TABLE 2.
Cox PH analysis for OS
| Univariable | Multivariable | |||||
|---|---|---|---|---|---|---|
| Variables | N Episodes (%) | HR (95%CI) | P (Cox-Wald) | P (log-rank) | HR (95%CI) | P (Cox-Wald) |
| Sex | ||||||
| Male | 8 (27.6) | 0.4 (0.05–3.1) | 0.4 | 0.3 | ||
| Female | 21 (72.4) | — | — | |||
| Primary site | ||||||
| Uterine | 9 (31.0) | 2.5 (0.6–9.9) | 0.2 | 0.3 | ||
| Extrauterine | 20 (68.9) | — | — | |||
| Age, years (median, range) | 39 (5–71) | 1.0 (0.9–1.1) | 0.3 | 0.3 | ||
| Metastatic at diagnosis | ||||||
| Yes | 7 (24.1) | 6.7 (1.1–41.2) | 0.03 | 0.02 | 5.1 (0.6–40.9) | 0.1 |
| No | 22 (75.9) | |||||
| TSC mutation | ||||||
| TSC2 mutated | 5 (17.2) | 0.8 (0.07–9.8) | 0.9 | 0.57 | ||
| TSC1/TSC2 wild type | 20 (68.9) | 0.4 (0.05–3.7) | 0.4 | |||
| TSC1 mutated | 4 (13.8) | — | — | |||
| TP53 | ||||||
| Mutated | 5 (17.2) | 3.8 (0.9–16.4) | 0.07 | 0.06 | ||
| Wild type | 24 (82.8) | — | — | |||
| Lines of therapy | ||||||
| 1 | 17 (58.6) | — | — | — | — | |
| 2 | 5 (17.2) | 14.4 (2.3–90.0) | 0.004 | 0.0004 | 4.5 (0.4–49.3) | 0.2 |
| ≥3 | 7 (24.1) | 1.1 (0.1–6.5) | 0.9 | 0.2 (0.01–3.8) | 0.3 | |
| TFE3 | ||||||
| Negative | 23 (79.3) | — | — | — | — | |
| Positive | 6 (20.7) | 8.0 (1.9–34.5) | 0.005 | 0.001 | 17.4 (1.5–204.1) | 0.02 |
| Histology | ||||||
| Malignant PEComa | 17 (58.6) | 8.1 (1.0–65.3) | 0.05 | 0.02 | 3.3 (0.1–82.0) | 0.5 |
| LAM/AML/Epithelioid AML | 12 (41.4) | — | — | — | — | |
| History of tuberous sclerosis | ||||||
| Yes | 8 (27.6) | 0.3 (0.04–2.7) | 0.3 | 0.3 | ||
| No | 21 (72.4) | — | — | |||
| Treatment (first line) | ||||||
| mTOR inhibitors | 24 (82.8) | 0.9 (0.2–3.6) | 0.8 | 0.8 | 1.1 (0.04–29.1) | 0.9 |
| Chemotherapy | 5 (17.2) | — | — | |||
Abbreviations: AML, angiomyolipoma; LAM, lymphangioleiomyomatosis; mTOR, mammalian target of rapamycin; PEComa, perivascular epithelioid cell tumors.