Cerebellar degeneration as presenting symptom of recurrent endometrial stromal sarcoma with sex-cord elements

Abstract

We report a 66-year-old woman with slowly progressive ataxia due to cerebellar atrophy. Imaging studies revealed multiple lesions in both the lungs and dorsal subpleural space. A biopsy identified the lesions as metastases of a low-grade endometrial stromal sarcoma containing sex-cord elements. The histological appearance was identical to a uterine tumor the patient was treated for with hysterectomy 16 years before. The metastases were removed surgically, and after 3 months ataxia had regressed. We conclude that the presenting cerebellar degeneration in this patient resulted from the metastatic recurrence of the endometrial tumor.

Keywords: Ataxia; Cerebellar atrophy; Endometrial stromal sarcoma; Paraneoplastic syndrome; Sex-cord elements.

Fig. 1

Oculomotor abnormalities as evident from electronystagmography. a Gaze-evoked nystagmus in fast right and left horizontal saccades. b Abolition of pursuit eye movement by saccades. c Normal optokinetic nystagmus in both horizontal directions.

Fig. 2

MRI scan of the brain. Slight atrophy of the upper cerebellar vermis and the superior semilunar lobes in a sagittal (a) and axial projection (b). Chest X-ray (c) and CT scan (d): several bilateral pulmonary metastases and a subpleural metastasis posterior of the left ventricle (arrow).

Fig. 3

HE morphology and immunohistochemistry of a lung metastasis of the low-grade ESS. a Morphological assessment revealed a tumor composed of round to oval cells with slightly pleomorphic nuclei and small nucleoli as well as hyaline intercellular substance between the tumor cells in the HE stain (×200 magnification). b Immunohistochemical analysis revealed CD10 positivity of the tumor cells (×200 magnification).