Spinal metastasis of endometrial stromal sarcoma: clinicopathological features and management

Abstract

Background: Endometrial Stromal Sarcoma (ESS) is a rare uterine malignancy which often metastasizes several years after initial diagnosis. Thoracic spine is a rare ESS metastatic site and its proper management is still not a consensus. We discuss the histopathological features and the management strategies through an illustrative case of a 77 year-old woman with metastasis to the thoracic spine 13 years after total hysterectomy for ESS.

Methods: Review of the literature and identification of 5 patients, including our present case, with ESS involving the spinal cord. We discuss the outcomes achieved after each therapy. In our case, the patient presented a mass involving the thoracic spinal canal constricting the spinal cord at T7 level. A two-level decompression laminectomy was performed and the lesion was partially excised.

Results: The histopathological along with the immunohistochemical profile mitotic rate indicated the endometrial stromal cells origin of the tumor, confirming the diagnosis of a low-grade ESS metastasis. The patient was managed with surgery in combination with postoperative radiation therapy.

Conclusions: The small number of published cases precludes definitive conclusions regarding standard management. However, it seems that treatment of metastatic ESS to the spine matches the same general concepts of spine metastasis, namely surgery followed by radiation therapy, due to clinical improvement and long-term disease control of the reported cases. Hormonal therapy may be considered in recurrent disease with strong expression of estrogen and progesterone receptors. However, these findings need confirmation in larger studies.