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Table 1 Summary of clinicopathologic characteristics of the two cases of uterine PEComa

From: Two cases of perivascular epithelioid cell tumor of the uterus: clinical, radiological and pathological diagnostic challenge

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
  1. 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