Prolonged response to exemestane following multiple surgical resections and hormonal therapies in a patient with recurrent endometrial stromal sarcoma

Abstract

BackgroundEndometrial stromal sarcomas (ESSs) are rare, indolent tumors with high recurrence rates. Management includes surgery and hormonal therapy given high estrogen and progesterone receptor (ER/PR) expression.CaseA pre-menopausal patient with stage II ESSs (ER +/PR +) underwent primary surgery followed by adjuvant megestrol. Recurrence in the bladder/upper vagina (ER +/PR −) was diagnosed one year later and treated with anterior pelvic exenteration and adjuvant letrozole. Two years later she recurred and was treated with radical surgery and adjuvant exemestane therapy (tumor ER strongly +/PR +). The patient then had a five-year disease free interval before being diagnosed with her third recurrence (ER +).ConclusionExemestane treatment for ESSs can lead to a prolonged response, even in the setting of progression after prior aromatase inhibitor treatment.

Aromatase inhibitor therapy for endometrial stromal sarcoma — two-centre experience

“…This is the longest reported ESS treatment with AIs. Anastrozole has shown clinical benefit, most frequently after progestins, for hormonally sensitive ESS tumors [7,8,18,22]. In another study which included patients with metastatic ESS, positive outcomes with non-steroidal AIs (letrozole or anastrozole) in both, first-(11 cases) and second-(7 cases) line treatment was demonstrated [25].…”

“…In unresectable disease, endocrine therapy is thought to be the treatment of choice since ESS is considered to be relatively radio-and chemo-therapy resistant. Under hormonal therapy, partial response (PR) or disease stabilization (SD) of ESS le- [7][8][9][10][11][12][13][14][15][16][17][18][19][20][21][22][23][24]. AIs were used as first-line or second-line (or later-line) treatment, salvage after prior chemotherapy or adjuvant therapy after complete resection of the metastatic lesions.…”

“…The authors stated that all available hormonal options should be used in sequence in the management of ESS. In the anecdotal reports on exemestane, a well-tolerated type I steroidal irreversible AI, a prolonged response, even in case of progression after prior AI treatment, was observed [8,22]. Based on different mechanisms of action and the lack of cross-resistance between different AIs, hormonal manipulations including continuing different AI therapy despite the failure of prior AI, or the addition of GnRH analogues to AI in ESS tumors that are ER+ seems to be justified.…”

Durable Response to Letrozole in a Patient with Relapsed Low Grade Endometrial Stromal Sarcoma Metastatic to the Lung - A Case Report

“…Therefore, it is not clear if lung metastasis is associated with a more aggressive course or with durable responses to aromatase inhibitors. In the reported literature we identified a few cases with lung metastasis treated with letrozole (Table 1) [6][7][8][9][10][11][12][13][14][15][16][17][18][19].…”

Gynecological Sarcomas: Molecular Characteristics, Behavior, and Histology-Driven Therapy

“…These tumors have a high rate of ER and PgR expression, with several studies reporting the activity of endocrine therapy. [91][92][93] In particular, progestins and AIs have been demonstrated to be effective in controlling metastatic disease. 60 Furthermore, some retrospective data suggest a role for adjuvant therapy.…”