M. Kuwamura et al., Spontaneous and ethyl-nitrosourea-induced medullomyoblastomas in cerebellar vermis defect (CVD) mutant rats, ACT NEUROP, 99(3), 2000, pp. 285-288
A 26-week-old female cerebellar vermis defect (CVD) rat, a mutant with cere
bellar vermis defect and cerebellar dysplasia, developed a brain tumor abou
t 10 mm in diameter. Histopathologically, the tumor consisted of diffuse pr
oliferation of small round to ovoid cells with hyperchromatic nuclei, occas
ionally containing round to strap-shaped myoblastic cells. Immunohistochemi
cally, the small round cells expressed neuron-specific enolase and synaptop
hysin, indicating neuronal differentiation; myoblastic components reacted t
o desmin, myoglobin, and vimentin. Based on these findings, the case was di
agnosed as a medullomyoblastoma (MMB). Furthermore, two cerebella tumors in
CVD rats, which were induced by transplacental application of ethyl-nitros
ourea, showed histopathology similar to the aforementioned case. MMB is a v
ery rare tumor in humans and animals; thus, it is noteworthy that MMBs deve
loped in CVD rats, involving the dysplastic cerebellum with abnormal migrat
ion of external granule cells.