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Review

Systematic Analysis of Perivascular Epithelioid Cell Neoplasms in the Female Reproductive Tract: A Comprehensive Review

ORCID Icon, , , &
Pages 283-295 | Received 09 Sep 2023, Accepted 19 Feb 2024, Published online: 01 Mar 2024

References

  • Bonetti F , PeaM, MartignoniG, ZamboniG. PEC and sugar. Am. J. Surg. Pathol.16(3), 307–308 (1992).
  • Pea M , MartignoniG, ZamboniG, BonettiF. Perivascular epithelioid cell. Am. J. Surg. Pathol.20(9), 1149–1153 (1996).
  • Simpson KW , Albores-SaavedraJ. HMB-45 reactivity in conventional uterine leiomyosarcomas. Am. J. Surg. Pathol.31(1), 95–98 (2007).
  • Stone CH , LeeMW, AminMBet al. Renal angiomyolipoma: further immunophenotypic characterization of an expanding morphologic spectrum. Arch. Pathol. Lab. Med.125(6), 751–758 (2001).
  • Hornick JL , FletcherCD. PEComa: what do we know so far?Histopathology48(1), 75–82 (2006).
  • Fernandez-Flores A . Evidence on the neural crest origin of PEComas. Rom. J. Morphol. Embryol.52(1), 7–13 (2011).
  • Ardeleanu C , BussolatiG. Telocytes are the common cell of origin of both PEComas and GISTs: an evidence-supported hypothesis. J. Cell. Mol. Med.15(12), 2569–2574 (2011).
  • Liu R , JiaW, ZouHet al. Expression of CD44 and CD29 by PEComa cells suggests their possible origin of mesenchymal stem cells. Int. J. Clin. Exp. Pathol.8(10), 13023–13033 (2015).
  • Schoolmeester JK , CarlsonJ, KeeneyGLet al. Alveolar soft part sarcoma of the female genital tract: a morphologic, immunohistochemical, and molecular cytogenetic study of 10 cases with emphasis on its distinction from morphologic mimics. Am. J. Surg. Pathol.41(5), 622–632 (2017).
  • Folpe AL , MentzelT, LehrHA, FisherC, BalzerBL, WeissSW. Perivascular epithelioid cell neoplasms of soft tissue and gynecologic origin: a clinicopathologic study of 26 cases and review of the literature. Am. J. Surg. Pathol.29(12), 1558–1575 (2005).
  • Yamada Y , YamamotoH, OhishiYet al. Sclerosing variant of perivascular epithelioid cell tumor in the female genital organs. Pathol. Int.61(12), 768–772 (2011).
  • Bennett JA , BragaAC, PintoAet al. Uterine PEComas: a morphologic, immunohistochemical, and molecular analysis of 32 tumors. Am. J. Surg. Pathol.42(10), 1370–1383 (2018).
  • Hornick JL , FletcherCD. Sclerosing PEComa: clinicopathologic analysis of a distinctive variant with a predilection for the retroperitoneum. Am. J. Surg. Pathol.32(4), 493–501 (2008).
  • Babayev E , FayKE, HorowitzJMet al. Perivascular epithelioid cell tumors (PEComa) in pregnancy with uterine rupture and ongoing abdominal gestation: a case report. Case Rep. Womens Health25, e00172 (2020).
  • Nguyen JMV , GhandehariH, Parra-HerranC, VicusD. Uterine rupture: an unusual presentation of a uterine perivascular epithelioid cell tumor (PEComa). Int. J. Gynecol. Cancer30(12), 2008–2011 (2020).
  • Ruco LP , PilozziE, WedardBMet al. Epithelioid lymphangioleiomyomatosis-like tumour of the uterus in a patient without tuberous sclerosis: a lesion mimicking epithelioid leiomyosarcoma. Histopathology33(1), 91–93 (1998).
  • Bosincu L , RoccaPC, MartignoniGet al. Perivascular epithelioid cell (PEC) tumors of the uterus: a clinicopathologic study of two cases with aggressive features. Mod. Pathol.18(10), 1336–1342 (2005).
  • Theofanakis C , ThomakosN, SotiropoulouM, RodolakisA. Perivascular epithelioid cell tumor of the uterus: report of two cases and mini-review of the literature. Int. J. Surg. Case Rep.28, 85–87 (2016).
  • Rothenberger R , JacksonA, KendlerA, HerzogT, BillingsleyC. An unusual case of uterine PEComa presenting with disseminated intravascular coagulation. Gynecol. Oncol. Rep.29, 76–78 (2019).
  • Gentile M , ZinnaM, ZanellaCet al. Uterine PEComa with aggressive behavior: a review with an additional case of spontaneous vaginal expulsion. Pathol. Res. Pract.216(6), 152991 (2020).
  • Giannella L , DelliCarpini G, MontikNet al. Ultrasound features of a uterine perivascular epithelioid cell tumor (PEComa): case report and literature review. Diagnostics (Basel)10(8), (2020).
  • Yamagata Y , KawauchiS, TamuraH, MurakamiA, SasakiK, SuginoN. A case of HMB45-negative perivascular epithelioid cell tumor (PEComa) of the uterine corpus: a possible diagnostic application of molecular-cytogenetic analysis. Eur. J. Gynaecol. Oncol.30(2), 216–219 (2009).
  • Poomtavorn Y , WarnnissornN, WarnnissornM, BoonyarangkulA. Caesarean section unmasking perivascular epithelioid cell tumour of the uterus. J. Obstet. Gynaecol.34(5), 441–442 (2014).
  • Nitahara K , SasakiM, IchikawaS, TsujiK, YoshidaY. Rupture of perivascular epithelioid cell neoplasm at 34 weeks’ gestation: a nonendometriosis case of spontaneous hemoperitoneum in pregnancy. J. Obstet. Gynaecol. Res.45(3), 709–713 (2019).
  • Tilstra M , MaksemJ, BakhruA, CarlanSJ, WilsonJ. Perivascular epithelioid cell tumor masquerading as retained placenta. J. Ultrasound Med.34(8), 1515–1517 (2015).
  • Fadare O . Perivascular epithelioid cell tumor (PEComa) of the uterus: an outcome-based clinicopathologic analysis of 41 reported cases. Adv. Anat. Pathol.15(2), 63–75 (2008).
  • Bleeker JS , QuevedoJF, FolpeAL. Malignant perivascular epithelioid cell tumor of the uterus. Rare Tumors4(1), e14 (2012).
  • Schoolmeester JK , HowittBE, HirschMS, DalCin P, QuadeBJ, NucciMR. Perivascular epithelioid cell neoplasm (PEComa) of the gynecologic tract: clinicopathologic and immunohistochemical characterization of 16 cases. Am. J. Surg. Pathol.38(2), 176–188 (2014).
  • Conlon N , SoslowRA, MuraliR. Perivascular epithelioid tumours (PEComas) of the gynaecological tract. J. Clin. Pathol.68(6), 418–426 (2015).
  • Vang R , KempsonRL. Perivascular epithelioid cell tumor (‘PEComa’) of the uterus: a subset of HMB-45-positive epithelioid mesenchymal neoplasms with an uncertain relationship to pure smooth muscle tumors. Am. J. Surg. Pathol.26(1), 1–13 (2002).
  • Yang YP , ZhuYL, WuWP, WangZM, ZhangJM. [Perivascular epithelioid cell tumor of uterus: report of 5 cases and literature review]. Zhonghua Bing Li Xue Za Zhi36(5), 302–306 (2007).
  • Fadare O , ParkashV, YilmazYet al. Perivascular epithelioid cell tumor (PEComa) of the uterine cervix associated with intraabdominal “PEComatosis”: a clinicopathological study with comparative genomic hybridization analysis. World J. Surg. Oncol.2, 35 (2004).
  • Shan W , ShiY, ZhuQet al. Five cases of uterine perivascular epithelioid cell tumors (PEComas) and review of literature. Arch. Gynecol. Obstet.299(1), 185–190 (2019).
  • Gu J , WangW, WangS. A retrospective case study of 13 uterine perivascular epithelioid cell neoplasm (PEComa) patients. Onco Targets Ther.14, 1783–1790 (2021).
  • Fang CL , LinYH, ChenWY. Microscopic endometrial perivascular epithelioid cell nodules: a case report with the earliest presentation of a uterine perivascular epithelioid cell tumor. Diagn. Pathol.7, 117 (2012).
  • Folpe AL , KwiatkowskiDJ. Perivascular epithelioid cell neoplasms: pathology and pathogenesis. Hum. Pathol.41(1), 1–15 (2010).
  • Fadare O . Uterine PEComa: appraisal of a controversial and increasingly reported mesenchymal neoplasm. Int. Semin. Surg. Oncol.5, 7 (2008).
  • Liu CH , ChaoWT, LinSC, LauHY, WuHH, WangPH. Malignant perivascular epithelioid cell tumor in the female genital tract: preferred reporting items for systematic reviews and meta-analyses. Medicine (Baltimore)98(2), e14072 (2019).
  • Gulavita P , FletcherCDM, HirschMS. PNL2: an adjunctive biomarker for renal angiomyolipomas and perivascular epithelioid cell tumours. Histopathology72(3), 441–448 (2018).
  • Valencia-Guerrero A , PintoA, AndersonWJ, TrevisanG, NucciMR, HirschMS. PNL2: a useful adjunct biomarker to HMB45 in the diagnosis of uterine perivascular epithelioid cell tumor (PEComa). Int. J. Gynecol. Pathol.39(6), 529–536 (2020).
  • Horn LC , EinenkelJ. Uterine perivascular epitheloid cell tumor (PEComa) with CD117 and PNL2 positivity and entrapped endometriotic glands, mimicking sex-cordlike differentiation. Ann. Diagn. Pathol.15(3), 216–218 (2011).
  • Adachi Y , HorieY, KitamuraYet al. CD1a expression in PEComas. Pathol. Int.58(3), 169–173 (2008).
  • Fadare O , LiangSX. Epithelioid smooth muscle tumors of the uterus do not express CD1a: a potential immunohistochemical adjunct in their distinction from uterine perivascular epithelioid cell tumors. Ann. Diagn. Pathol.12(6), 401–405 (2008).
  • Ahrens WA , FolpeAL. CD1a immunopositivity in perivascular epithelioid cell neoplasms: true expression or technical artifact? A streptavidin-biotin and polymer-based detection system immunohistochemical study of perivascular epithelioid cell neoplasms and their morphologic mimics. Hum. Pathol.42(3), 369–374 (2011).
  • Park SH , RoJY, KimHS, LeeES. Perivascular epithelioid cell tumor of the uterus: immunohistochemical, ultrastructural and molecular study. Pathol. Int.53(11), 800–805 (2003).
  • Gao Z , BhuiyaT, AndersonA. Perivascular epithelioid cell tumour (PEComa) of the uterus associated with malignant neoplasm of the female genital tract. J. Obstet. Gynaecol.24(5), 600–604 (2004).
  • Shrestha S , MeyersC, ShenJet al. Ang-1 and Ang-2 expression in angiomyolipoma and PEComa family tumors. J Orthop.14(1), 154–160 (2017).
  • Bennett JA , OlivaE. Perivascular epithelioid cell tumors (PEComa) of the gynecologic tract. Genes Chromosomes Cancer60(3), 168–179 (2021).
  • Musella A , DeFelice F, KyriacouAKet al. Perivascular epithelioid cell neoplasm (PEComa) of the uterus: a systematic review. Int. J. Surg.19, 1–5 (2015).
  • Akumalla S , MadisonR, LinDIet al. Characterization of clinical cases of malignant PEComa via comprehensive genomic profiling of DNA and RNA. Oncology98(12), 905–912 (2020).
  • Liu F , ZhangR, WangZYet al. Malignant perivascular epithelioid cell tumor (PEComa) of cervix with TFE3 gene rearrangement: a case report. Int. J. Clin. Exp. Pathol.7(9), 6409–6414 (2014).
  • Agaram NP , SungYS, ZhangLet al. Dichotomy of genetic abnormalities in PEComas with therapeutic implications. Am. J. Surg. Pathol.39(6), 813–825 (2015).
  • Schoolmeester JK , DaoLN, SukovWRet al. TFE3 translocation-associated perivascular epithelioid cell neoplasm (PEComa) of the gynecologic tract: morphology, immunophenotype, differential diagnosis. Am. J. Surg. Pathol.39(3), 394–404 (2015).
  • Tan Y , ZhangH, XiaoEH. Perivascular epithelioid cell tumour: dynamic CT, MRI and clinicopathological characteristics – analysis of 32 cases and review of the literature. Clin. Radiol.68(6), 555–561 (2013).
  • Tirumani SH , ShinagareAB, HargreavesJet al. Imaging features of primary and metastatic malignant perivascular epithelioid cell tumors. AJR Am. J. Roentgenol.202(2), 252–258 (2014).
  • Pattamapaspong N , KhunamornpongS, PhongnarisornC, PojchamarnwiputhS. Malignant perivascular epithelioid cell tumour of the round ligament mimics leiomyoma on computed tomography. Singapore Med. J.50(7), e239–e242 (2009).
  • Mor E , VisenziC, MarasciuloF, LongoFR, QuagliaF. Ultrasound appearance of perivascular epithelioid cell tumor (PEComa). Ultrasound Obstet. Gynecol.57(5), 844–845 (2021).
  • Nishio N , KidoA, MinamiguchiSet al. MR findings of uterine PEComa in patients with tuberous sclerosis: report of two cases. Abdom. Radiol. (NY)44(4), 1256–1260 (2019).
  • Yoshizawa H , KawaiS, HirotaY, UedaT, KurodaM, FujiiT. A case of uterine lymphangioleiomyomatosis in a young woman that was identified via peritonitis. J. Obstet. Gynaecol. Res.45(2), 482–486 (2019).
  • Zamecnik M , MichalM. HMB45+ hyalinized epithelioid tumor of the uterus is linked to epithelioid leiomyoma rather than to PEC-omas. Int. J. Surg. Pathol.9(4), 341–343 (2001).
  • Silva EG , DeaversMT, BodurkaDC, MalpicaA. Uterine epithelioid leiomyosarcomas with clear cells: reactivity with HMB-45 and the concept of PEComa. Am. J. Surg. Pathol.28(2), 244–249 (2004).
  • Hurrell DP , MccluggageWG. Uterine leiomyosarcoma with HMB45+ clear cell areas: report of two cases. Histopathology47(5), 540–542 (2005).
  • Yavuz E , CakrC, TuzlalS, AhskalB, TopuzS, IlhanR. Uterine perivascular epithelioid cell tumor coexisting with pulmonary lymphangioleiomyomatosis and renal angiomyolipoma: a case report. Appl. Immunohistochem. Mol. Morphol.16(4), 405–409 (2008).
  • Wang YY , ShiQF. [Endometrial stromal sarcoma complicating uterine perivascular epithelioid cell tumor: report of a case]. Zhonghua Bing Li Xue Za Zhi42(5), 345–346 (2013).
  • Dimmler A , SeitzG, HohenbergerW, KirchnerT, FallerG. Late pulmonary metastasis in uterine PEComa. J. Clin. Pathol.56(8), 627–628 (2003).
  • Greene LA , MountSL, SchnedAR, CooperK. Recurrent perivascular epithelioid cell tumor of the uterus (PEComa): an immunohistochemical study and review of the literature. Gynecol. Oncol.90(3), 677–681 (2003).
  • Armah HB , ParwaniAV. Malignant perivascular epithelioid cell tumor (PEComa) of the uterus with late renal and pulmonary metastases: a case report with review of the literature. Diagn. Pathol.2, 45 (2007).
  • Gadducci A , ZannoniGF. Perivascular epithelioid cell tumors (PEComa) of the female genital tract: a challenging question for gynaecologic oncologist and pathologist. Gynecol. Oncol. Rep.33, 100603 (2020).
  • Ciarallo A , MakisW, HickesonM, DerbekyanV. Malignant perivascular epithelioid cell tumor (PEComa) of the uterus: serial imaging with F-18 FDG PET/CT for surveillance of recurrence and evaluation of response to therapy. Clin. Nucl. Med.36(4), e16–e19 (2011).
  • Phongnarisorn C , KhunamornpongS, PattamapaspongN, SrisomboonJ. Laparoscopic radical excision of primary round ligament perivascular epithelioid cell tumor mimicking leiomyoma. J. Minim. Invasive Gynecol.16(5), 626–629 (2009).
  • Yamamoto E , InoK, SakuraiM, TakigawaS, IwaseA, KikkawaF. Fertility-sparing operation for recurrence of uterine cervical perivascular epithelioid cell tumor. Rare Tumors2(2), e26 (2010).
  • Jeon IS , LeeSM. Multimodal treatment using surgery, radiotherapy, and chemotherapy in a patient with a perivascular epithelioid cell tumor of the uterus. J. Pediatr. Hematol. Oncol.27(12), 681–684 (2005).
  • Liu JL , LinYM, LinMC, YehKT, HsuJC, ChinCJ. Perivascular epithelioid cell tumor (PEComa) of the uterus with aggressive behavior at presentation. Hematol. Oncol. Stem Cell Ther.2(3), 426–430 (2009).
  • Italiano A , DelcambreC, HosteinIet al. Treatment with the mTOR inhibitor temsirolimus in patients with malignant PEComa. Ann. Oncol.21(5), 1135–1137 (2010).
  • Starbuck KD , DrakeRD, BuddGT, RosePG. Treatment of advanced malignant uterine perivascular epithelioid cell tumor with mTOR Inhibitors: single-institution experience and review of the literature. Anticancer Res.36(11), 6161–6164 (2016).
  • Ross C , SharmaS, LoucaO, ScurrM, HayesA, JudsonI. A patient presenting with a perivascular epithelioid cell tumor in the broad ligament: a case report. J. Med. Case Rep.5, 383 (2011).
  • Bunch K , SundeJ. Fertility sparing treatment of a malignant uterine perivascular epithelioid cell tumor: a case report. Gynecol. Oncol. Case Rep.8, 14–16 (2014).
  • Gao F , HuangC, ZhangYet al. Combination targeted therapy of VEGFR inhibitor, sorafenib, with an mTOR inhibitor, sirolimus induced a remakable response of rapid progressive uterine PEComa. Cancer Biol. Ther.17(6), 595–598 (2016).
  • Kopparthy P , MurphyM. Rapid and durable response with nab-sirolimus after everolimus failure in a patient with perivascular epithelioid cell tumors (PEComas) of the uterus. Cureus13(5), e14951 (2021).
  • Liapi A , MathevetP, HerreraFG, HastirD, SarivalasisA. VEGFR inhibitors for uterine metastatic perivascular epithelioid tumors (PEComa) resistant to mTOR inhibitors. A case report and review of literature. Front. Oncol.11, 641376 (2021).
  • Prizant H , TayaM, LermanIet al. Estrogen maintains myometrial tumors in a lymphangioleiomyomatosis model. Endocr. Relat. Cancer23(4), 265–280 (2016).

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