Wg. Mccluggage et al., ENDOMETRIAL STROMAL SARCOMA WITH SEX CORD-LIKE AREAS AND FOCAL RHABDOID DIFFERENTIATION, Histopathology, 29(4), 1996, pp. 369-374
An unusual uterine lesion is described in a patient with postmenopausa
l bleeding, Grossly, a yellow, polypoid mass projected into the uterin
e cavity, Histological examination showed a distinct biphasic pattern,
with areas of typical low-grade endometrial stromal sarcoma and areas
where tumour cells were arranged in cords and trabeculae, resulting i
n a sex cord-like pattern. In these areas the cells assumed a rhabdoid
morphology with eccentric vesicular nuclei, prominent nucleoli and eo
sinophilic hyaline cytoplasmic inclusions, Immunohistochemistry showed
positive cytoplasmic staining of both components for vimentin, desmin
and the cytokeratin marker CAM 5.2, but no staining for CEA and EMA.
Electronmicroscopy revealed prominent paranuclear arrays of intermedia
te filaments, This is the second reported case of endometrial stromal
sarcoma with rhabdoid differentiation and the first documented example
of rhabdoid cells in sex cord-like areas. The report adds to the list
of diverse neoplasms which may display a characteristic rhabdoid morp
hology and supports the hypothesis that extrarenal rhabdoid tumours ar
e not a distinct clinicopathological entity, A diagnosis of malignant
rhabdoid tumour of the uterus should be considered only when extensive
sampling fails to disclose areas with an appearance typical of an end
ometrial stromal lesion.