Table 1 Features of uterine TFE3 translocation associated PEComa
| Â | 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 | Ns |
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 |