Table 1 Summary of clinicopathologic characteristics of the two cases of uterine PEComa
Characteristics | Case 1 (uterine PEComa, malignant) | Case 2 (uterine PEComa, benign) |
|---|---|---|
Age (year) | 62 | 38 |
Association of TSC | No | Yes |
Synchronous cancer | No | Yes (epithelial ovarian cancer) |
Initial presentation | Abnormal uterine bleeding | No |
Tumor site/size (cm) | Myometrial mass in uterus/4.6Â cm; anterior mass in vaginal wall/1.2Â cm | Submucosal mass in uterus/4.6Â cm |
Extent of disease | Involvement of both lung as well as anterior vagina | Involvement of peri-aortic lymph nodes |
Radiologic findings (MRI) | Degenerative fibroid or leiomyosarcoma | Leiomyoma |
Histologic findings | Epithelioid; infiltrative; tumor necrosis present; cytologic atypia present; high mitotic activity (4/10HPFs) | Epithelioid; focal Infiltrative; major cytological or nuclear pleomorphism absent; low mitotic rate; |
 Positive IHC profile | HMB-45, SMA | HMB-45, SMA |
 Negative IHC profile | PAN-CK, desmin, S-100, melan-A, EMA, CD10 | PAN-CK, desmin, S-100, melan-A, EMA, CD10, c-kit |
Treatment | ||
 Surgical | Hysterectomy; BSO; partial omentectomy; appendectomy; mass resection in the anterior vagina | Hysterectomy; BSO; infracolic omentectomy; pelvic LN dissection; para-aortic LN dissection; appendectomy |
 Adjuvant | mTOR inhibitor—everolimus (weekly, ongoing) | Paclitaxel and carboplatin CTX (6 cycles of tri-weekly) |
Follow-up | Lung and bone metastases, AWD at 18Â months | ANED at 6Â months |