Table 2.
Summary of data upon hormonal therapy used for the treatment of uLMS and aggressive angiomyxoma.
| Early Stage | ||||||
| Study (Year) | Type of Study | Number of Patients | Setting | Treatment | Results | Response |
| Slomovitz et al. (2019) [66] | Open-label phase II trial | 4/9 experimental arm | Early stage | AI | NED (3/4) PD (1/4) |
Median PFS: not reached (NR). Progression-free rate at 12 and 24 months: 100% |
| Both early and advanced stage | ||||||
| Study (year) | Type of study | Number of patients | Setting | Treatment | Results | Response |
| Ioffe et al. (2009) [29] | Retrospective study | 3 | Adjuvant | AI | NED | Response duration: 30–50 months |
| 5 | Advanced/ Metastatic |
AI (4/5) TAM (1/5) |
SD (3/4) PR (1/4) SD (1/1) |
Response duration: 18–72 months | ||
| Advanced disease | ||||||
| Study (year) | Type of study | Number of patients | Setting | Treatment | Results | Response |
| George et al. (2014) [67] | Phase II | 27 | Advanced/ Metastatic |
Letrozole | SD (14/27) PFS at 12 weeks: 50% |
PFS at 12 weeks: 50% |
| Thanopoulou et al. (2014) [62] | Retrospective study | 16 | Advanced/ Metastatic |
1st line AI +/− GhRHa |
SD (10/16) PD (4/16) PR (2/16) |
Median PFS: 14 months (95% CI: 0–30 months) |
| 2nd line (6/16) AI +/− GnRHa |
PR (1/6) SD (3/6) PD (2/6) |
Median PFS: not reached. The 1-year progression-free rate for the 2nd line AI was 80%. | ||||
| O’ Cearbhaill et al. (2010) [69] | Retrospective study | 34 | Advanced/ Metastatic |
AI | PR (3/34) SD (11/34) PD (20/34) |
Median PFS: 2.9 months (95% CI: 1.8–5.1). The 1-year PFS rate was 28% (95% CI: 11–48%). |
| Kim et al. (2013) [73] | Case report | 1 | Advanced/ Metastatic |
MPA | SD | PFS: 12 months |
| Fuca et al. (2019) [76] | Retrospective study | 36 | Advanced/ Metastatic |
1st line (13/36) AI +/− GnRHa (11/13) TAM (2/13) |
CR (2/13) PR (4/13) SD (5/13) PR (2/2) |
Overall response rate (ORR) of 8/13 (62%). Median PFS of 24 months (95%, CI). Median time to response: 3 months. 7 out of 13 patients (53.8%) experienced a progression of disease with a median PFS of 24.6 months |
| 2nd line (3/13)AI +/− GnRHa | CR (1/3) PR (1/3) SD (1/3) |
Time to best response range: 2.8–21.9 months; PFS range: 7.3–79.5 months | ||||
MA: megestrol acetate; NED: no evidence of disease; CR: complete response; NSP: not specified progestins; PR: partial response; MPA: medroxyprogesterone acetate; SD: stable disease; AI: aromatase inhibitors; PD: progression of disease; TAM: tamoxifen; GnRHa: gonadotropin-releasing hormone agonist. NA: not available.