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

Abstract

Objectives: Endocrine therapy is the recommended systemic treatment for steroid receptor positive endometrial stromal sarcoma (ESS). There is no current consensus on the optimal hormonal therapy for ESS. The literature offers several reports on advanced/recurrent/metastatic ESS patients treated with progestins, whereas data on the efficacy of aromatase inhibitors are scarce. Material and methods: We retrospectively identified cases treated for ESS with aromatase inhibitors at our institutions. There were five patients with advanced or unresectable recurrent estrogen, progesterone and androgen receptor-positive ESS, treated with aromatase inhibitors: letrozole or anastrozole (at a daily dose of 2.5 mg and 1 mg, respectively), as first-line endocrine therapy in all but one case treated following progression with megestrol acetate. Results: Disease stabilization was achieved in four cases (80%), including two with long-term progression-free survival for up to 10 years attained under letrozole treatment, and one case after prior progestin treatment. During therapy, no substantial toxicity was observed. Conclusions: Aromatase inhibitors as first-or second-line endocrine treatment achieve disease control in most steroid receptor positive ESS. Our series of cases is evidence of aromatase inhibitors efficacy as long-term endocrine treatment option for ESS patients.

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

“…However, efficacy of progestins such as megesterol acetate, and aromatase inhibitors (AI) have been demonstrated for patients with unresectable advanced disease [6,7]. Owing to its rarity, the data showing objective responses with these agents are derived from case reports and small series, mainly with megesterol acetate and letrozole [8]. Serkies et al, in their retrospective study, identified only 5 cases treated with aromatase inhibitors from two institutions over a 10-year period, which highlights the rarity of this disease [9].…”

“…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].…”

Uterine sarcomas: 7-year experience from a tertiary center

“…National Comprehensive Cancer Network (NCCN) and ESMO guidelines recommend adjuvant CT for high risk patients with uterine sarcoma (6) . Hormonal therapy have been suggested to be efficacious in the treatment of ESS, but there is a lack of data regarding the optimal usage (26) . According to the guideline of DGGG and OEGGG, adjuvant CT should not be generally administered and it should depend on the presence of other risk factors (15) .…”

“…According to the guideline of DGGG and OEGGG, adjuvant CT should not be generally administered and it should depend on the presence of other risk factors (15) . It is also remarkable that the molecular patterns of these tumors are totally different (26,27) . In a large retrospective series including 419 patients with uterine sarcomas, the stage of disease was reported as the most important prognostic factor for all tumor types.…”