Low-grade endometrial stromal sarcoma

Abstract

Endometrial stromal sarcoma is a rare malignant neoplasm that originates in the
endometrial stroma. It can have several forms of differentiation, including smooth
muscle and sex cord. Low-grade endometrial stromal sarcoma is a rare type of endometrial tumor, constituting only 0.2% of all uterine neoplasms. Its etiology is
unknown, but some cases are associated with obesity, polycystic ovary syndrome,
diabetes mellitus, early menarche, and estrogen replacement therapy or tamoxifen.
Symptomatology is nonspecific, ranging from pelvic pain to progressive abnormal
genital bleeding, and is difficult to recognize by radiological imaging. In most cases, the diagnosis is made by pathological evaluation. Immunohistochemical staining can
also help differentiate it from other neoplasms. Therefore, it is important to have a high index of suspicion for this type of rare neoplasm. Its treatment is surgical
and its follow-up should be long term, due to the high risk of late recurrences and
metastasis. A case of low-grade endometrial stromal sarcoma is presented.