High grade endometrial stromal sarcoma tumor recurrence

Abstract

BACKGROUND: Endometrial stromal sarcomas (ESS) are very rare type of malignant uterine tumors, making up around 0.2-1% of all uterine neoplasms and less than 10% of uterine sarcomas.

CLINICAL CASE: 59 year old lady, previously treated for grade III invasive ductal breast carcinoma with surgery, anthracycline chemotherapy and external beam radiotherapy to the pelvis to ablate the ovaries, presented with postmenopausal bleeding. A transvaginal ultrasound scan (TVUS) showed a 4.5cm by 5.1cm heterogeneous area in the endocavity, suspicious of haematometria. Hysteroscopy, dilation and curettage (D&C) noted multiple clots as well as a mass, suspicious of a fibroid or tumour arising from the endometrium. An MRI of the uterus reported a pedunculated intracavitary fibroid. A total abdominal hysterectomy with bilateral salpingo-oophorectomy (TAH + BSO) was performed in view of worsening symptoms. Histology reported high grade endometrial stromal sarcoma, limited to the uterus, measuring 75mm in greatest dimension, pT1b Nx (FIGO IB). Six months later the patient presented again with pv bleeding. Upon examination a polypoid exophytic mass was noted in the vaginal vault. A sample was taken and sent for histology, which reported features in keeping with recurrence of the previous endometrial stromal sarcoma. An MRI of the pelvis showed vaginal vault stump recurrence and a 1.7cm serosal nodule on the mid sigmoid colon. CT of the abdomen and pelvis showed no evidence of extra pelvic disease. Patient was scheduled for a resection of the upper vagina including the vault nodule and adjacent sigmoid colon. Histology of the resected specimens reported recurrence of the high grade uterine stromal sarcoma in the vaginal vault, which invaded the sigmoid colon.

CONCLUSION: The mainstay treatment for ESS is a TAH + BSO. This case demonstrates that although complete remission can be obtained, recurrence can still happen. Clinicians should follow these patients closely, even when disease free. Early diagnosis and surgical removal of recurrence is of utmost importance to survival of these patients.