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Table 1 Features of uterine TFE3 translocation associated PEComa

From: TFE3-associated perivascular epithelioid cell tumor with complete response to mTOR inhibitor therapy: report of first case and literature review

  Year Age (years) Site Clinical features Past h/o Size (cm) Pathology Treatment Follow-up Outcome
1 Cho 2008 [13] 9 Uterus, lower uterine segment Vaginal spotting, metastases to pelvic lymph nodes at presentation None 5 Alveolar, epitheliod TAH+pelvic LN dissection ALL occured at 25 months Died at 33 months because of ALL, no e/o recurrence of pecoma at time of death
2 Liu 2014 [14] 34 Cervix AUB None 9 Sheets/ alveolus/nests Resection of cervical mass 5 months Alive
3 Schoolmester 2015 [5] 53 Uterine corpus AUB None 17 Sheet like nested Supracervical hysterectomy, RSO 2 months: cervix and metastases to omentum treated by radical trachelectomy, upper vaginectomy, omentectomy and adjuvant chemotherapy 11 months: small and large intestine and intraabomdinal cavity treated by debulking and adjuvant chemotherapy Alive; intraabdominal recurrence led to diagnosis revision from high grade LMS to pecoma; recently started sirolimus regimen
4 Schoolmester 2015 [5] 49 Uterine corpus Uterine mass Hodgkin lymphoma treated with ABVD chemotherapy (6 years prior) 33 Nested TAH-BSO 25 months Recurrence -none Alive, ned
5 Schoolmester 2015 [5] 47 Pelvis, site not identified Pelvic pain Morcellated supracervical hysterectomy with cellular leiomyomata (1 year prior) 8 Nested Local excision of pelvic mass, radical trachelectomy, bso, pelvic and paraaortic lymphadenopathy, omentectomy, staging biopsies 57 months Recurrence-urinary bladder treated by excision Alive, ned
6 Schoolmester 2015 [5] 46 Uterine corpus Unknown None 1 Nested Hysterectomy 1 month
Recurrence- none
Alive. Ned
7 Choi 2016 [15] 67 Uterus AUB Ns 6 Spindle cells TAH+ BSO Ns
Multiple metastasis in lung and liver
8 Bennet 2018 [6] Ns Uterus Ns Ns Ns Nested Ns 19 months Alive
9 Gianella 2020 [11] 45 Uterus Cyclic abdominopelvic pain and chronic constipation K/c/o breast cancer, treated with quadrantectomy, axillary dissection, and radiotherapy,
Followed by tamoxifen therapy for five years
4 Nested architecture with thin-walled vascular spaces and was Composed of large cells with a clear to granular eosinophilic cytoplasm, round to ovoid nucleus, and Prominent nucleoli TLH with a bilateral salpingectomy. 2 years, no recurrence Alive
10 Hu 2020 [16] 53 Uterine endom etrial polyp Irregular menstruation K/c/o ca breast, h/o MRM f/b tamoxifen x 4 years 2 Epitheliod cells with nested architecture TLH 5months Alive
  1. Note: TAH total abdominal hysterectomy, TLH total laproscopic hysterectomy, RSO right salpingoopherectomy, AUB abnormal uterine bleeding, ALL acute lymphocytic leukemia, LMS liomyosarcoma, NS not specified, MRM modified radical mastectomy, BSO bilateral salphingoophorectomy, NED no evidence of disease