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/4.6 cm; anterior mass in vaginal wall/1.2 cm	Submucosal mass in uterus/4.6 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 18 months	ANED at 6 months

PEComas perivascular epithelioid tumors, TSC tuberous sclerosis complex, MRI magnetic resonance imaging, HPF high power field, IHC immunohistochemical, HMB-45 human melanoma black 45, SMA smooth muscle actin, PAN-CK pan-cytokeratin, EMA epithelial membrane antigen, BSO bilateral salpingo-oophorectomy, CTX chemotherapy, AWD alive with disease, ANED alive with no evidence of disease