The Journal of Minimally Invasive Gynecology
Volume 16, Issue 5 , Pages 527-532 , September 2009

Perivascular Epithelioid Cell Neoplasms: A Systematic Review of Prognostic Factors

  • Nazila Zekry, MD

      Affiliations

    • Hoag Memorial Hospital, Department of Pathology, Newport Beach, CA
    • ,
  • Mark A. Rettenmaier, MD

      Affiliations

    • Gynecologic Oncology Associates, Hoag Cancer Center, Newport Beach, CA
    • ,
  • Lisa N. Abaid, MD

      Affiliations

    • Gynecologic Oncology Associates, Hoag Cancer Center, Newport Beach, CA
    • ,
  • Cameron R. John, PhD

      Affiliations

    • Department of Behavioral Sciences, Utah Valley University, Orem, UT
    • ,
  • John P. Micha, MD

      Affiliations

    • Gynecologic Oncology Associates, Hoag Cancer Center, Newport Beach, CA
    • ,
  • John V. Brown III, MD

      Affiliations

    • Gynecologic Oncology Associates, Hoag Cancer Center, Newport Beach, CA
    • ,
  • Bram H. Goldstein, PhD

      Affiliations

    • Gynecologic Oncology Associates, Hoag Cancer Center, Newport Beach, CA
    • Corresponding Author InformationCorresponding author: Bram Goldstein, PhD, Gynecologic Oncology Associates, 351 Hospital Road, Suite 507, Newport Beach, CA 92663.

Received 24 February 2009 ,Accepted 30 April 2009.

References 

  1. Silva EG, Deavers MT, Bodurka DC, Malpica A. Uterine epithelioid leiomyosarcomas with clear cells: reactivity with HMB-45 and the concept of PEComa. Am J Surg Pathol. 2004;28:244–249
  2. Hornick JL, Fletcher CD. Sclerosing PEComa: clinicopathologic analysis of a distinctive variant with a predilection for the retroperitoneum. Am J Surg Pathol. 2008;32:493–501
  3. Fadare O, Parkash V, Yilmaz Y, et 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. 2004;2:35–46
  4. Bonetti F, Martignoni G, Colato C, et al. Abdominopelvic sarcoma of perivascular epithelioid cells. Report of four cases in young women, one with tuberous sclerosis. Mod Pathol. 2001;14:563–568
  5. Jeon IS, Lee SM. Multimodal treatment using surgery, radiotherapy, and chemotherapy in a patient with a perivascular epithelioid cell tumor of the uterus. J Pediatr Hematol Oncol. 2005;27:681–684
  6. Baek JH, Chung MG, Jung DH, Oh JH. Perivascular epithelioid cell tumor (PEComa) in the transverse colon of an adolescent: a case report. Tumori. 2007;93:106–108
  7. Vang R, Kempson RL. 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. 2002;26:1–13
  8. Agaimy A, Wünsch PH. Perivascular epithelioid cell sarcoma (malignant PEComa) of the ileum. Pathol Res Pract. 2006;202:37–41
  9. Prasad SR, Sahani DV, Mino-Kenudson M, et al. Neoplasms of the perivascular epithelioid cell involving the abdomen and the pelvis: cross-sectional imaging findings. J Comput Assist Tomogr. 2007;31:688–696
  10. Folpe AL, Mentzel T, Lehr HA, Fisher C, Balzer BL, Weiss SW. 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. 2005;29:1558–1575
  11. Bernardo Vega R, Camino FV, Frenandez MDC, Segura GQ, Gonzales JM, Gonzales-Tejero C. The PEComa tumor: Could it be considered an independent neoplastic entity?. J Gynecol Surg. 2005;21:161–166
  12. Pea M, Martignoni G, Zamboni G, Bonetti F. Perivascular epithelioid cell. Am J Surg Pathol. 1996;20:1149–1153
  13. Fadare O. Perivascular epithelioid cell tumor (PEComa) of the uterus: an outcome-based clinicopathologic analysis of 41 reported cases. Adv Anat Pathol. 2008;15:63–75
  14. Osei DA, Alvandi F, Brooks JS, Ogilvie CM. PEComa of the upper extremity: a unique case and description of an initial response to neoadjuvant chemotherapy. Sarcoma. 2007;2007:53056
  15. Weinreb I, Howarth D, Latta E, Ghazarian D, Chetty R. Perivascular epithelioid cell neoplasms (PEComas): four malignant cases expanding the histopathological spectrum and a description of a unique finding. Virchows Arch. 2007;450:463–470
  16. Liegl B, Hornick JL, Fletcher CD. Primary cutaneous PEComa: distinctive clear cell lesions of skin. Am J Surg Pathol. 2008;32:608–614
  17. Kim HJ, Lim SJ, Choi H, Park K. Malignant clear-cell myomelanocytic tumor of broad ligament—a case report. Virchows Arch. 2006;448:867–870
  18. Ong LY, Hwang WS, Wong A, Chan MY, Chui CH. Perivascular epithelioid cell tumour of the vagina in an 8 year old girl. J Pediatr Surg. 2007;42:564–566
  19. Fink D, Marsden DE, Edwards L, Camaris C, Hacker NF. Malignant perivascular epithelioid cell tumor (PEComa) arising in the broad ligament. Int J Gynecol Cancer. 2004;14:1036–1039
  20. Pan CC, Jong YJ, Chai CY, Huang SH, Chen YJ. Comparative genomic hybridization study of perivascular epithelioid cell tumor: molecular genetic evidence of perivascular epithelioid cell tumor as a distinctive neoplasm. Hum Pathol. 2006;37:606–612
  21. Parfitt JR, Bella AJ, Wehrli BM, Izawa JI. Primary PEComa of the bladder treated with primary excision and adjuvant interferon-alpha immunotherapy: a case report. BMC Urol. 2006;6:20
  22. Lau SK, Marchevsky AM, McKenna RJ, Luthringer DJ. Malignant monotypic epithelioid angiomyolipoma of the retroperitoneum. Int J Surg Pathol. 2003;11:223–228
  23. Armah HB, Parwani AV. 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. 2007;2:45
  24. Dimmler A, Seitz G, Hohenberger W, Kirchner T, Faller G. Late pulmonary metastasis in uterine PEComa. J Clin Pathol. 2003;56:627–628
  25. Azad NS, Aziz AB, Pervez S, Kayani N. Uterine perivascular epithelioid cell tumour presenting as a cervical mass. J Pak Med Assoc. 2006;56:83–84
  26. Gao Z, Bhuiya T, Anderson A. Perivascular epithelioid cell tumour (PEComa) of the uterus associated with malignant neoplasm of the female genital tract. J Obstet Gynaecol. 2004;24:600–604
  27. Gan MF, Jin M, Yu CK, Cai JF. Perivascular epithelioid cell tumor (PEComa) of the uterine. Zhonghua Bing Li Xue Za Zhi. 2006;35:314–315
  28. Fukunaga M. Perivascular epithelioid cell tumor of the uterus: report of four cases. Int J Gynecol Pathol. 2005;24:341–346
  29. Darai E, Bazot M, Barranger E, Detchev R, Cortez A. Epithelioid angiomyolipoma of the uterus: a case report. J Reprod Med. 2004;49:578–581
  30. Park HK, Zhang S, Wong MK, Kim HL. Clinical presentation of epithelioid angiomyolipoma. Int J Urol. 2007;14:21–25
  31. Greene LA, Mount SL, Schned AR, Cooper K. Recurrent perivascular epithelioid cell tumor of the uterus (PEComa): an immunohistochemical study and review of the literature. Gynecol Oncol. 2003;90:677–681
  32. Michal M, Zamecnik M. Hyalinized uterine mesenchymal neoplasms with HMB-45-positive epithelioid cells: epithelioid leiomyomas or angiomyolipomas? Report of four cases. Int J Surg Pathol. 2000;8:323–328
  33. Ruco LP, Pilozzi E, Wedard BM, et al. Epithelioid lymphangioleiomyomatosis-like tumour of the uterus in a patient without tuberous sclerosis: a lesion mimicking epithelioid leiomyosarcoma. Histopathology. 1998;33:91–93
  34. Anderson AE, Yang X, Young RH. Epithelioid angiomyolipoma of the ovary: a case report and literature review. Int J Gynecol Pathol. 2002;21:69–73
  35. Evert M, Wardelmann E, Nestler G, Schulz HU, Roessner A, Röcken C. Abdominopelvic perivascular epithelioid cell sarcoma (malignant PEComa) mimicking gastrointestinal stromal tumour of the rectum. Histopathology. 2005;46:115–117
  36. Walter AW, Shearer PD, Pappo AS, et al. A pilot study of vincristine, ifosfamide, and doxorubicin in the treatment of pediatric non-rhabdomyosarcoma soft tissue sarcomas. Med Pediatr Oncol. 1998;30:210–216
  37. Liang SX, Pearl M, Liu J, Hwang S, Tornos C. “Malignant” uterine perivascular epithelioid cell tumor, pelvic lymph node lymphangioleiomyomatosis, and gynecological pecomatosis in a patient with tuberous sclerosis: a case report and review of the literature. Int J Gynecol Pathol. 2008;27:86–90
  38. LoehrerSr PJ, Sledge GW, Nicaise C, et al., Omura G, Braun TJ Ifosfamide plus doxorubicin in metastatic adult sarcomas: a multi-institutional phase II trial. J Clin Oncol. 1989;7:1655–1659
  39. Bosincu L, Rocca PC, Martignoni G, et al. Perivascular epithelioid cell (PEC) tumors of the uterus: a clinicopathologic study of two cases with aggressive features. Mod Pathol. 2005;18:1336–1342
  40. Rigby H, Yu W, Schmidt MH, Fernandez CV. Lack of response of a metastatic renal perivascular epithelial cell tumor (PEComa) to successive courses of DTIC based-therapy and imatinib mesylate. Pediatr Blood Cancer. 2005;45:202–206
  41. Rammeh Rommani S, Trabelsi A, Attia L, et al. Perivascular epithelioid cell tumor of the uterus: a case report. Pathologica. 2006;98:649–651
  42. Antman K, Crowley J, Balcerzak SP, et al. An intergroup phase III randomized study of doxorubicin and dacarbazine with or without ifosfamide and mesna in advanced soft tissue and bone sarcomas. J Clin Oncol. 1993;11:1276–1285

 The authors have no commercial, proprietary, or financial interest in the products or companies described in this article.

PII: S1553-4650(09)00237-4

doi: 10.1016/j.jmig.2009.04.017

The Journal of Minimally Invasive Gynecology
Volume 16, Issue 5 , Pages 527-532 , September 2009

Article Tools

* Image Usage & Resolution