Long-Term Survival of Patients Given Hormonal Therapy for Metastatic Endometrial Stromal Sarcoma

Abstract

Endometrial stromal sarcoma (ESS) is a rare neoplasm, mainly observed in premenopausal women. We describe two women 44 and 34 years old at the time ESS diagnosis, who developed lung metastases 3 and 6 years, respectively, after initial treatment: hysterectomy without (case 1) or with oophorectomy (case 2), followed by hormone replacement therapy (HRT) for the latter. Their estrogen (ER) and progesterone receptors (PR) were analyzed biochemically in metastatic lung tissue, yielding respective concentrations of ER 242 and 184, and PR 910 and 100 fmol/mg of cytosol protein. Both patients started treatment with the aromatase inhibitor aminoglutethimide (500 mg qid) after surgery for the first patient and after stopping HRT for the second. Under aromatase-inhibitor therapy, both patients achieved a complete response, patient 1 remains disease- free with 14+ years of follow-up, and patient 2 with 7+ years. Our data suggest that an aromatase inhibitor may be an effective treatment for ESS. Furthermore, routine ER and PR analyses could be useful to predict the response to hormonal therapy in ESS.

Aromatase expression in low-grade endometrial stromal sarcomas: an immunohistochemical study

“…24,25 The demonstration of aromatase expression in low-grade endometrial stromal sarcomas may be a rationale for a study of aromatase inhibitors in the treatment of women with lowgrade endometrial stromal sarcomas. Spano et al 26 have reported initial results of such adjuvant hormonal therapy. In their series, two patients with metastatic low-grade endometrial stromal sarcomas had a complete response after treatment with an aromatase inhibitor.…”

Endometrial sarcoma. Case report and review of the literature

“…Hormone therapy include: a) megestrol/medroxyprogesterone, gonadotrophin releasing hormone analogues and aromatase inhibitors, like letrazole and anastrozole. Spano JP et al [6] and Alkasi et al [7], are 2 case reports which shows 10 year free survival rate for the patients that received aromatase inhibitor for 10 years. Chu et al [8], study compared the outcome for the patients who received adjuvant megestrol 160mg/day with those who didn't.…”

Systemic therapy for endometrial stromal sarcomas: current treatment options

“…Data on the efficacy of AIs for ESS are scarce, with most published studies being case reports [23][24][25][26][27][28][29][30]. There is a report of complete responses lasting for more than 7 and 14 years in two patients with lung metastases treated with aminoglutethimide (500 mg bid) [28].…”

“…There is a report of complete responses lasting for more than 7 and 14 years in two patients with lung metastases treated with aminoglutethimide (500 mg bid) [28]. Another study reported two year disease stabilization in ESS with sex-cord compound, treated with anastrozole and megestrol acetate [25].…”