Review Article

“Malignant” Perivascular Epithelioid Cell Neoplasm: Risk Stratification and Treatment Strategies

Table 5

Reported cases of non-surgical treatment of PEComa-NOS.

Reference/yearAge/SexLocationSize (cm)Folpe cat.ManagementOutcomeTreatment details

Neoadjuvant cases
Folpe et al. [7]49 FShoulderN/AMalignantNA CT, resectionNED at 11 moIfos/Adria—residual dz at resection
Jeon and Sung [34] and Jeon and Yi [35]9 FUterus6.5MalignantNA CT, resection, Adj CTNED at 16 mo; ALL diagnosed at 16 moVincr/Ifos/Adria—2 NA cycles, 6 adj cycles concurrent with 45 Gy.
Ong et al. [36]8 FVagina2UnknownNA CT, resectionNED at 6 moIfos/Vincr/Actino D x3—prog
Osei et al. [37]49 FUE soft tissue5.3MalignantSequential NA CT then RT followed by resectionSingle lung metastasis at 14 mo—resectedIfos/Adria x6—80% reduction, then 50 Gy RT—prog
Weinreb et al. [38]68 MThigh7.8MalignantNA RT, resectionNED at 11 mo
Yamashita et al. [39]39 FTibia6.5MalignantNA RT, resectionNED at 34 mo

Adjuvant Cases
Bosincu et al. [40]48 FUterus7MalignantResection, Adj HTNED at 36 moTamoxifen
Chen et al. [41]16 FAbdominal27MalignantResection, Adj CTRecurrence at 2 mo; no further follow-up(+) LN at surgery
Fink et al. [42]51 FBroad Lig17UnknownResection, Adj RTNED at 15 mo50.4 Gy
Folpe et al. [43]29 MFalciform Lig20MalignantResection, Adj RTLung metastases at 3 mo; dead of other causes at 1 yr
Folpe et al. [43]10 FFalciform Lig5UnknownResection, Adj CTLost to follow-upVincr, Actino D, Cyclophos
Folpe et al. [7]71 MForearm9MalignantResection, Adj RTNED at 10 mo
Folpe et al. [7]48 FCervix2UnknownResection, Adj RTNED at 21 mo
Folpe et al. [7]77 FNeck2.6UnknownResection, Adj RTNED at 6 mo
Folpe et al. [7]56 FUterus9MalignantResection, Adj CT and RTLung and bony metastases at 11 mo; alive at 11 mo
Folpe et al. [7]59 FUterus14.5MalignantResection, Adj CTLiver and lung metastases at 30 mo; alive at 30 mo
Folpe et al. [7]46 FMesentery12MalignantResection, Adj CTIntra-abdominal and liver metastases at 22 mo; DOD at 27 mo“Multiple” cycles of adjuvant CT
Folpe et al. [7]36 FUterusN/AMalignantResection, Adj CTLung metastases at 12 mo, liver metastases at 36 mo; DOD at 39 mo
Hornick and Fletcher [11]50 FPelvis13MalignantResection, Adj RTLung, liver, abdominal wall metastases at 39 mo, alive at 46 mo
Pan et al. [20]46 MProstate8.5MalignantResection, Adj RTPulmonary metasases at 3 yrs, DOD at 4 yrsCis/Adria x6 cycles
Parfitt et al. [44]48 MBladder3UnknownResection, Adj ITNED at 48 moIFN-α
Ryan et al. [45]15 FRectum3.7MalignantResection, Adj CTNED at 9 mo(+) LN at surgery; Ifos/Adria
Silva et al. [4]76 FUterusN/AMalignantResection, Adj RTNED at 8 mo
Subbiah et al. [46]58 FRetroperitoneal17UnknownResection, Adj CTLiver metastases at 2 yrs, resected with subsequent liver and lung metastases2 cycles adj Adria; RT/adria after resection of liver recurrence; temsirolimus/bortezomib at 2nd recurrence—prog
Vang and Kempson [47]75 FUterus5UnknownResection, Adj RTNED at 30 mo

Metastatic/Recurrent Cases
Bonetti et al. [48]19 FUterus5.5MalignantSubtotal resection of primary, CT followed by RTLocal recurrence at 10 mo, diffuse metastatic disease at 18 mo
Léon et al. [49]76 FAbdominal15MalignantRT to sacral recurrenceProgressive metastatic disease13 Gy RT to sacrum
Léon et al. [49]38 FRetroperitonealN/AMalignantCRTDOD at 20 moRT/Thal
Fukunaga et al. [50]40 FUterus30MalignantDebulking surgery, CRTDOD at 17 moMulitple omental metastases at initial surgery
Greene et al. [51]79 FUterus13MalignantDebulking of pelvic recurrence, CTDOD at “several” moPaclitaxel x1 cycle
Italiano et al. [52]55 FUterusN/AUnknownResection of recurrence, CT, CRT following 2nd recurrence, mTOR following progressionProgressive metastatic disease; alive at 16 mo following recurrenceIfos/Adria, RT+etop, Temsirolimus, Gem
Italiano et al. [52]69 FUterusN/AUnknownmTOR, resection of solitary lung metastasisNED at 9 moTemsirolimus; response allowed surgical resection
Peng et al. [53]47 MPelvis12MalignantSubtotal resection of primary, CTDOD at 9 moIfos/Epi x2—prog; Adria/Oxali/Thal x 2—prog; Nada, Dacarb x2—prog
Silva et al. [4]47 FUterusN/AMalignantResection of primary; RT followed by CTDOD at 30 moLung metastases at diagnosis; Adria/Cis x8 cycles
Silva et al. [4]73 FUterusN/AMalignantResection of primary; CTDOD at 9 moPaclitaxel x6 cycles
Silva et al. [4]43 FUterusN/AMalignantResection of primary; CTAlive with progressive disease at 6 moIfos/Adria x5 cycles
Wagner et al. [54]70 MKidney9UnknownTKI, mTOR inhibitor, resection of recurrence40% reduction in size of isolated recurrence, NED after resectionSunitinib, sirolimus
Wagner et al. [54]65 MRetroperitoneal20MalignantResection of recurrences, mTOR inhibitor“Near complete dissapperance” of multifocal disease at 12 mo, alive at 16 mo following recurrenceSirolimus
Wagner et al. [54]61 FCervix9MalignantmTOR inhibitorInitial response, DOD at 8 moSirolimus
Yamashita et al. [39]35 MVertebral1.8MalignantCRTBone metastases at 9 mo, alive at 12 moPelvic mets—no tx details
Yamashita et al. [39]42 FUterus15MalignantResection of humeral recurrence, CTDOD at 10 mo with diffuse metastases

Legend: NA: Neoadjuvant, Adj: Adjuvant, CT: Chemotherapy, RT: Radiation therapy, IT: Immunotherapy, HT: Hormonal therapy, NED: No evidence of disease, DOD: Dead of disease, Ifos: Ifosfamide, Adria: Doxorubicin, Vincr: Vincristine, Actino D: Actinomycin D, CTX: Cyclophosphamide, Cis: Cisplatin, Thal: Thalidomide, Epi: Epirubicin, Oxali: Oxaliplatin, Nada: Nadplatin, Dacarb: Dacarbazine, Etop: Etoposide, Gem: Gemcitabine, TKI: Tyrosine kinase inhibitor.