Progestogen‐induced regression in low‐grade endometrial stromal sarcoma. Case report and literature review

Abstract

A 42-year-old nulliparous woman presented with a large pelvic mass. Laparotomy showed a 20-cm partially cystic lesion suspended in the omcntum, as well as a smallcr solid mass in the rcctovaginal spacc with a protrusion into the pouch of Douglas. The cystic lesion was excised. The ovarics appeared normal and were conserved. Removal of thc lesion in the rectovaginal spacc was delayed until six wccks later, after the histology had been reviewed. This mass was shelled out easily and the capsule left intact.Thc past history was significant; ten years previously she had been treated, at another hospital for menorrhagia and dysuria of eight months duration. Examination had shown an irregularly shaped uterus, enlarged to the size of a 16-week prcgnancy, and a total hysterectomy was performed for presumed fibroids. Histology showed 'stromal cndornctriosis'.A year after removal of the pelvic recurrence a chcst X-ray showed a ncw opacity in the lcft upper lung ficld. At thoracotomy a 1.5 cm pinkish brown, partly haemorrhagic, wcll-circumscrihcd subpleural lesion was removed with a margin of normal lung. Two months later at another abdominal operation a 2.5 cm recurrence was rcmoved from just to the left of the rectum below thc pelvic peritoneum. Four months later a computed tomography scan of the pelvis showcd recurrent tumour lying behind the bladder and extending out of the pelvis on the lcft in relation to the psoas muscle.At this stagc the patient had undergone a total o f four opcrations for recurrent o r metastatic

Expression of oestrogen and progesterone receptors in low-grade endometrial stromal sarcomas

“…7, score 12). PR, peroxidase-antiperoxidase, original magnification × 200 Krumholz et al, 1973;Gloor et al, 1982;Baker et al, 1984;Tsukamoto et al, 1985;Keen and Philip, 1989;Montag and Manart, 1989;Rad and Lowe, 1990;Horowitz et al, 1996). Scribiner and Walker (1998) reported preoperative treatment of LGSS with progestins.…”

CD10 is a Marker for Normal and Neoplastic Endometrial Stromal Cells

“…Uterine smooth muscle tumors are also positive for smooth muscle markers and sex steroid receptors (7), but sex steroid receptor expression is often decreased in both high-grade ESSs and leiomyosarcomas (6,7). Such diverse differentiation sometimes leads to diagnostic difficulties among uterine sarcomas, and because progestin treatment is often clinically effective for patients with ESS (8,9), there is a need to find an effective way of differentiating ESS from leiomyosarcoma and other uterine mesenchymal tumors.…”

Endometrial Stromal Sarcomas Frequently Express Epidermal Growth Factor Receptor (EGFR, HER-1)

“…21 Studies have revealed that therapy with progestational agents is an important adjunct to surgical treatment in cases of lowgrade ESSs. 12,16,18,22 Recurrent or metastatic low-grade ESSs have been reported to be stabilized or suppressed with progestational agents in more than 50% of patients. 16,18,22 However, although most of low-grade ESSs have been shown to express ER and PR, a significant amount of the tumors, even with high ER and PR content, do not respond to adjuvant hormonal treatment.…”

“…12,16,18,22 Recurrent or metastatic low-grade ESSs have been reported to be stabilized or suppressed with progestational agents in more than 50% of patients. 16,18,22 However, although most of low-grade ESSs have been shown to express ER and PR, a significant amount of the tumors, even with high ER and PR content, do not respond to adjuvant hormonal treatment. 18,22 Epidermal growth factor receptor (EGFR), also known as HER-1, is characteristically expressed in normal endometrial stromal cells.…”