and it is reasonable to consider whether this has a prognostic effect. Two case reports in adults with MMD demonstrated survival of less than 1 month [5] and less than 3 years [3]. The findings of these authors provide no evidence to suggest there is any prognostic advantage to presentation in adulthood.…”

“…The majority of patients have survived less than 1 year from diagnosis [5]. The longest reported survival in a patient treated with either radiotherapy or chemotherapy alone is 4 years [18].…”

“…Our patient also had a protracted symptomatic prodrome before diagnosis and it is necessary to contemplate whether this could be related to his positive outcome, suggesting a slower growing tumor. The duration of symptoms prior to diagnosis is typically short, mostly 3 months or less (Rao et al 1990) [5]. However, longer symptomatic periods prior to diagnosis have been reported and have not been associated with improved long-term survival.…”

“…The majority of patients affected by MMD are children under the age of ten [2–4]. However, cases in adults have been reported, the oldest being 40 years [5–7]. …”

Unusually long survival in a case of medullomyoblastoma

“…Only a few cases have survived 3 years post-treatment. 6 Rao et al 18 reviewed the literature to 1990 and reported that 10 of 21 cases of MMB had a very short survival: only 5 cases survived longer than 6 months and 3 patients survived for more than 2 years, emphasizing the poor outcome for MMB patients. Lewis et al 12 reported a case who survived for more than 4 years.…”