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Publisher: Peytchinski Publishing Ltd.
ISSN: 1312-773X (Online)
Issue: 2019, vol. 25, issue4
Subject Area: Medicine
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DOI: 10.5272/jimab.2019254.2759
Published online: 11 November 2019
Review article
J of IMAB. 2019 Oct-Dec;25(4):2759-2768;
MALIGNANT CERVICAL PECOMA: A CASE REPORT AND REVIEW OF THE LITERATURE
Svetlana Mateva1
, Margarita Nikolova1, Tihomir Totev2, Grigor Gorchev3,
1) Department of Clinical Pathology, Faculty of Medicine, Medical University - Pleven, Bulgaria
2) Institute of Science and Research, Medical University - Pleven, Bulgaria
3) Saint Marina University Hospital - Pleven, Medical University - Pleven, Bulgaria.
ABSTRACT:
Background: Perivascular epithelioid cell tumors represent a family of mesenchymal tumours characterised by co-expression of melanocytic and muscle markers. The PEComas family of tumours includes angiomyolipoma, lymphangioleiomyoma, clear cell "sugar" tumour of the lung and rare clear cell tumours of various location. The designation PEComa, not otherwise specified, has been applied to these "unusual clear cell tumours" which are rare and mainly located in the uterine body. Most PEComas follow a benign clinical course, but tumours with aggressive behaviour have been increasingly reported. Folpe first proposed criteria for assessment of malignancy in 2005. To the best of our knowledge, 35 uterine non-benign and only 13 cervical PEComas have been reported in the available medical literature in the English language.
Case description: We report a case of 57-year-old woman with malignant cervical PEComa, emphasising the diagnostic challenges. We have tried to evaluate the malignant potential of uterine PEComas according to Folpe criteria.
Keywords: PEComa, smooth muscle epithelioid tumour, tuberous sclerosis complex,
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Please cite this article as: Mateva S, Nikolova M, Totev T, Gorchev G. Malignant Cervical PEComa: A Case Report and Review of the Literature. J of IMAB. 2019 Oct-Dec;25(4):2759-2768. DOI: 10.5272/jimab.2019254.2759
Correspondence to: Svetlana A. Mateva, St. Marina University Hospital, Department of Pathology, Bulgarska aviatsia str. Pleven, 5800, Bulgaria; E-mail: matevasvetlana@gmail.com
REFERENCES:
1. Grimer RJ, Hogendoorn PCW, Vanel D. Neoplasms with perivascular epithelioid cell differentiation (PEComas), in: WHO Classification of Tumours of Soft Tissue and Bone, WHO Classification of Tumours, 4th Edition, Volume 5. Edited by Fletcher CDM, Bridge JA, Hogendoorn PCW, Mertens F. IARC Press, Lyon. February 1, 2013. pp. 230-231. [Internet].
2. Oliva E, Carcangiu ML, Carinelli SG, Ip P, Loening T, Nucci MR. Mesenchymal tumors, in: WHO Classification of Tumours of the Female Reproductive Organs (Medicine) 4th Edition, Volume 6. Kurman RJ, Carcangiu ML, Herrington CS, Young RH (Eds.). IARC, Lyon, France. June 12, 2014, pp. 146-147. [Internet].
3. 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 Dec;29(12):1558-75. [PubMed] [Crossref].
4. Bonetti F, Pea M, Martignoni G, Zamboni G. PEC and sugar. Am J Surg Pathol. 1992; 16: 307-308. [PubMed] [Crossref].
5. Apitz K. Die geschwulste und Gewebsmissbildungen Nierenrinde. II Midteilung Die mesenchymalen Neubildungen. Virchows Arch. 1943; 311:306-327.
6. Fadare O, Parkash V, Yilmaz Y, Mariappan MR, Ma L, Hileeto D, et al. Perivascular epithelioid cell tumor (PEComa) of the uterine cervix associated with intra-abdominal "PEComatosis": A clinicopathological study with comparative genomic hybridization analysis. World J Surg Oncol. 2004 Oct; 2(1):35. [PubMed][Crossref].
7. Armah HB, Parwani AV. Perivascular epithelioid cell tumor. Arch Pathol Lab Med. 2009 Apr;133(4):648-54. [PubMed] [Crossref].
8. Conlon N, Soslow RA, Murali R. Perivascular epithelioid tumours (PEComas) of the gynaecological tract. J Clin Pathol. 2015 Jun;68(6):418-26. [PubMed] [Crossref].
9. Kudela E, Biringer K, Kasajova P, Nachajova M, Adamkov M. Perivascular epithelioid cell tumors of the uterine cervix. Pathol Res Pract. 2016 Aug;212(8):667-71. [PubMed] [Crossref].
10. Azad NS, Aziz AB, Pervez S, Kayani N. Uterine perivascular epithelioid cell tumor presenting as a cervical mass. J P Med Assoc. 2006 Feb;56(2):83-4. [PubMed].
11. Bradshaw MJ, Folpe AL, Croghan GA. Perivascular epithelioid cell neoplasm of the uterine cervix: unusual tumor in an unusual location. Rare Tumors. 2010 Dec;2(4):e56. [PubMed] [Crossref].
12. Wagner AJ, Malinowska-Kolodziej I, Morgan JA, Qin W, Fletcher CD, Vena N, et al. Clinical activity of mTOR inhibition with sirolimus in malignant perivascular epithelioid cell tumors: targeting the pathogenic activation of mTORC1 in tumors. J Clin Oncol. 2010 Feb;28:835-840. [PubMed] [Crossref].
13. Yamamoto E, Ino K, Sakurai M, Takigawa S, Iwase A, Kikkawa F. Fertility-sparing operation for recurrence of the uterine cervical perivascular epithelioid cell tumor. Rare Tumors. 2010 Jun 30;2(2):e26.. [PubMed] [Crossref].
14. Lim GS, Oliva E. The morphologic spectrum of uterine PEC-cell associated tumors in a patient with tuberous sclerosis. Int J Gynecol Pathol. 2011 Mar;30(2):121-8. [PubMed] [Crossref].
15. Zhang C, Pan F, Qiao J, Jiang J, Du Y, Zhao C. Perivascular epithelioid cell tumor of the cervix with malignant potential. Int J Gynecol Obstet. 2013 Oct;123(1):72-3. [PubMed] [Crossref].
16. Çelık H, Kefelı M, Çetınkaya M, Yıldız L. Perivascular Epithelioid Cell Tumor (PEComa) of the Uterine Cervix in a Patient with Tuberous Sclerosis Complex: A Literature Review. Turkish Journal of Pathology. 2014; 34(1):82-86. [PubMed] [Crossref].
17. Natella V, Merolla F, Giampaolino P, Bifulco G, Mainenti PP, Insabato L. A huge malignant perivascular epithelioid cell tumor (PEComa) of the uterine cervix and vagina. Pathol Res Pract. 2014 Mar;210(3):186-8. [PubMed] [Crossref].
18. Liu F, Zhang R, Wang ZY, Xia Q, Shen Q, Shi S, et al. Malignant perivascular epithelioid cell tumor (PEComa) of cervix with TFE3 gene rearrangement: a case report. Int J Clin Exp Pathol. 2014 Aug;7(9):6409-14. [PubMed].
19. Kovac O , Babal P, Kajo K, Kobzova D, Copakova L, Cermak M. Perivascular Epithelioid Cell Tumor (PEComa) of the Uterine Cervix: A Case Report of a 43-Yr-Old Woman With Abnormal Uterine Bleeding Treated With Hysterectomy. Int J Gynecol Pathol. 2018 Sep;37(5):492-496. [PubMed] [Crossref].
20. Fukunaga M. Perivascular epithelioid cell tumor of the uterus: report of four cases. Int J Gynecol Pathol. 2005 Oct; 24(4):341-6. [PubMed].
21. Ruco LP, Pilozzi E, Wedard BM, Marzullo A, D'Andrea V, De Antoni E, et al. Epithelioid lymphangioleiomyomatosis-like tumour of the uterus in a patient without tuberous sclerosis: a lesion mimicking epithelioid leiomyosarcoma. Histopathology. 1998 Jul;33(1):91-3. [PubMed] [Crossref].
22. Bonetti F, Martignoni G, Colato C, Manfrin E, Gambacorta M, Faleri M, et al. Abdominopelvic sarcoma of perivascular epithelioid cells. Report of four cases in young women, one with tuberous sclerosis. Mod Pathol. 2001 Jun;14(6):563-8. [PubMed] [Crossref].
23. 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 Jan;26(1):1-13. [PubMed] [Crossref].
24. Dimmler A, Seitz G, Hohenberger W, Kirchner T, Faller G. Late pulmonary metastasis in uterine PEComa. J Clin Pathol. 2003; 56(8):627-8. [PubMed] [Crossref].
25. 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 Sep;90(3):677-81. [PubMed] [Crossref].
26. Park SH, Ro JY, Kim HS, Lee ES. Perivascular epithelioid cell tumor of the uterus: immunohistochemical, ultrastructural and molecular study. Pathol Int. 2003 Nov;53(11):800-5. [PubMed].
27. Nikolova M, Natchev R, Gortchev G. HMB-45positive perivascular epithelioid cell tumor ("PEComa") of the uterus. Clin Applic Immun. 2004 Sep;3(2):397-9.
28. 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 Dec; 27(12):681-4. [PubMed].
29. Bosincu L, Rocca PC, Martignoni G, Nogales FF, Longa L, Maccioni A. Perivascular epithelioid cell (PEC) tumors of the uterus: a clinicopathologic study of two cases with aggressive features. Mod Pathol. 2005 Oct; 18(10):1336-42. [PubMed] [Crossref].
30. Iravanlo G, Nozarian Z. Uterine perivascular epithelioid cell tumor” case report. Acta Medica Iranica. 2006; 44(1): 63-64.
31. 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 Dec 3;2:45. [PubMed] [Crossref].
32. Gan MF, Yu CK, Jin M, Lu HS, Li HM. Perivascular epithelioid cell tumor of the uterus: report of three cases. Chin Med J. 2007 Mar;120(6):526–8. [PubMed].
33. Cho HY, Chung DH, Khurana H, Zhai QJ, Ro JY. The role of TFE3 in PEComa. Histopathology. 2008 Aug; 53(2):236-49. [PubMed] [Crossref].
34. 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 Jan;27(1):86-90. [PubMed] [Crossref].
35. Liu JL, Lin YM, Lin MC, Yeh KT, Hsu JC, Chin CJ. Perivascular epithelioid cell tumor (PEComa) of the uterus with aggressive behavior at presentation. Hematol Oncol Stem Cell Ther. 2009; 2(3):426-30. [PubMed].
36. Italiano A, Delcambre C, Hostein I, Cazeau AL, Marty M, Avril A, et al. Treatment with the mTOR inhibitor temsirolimus in patients with malignant PEComa. Ann Oncol. 2010 May;21(5):1135-7. [PubMed] [Crossref].
37. Yamashita K, Fletcher DM. PEComa presenting in bone: clinicopathologic analysis of 6 cases and literature review. Am J Surg Pathol. 2010; 34(11):1622-1629. [PubMed] [Crossref]
38. Bleeker JS, Quevedo JF, Folpe AL. “Malignant” Perivascular Epithelioid Cell Neoplasm: Risk Stratification and Treatment Strategies. Sarcoma. 2012;2012:541626. [Crossref]
39. Chamsy D, Purinton SC, Carrick KS, Richardson DL. Malignant Uterine Perivascular Epithelioid Cell Tumor: A Case Report. Clinical Ovarian & Other Gynecologic Cancer. 2012 Jun;5(1):37-9. [Crossref]
40. Cossu A, Paliogiannis P, Tanda F, Dessole S, Palmieri G, Capobianco G.Uterine perivascular epithelioid cell neoplasms (PEComas): report of two cases and literature review. Eur J Gynaecol Oncol. 2014; 35(3):309-12. [PubMed].
41. Kang JB, Seo JW, Park YH, Jang PR. Malignant Perivascular Epithelioid Cell Tumor of the Uterus with Lung Metastasis. The Korean Journal of Pathology. 2014 Dec;48(6):454-7. [PubMed] [Crossref].
42. Fitzpatrick M, Pulver T, Klein M, Murugan P, Khalifa M, Amin K. Perivascular Epithelioid Cell Tumor of the Uterus with Ovarian Involvement: A Case Report and Review of the Literature. Am J Case Rep. 2016 May;17:309-14. [PubMed] [Crossref].
43. Kwon BS, Suh DS, Lee NK, Song YJ, Choi KU, Kim KH. Two cases of perivascular epithelioid cell tumor of the uterus: clinical, radiological and pathological diagnostic challenge. Eur J Med Res. 2017 Mar;22(1):7. [PubMed] [Crossref].
44. Okamoto S, Komura M, Terao Y , Kurisaki-Arakawa A, Hayashi T, Saito T, et al. Pneumothorax caused by cystic and nodular lung metastases from a malignant uterine perivascular epithelioid cell tumor (PEComa). Respiratory Medicine Case Reports. 2017 Jun;22:77-82. [PubMed] [Crossref].
45. Wang X, Fu Y, Li B. Perivascular epithelioid cell tumor (PEComa) presents an endometrial polyp pattern: Case report and literature review. Hum Pathol (NY). 2018 Sep;13:66-68. [Crossref]
46. Schoolmeester JK, Howitt BE, Hirsch MS, Dal Cin P, Quade BJ, Nucci MR. Perivascular epithelioid cell neoplasm (PEComa) of the gynecologic tract: clinicopathologic and immunohistochemical characterization of 16 cases. Am J Surg Pathol. 2014 Feb;38(2):176-88. [PubMed] [Crossref].
47. Acosta AM, Adley BP. Predicting the Behavior of Perivascular Epithelioid Cell Tumors of the Uterine Corpus. Arch Pathol Lab Med. 2017 Mar; 141:463-469. [PubMed] [Crossref].
48. 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 Feb;28(2):244-9. [PubMed] [Crossref]..
Received: 28 February 2019
Published online: 11 November 2019