PEComas: what are the treatment options? Literature review and personal experience


Citar

Texto integral

Acesso aberto Acesso aberto
Acesso é fechado Acesso está concedido
Acesso é fechado Acesso é pago ou somente para assinantes

Resumo

Background. Perivascular epithelioid cell tumors (PEComas) are extremely rare mesenchymal neoplasms that can affect almost any organ. Difficulties of diagnosis, lack of prognostic factors and adequate treatment reduce the overall survival of patients with this pathology. The article presents the latest achievements in the field of drug treatment of malignant PEComas. Description of the clinical case. An own clinical observation of successful application of everolimus in 40-year-old patient suffering from a malignant PEComa of the uterus with lung metastases is presented. Therapy lasts more than 7 months, the maximum effect of the treatment is stabilization of the process. Conclusion. Standard chemotherapy regimens are ineffective for PEComas and are accompanied by the rapid development of process progression. Analysis of the molecular mechanisms of the disease led to the emergence of a new class of drugs - mTOR inhibitors, currently the main method of drug treatment of this pathology. Nevertheless, the issue of choosing the second and subsequent lines of therapy has not been fully studied. However, timely and correct diagnosis determines a higher efficacy of the treatment and, therefore, improves the quality of life and life expectancy of this category of patients.

Texto integral

Acesso é fechado

Sobre autores

Svetlana Protsenko

N.N. Petrov National Medical Research Center of Oncology

Email: s.protsenko@list.ru
MD, Professor, Head of the Department of Chemotherapy and Innovative Technologies 68, Leningradskaya Street, Pesochny settlement, St. Petersburg 197758, Russian Federation

E. Degtiareva

N.N. Petrov National Medical Research Center of Oncology

St. Petersburg, Russia

G. Teletaeva

N.N. Petrov National Medical Research Center of Oncology

St. Petersburg, Russia

E. Levchenko

N.N. Petrov National Medical Research Center of Oncology

St. Petersburg, Russia

N. Levchenko

N.N. Petrov National Medical Research Center of Oncology

St. Petersburg, Russia

A. Mishchenko

N.N. Petrov National Medical Research Center of Oncology

St. Petersburg, Russia

I. Burovik

N.N. Petrov National Medical Research Center of Oncology

St. Petersburg, Russia

A. Artemyeva

N.N. Petrov National Medical Research Center of Oncology

St. Petersburg, Russia

E. Imyanitov

N.N. Petrov National Medical Research Center of Oncology

St. Petersburg, Russia

A. Semenova

N.N. Petrov National Medical Research Center of Oncology

St. Petersburg, Russia

A. Novik

N.N. Petrov National Medical Research Center of Oncology

St. Petersburg, Russia

D. Latipova

N.N. Petrov National Medical Research Center of Oncology

St. Petersburg, Russia

Bibliografia

  1. Armah H.B., Parwani A.V. Perivascular Epithelioid Cell Tumor Arch Pathol Lab Med. 2009;133(4):648-54. doi: 10.1043/15432165-133.4.648.
  2. Thway K., Fisher C. PEComa: morphology and genetics of a complex tumor family Ann Diagn Pathol. 2015;19(5):359-68. Doi: 10.1016/j. anndiagpath.2015.06.003.
  3. Fletcher C.D., Unni K.K., Mertens F. World health organization classification of tumors: pathology and genetics of tumors of soft tissue and bone. Lyon: IARC Press, 2002. Р 12-224.
  4. Shankar V PEComa - general. PathologyOutlines. com website. http://www.pathologyoutlines.com/topic/softtissuepecgeneral.html. 2019.
  5. Starbuck K.D., Drake R.D., Budd G.T., et al. Treatment of Advanced Malignant Uterine Perivascular Epithelioid Cell Tumor with mTOR Inhibitors: Single-institution Experience and Review of the Literature. Anticancer Res. 2016;36(11):6161-64.
  6. Schoolmeester J.K., Dao L.N., Soslow R.A. TFE3 Translocation Associated Perivascular epithelial cell tumor (PEComa) of the Gynecologic Tract: Morphology, Immunophenotype, Differential Diagnosis. Am J Surg Pathol. 2015;39(3):394-404. doi: 10.1097/PAS.0000000000000349.
  7. Zhang S., Chen F, Huang X., et al. Perivascular epithelial cell tumor (PEComa) of the pancreas: A case report and review of literature. Medicine (Baltimore). 2017;96(22):e7050. doi: 10.1097/MD.0000000000007050.
  8. Sadeghi S., Krigman H., Maluf H. Perivascular epithelioid clear cell tumor of the common bile duct. Am J Surg Pathol. 2004;28(8):1107-10.
  9. Doyle L.A., Hornick J.L., Fletcher C.D. PEComa of the gastrointestinal tract: clinicopathologic study of 35 cases with evaluation of prognostic parameters. Am J Surg Pathol. 2013;37(12):1769-82. doi: 10.1097/PAS.0b013e31829caab3.
  10. Kwazneski II D., Merrill M., Young J., et al. Angiomyolipoma and Malignant PEComa: Discussion of Two Rare Adrenal Tumors. Case Rep Oncol Med. 2016;2016:5204092. doi: 10.1155/2016/5204092.
  11. Eken A., Saglican Y Primary perivascular epithelioid cell tumour (PEComa) of the prostate. Can Urol Assoc J. 2014;8(5-6):E455-57. doi: 10.5489/cuaj.1752.
  12. Schoolmeester J.K., Howitt B.E., Hirsch M.S., et al. Perivascular epithelioid cell neoplasm (PEComa) of the gynecologic tract: clinicopathologic and immunohistochemical characterization of 16 cases. Am J Surg Pathol. 2014;38(2):176-88. doi: 10.1097/PAS.0000000000000133.
  13. Okamoto K., Okada Y., Ohno K., et al. A rare case of perivascular epithelioid cell tumor (PEComa) of the greater omentum. World J Surg Oncol. 2018;16(1):113. doi: 10.1186/s12957-018-1407-5.
  14. Llamas-Velasco M., Requena L., Mentzel T. Cutaneous perivascular epithelioid cell tumors: A review on an infrequent neoplasm. World J Methodol. 2016;6(1):87-92. doi: 10.5662/wjm.v6.i1.87.
  15. Lao I.W., Yu L., Wang J. Malignant perivascular epithelioid cell tumor (PEComa) of the femur: a case report and literature review. Diagn Pathol. 2015;10:54. doi: 10.1186/s13000-015-0292-2.
  16. Paliogiannis P, Palmieri G., Tanda F, et al. Perivascular Epithelioid Cell Tumors (PEComas) of the Orbit. J Pathol Transl Med. 2017;51(1):7-8. doi: 10.4132/jptm.2016.10.26.
  17. Huilin Niu, Wang F.W., Zhang P.J., et al. Cardiac Epithelioid PEComa: Report of Two Cases and Review of the Literature. Case Reports in Oncological Medicine. 2012. doi: 10.1155/2012/521678.
  18. Wejman J., Nowak K., Gielniewska L., et al. PEComa of the mesentery coexisting with colon cancer: a case report. Diagn Pathol. 2015;10:31. doi: 10.1186/s13000-015-0265-5.
  19. Нечушкина И.В. Пекомы. Онкопедиатрия. 2016;3(4):267-76.
  20. Unluoglu S., Bayol U., Korkmaz N., et al. Perivascular epithelioid cell tumor of the ileum presenting as diverticulitis. Case Rep Pathol. 2012;2012:476941. doi: 10.1155/2012/476941.
  21. Osei D.A., Alvandi F., Brooks J.S., et al. PEComa of the Upper Extremity: A Unique Case and Description of an Initial Response to Neoadjuvant Chemotherapy. Sarcoma. 2007;2007:53056.
  22. Bergamo F., Maruzzo M., Basso U., et al. Neoadjuvant sirolimus for a large hepatic perivascular epithelioid cell tumor (PEComa). World J Surg Oncol. 2014;12:46.
  23. Bleeker J.S., Quevedo J.F., Folpe A.L. "Malignant" Perivascular Epithelioid Cell Neoplasm: Risk Stratification and Treatment Strategies. Sarcoma. 2012;2012:541626. doi: 10.1155/2012/541626
  24. Hindi N., Sanfilippo R., Stacchiotti S., et al. 1450P -Systemic therapy in perivascular epithelioid cell tumors (PEComa). Ann Oncol. 2014;25(4): iv506-iv506.
  25. Bissler J.J., McCormack F.X., Young L.R., et al. Sirolimus for Angiomyolipoma in Tuberous Sclerosis Complex or Lymphangioleiomyomatosis. N Engl J Med. 2008;358(2):140-51. doi: 10.1056/NEJMoa063564.
  26. Benson C., Vitfell-Rasmussen J., Maruzzo M., et al. A retrospective study of patients with malignant PEComa receiving treatment with sirolimus or temsirolimus: the Royal Marsden Hospital experience. Anticancer Res. 2014;34(7): 3663-68.
  27. Wagner A.J., Malinowska-Kolodziej I., Morgan J.A., et al. Clinical activity of mTOR inhibition with siroiimus in malignant perivascular epithelioid cell tumors: targeting the pathogenic activation of mTORCI in tumors. J Clin Oncol. 2010;28(5):835-40. doi: 10.1200/JCO.2009.25.2981
  28. RAPAMUNE (sirolimus) tablets label - FDA. URL: https://www.accessdata.fda.gov/drugsatfda_docs/label/2017/021083s059.021110s076lbl.pdf.
  29. Italiano А, Delcambre C., Hostein I., et al. Treatment with the mTOR inhibitor temsirolimus in patients with malignant PEComa. Ann Oncol. 2010;21(5):1135-37. doi: 10.1093/annonc/mdq044.
  30. Flechter E., Zohar Y, Guralnik L., et al. Long-lasting stable disease with mTOR inhibitor treatment in a patient with a perivascular epithelioid cell tumor: A case report and literature review. Oncol Lett. 2016;12(6):4739-43. doi: 10.3892/ol.2016.5231.
  31. Gennatas C., Michalaki V., Kairi P.V., et al. Successful treatment with the mTOR inhibitor everolimus in a patient with Perivascular epithelioid cell tumor. World J Surg Oncol. 2012;10:181. doi: 10.1186/1477-7819-10-181.
  32. Mehren M., Kane J.M. Ill, Benjamin R.S., et al. Soft Tissue Sarcoma, Version 2.2019, NCCN Clinical Practice Guidelines in Oncology. J National Comprehensive Cancer Network: JNCCN. 2018;16(5):536-63.
  33. Casali P.G., Abecassis N., Bauer S., et al. On behalf of the ESMO Guidelines Committee and EURACAN. Soft tissue and visceral sarcomas: ESMO-EURACAN Clinical Practice Guidelines for diagnosis, treatment and follow-up. Ann Oncol. 2018;29(4): iv51-iv67.
  34. Gao F., Huang C., Zhang Y, et 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. 2016;17(6):595-98. doi: 10.1080/15384047.2016.1167290.
  35. Scheppach W., Reissmann N., Sprinz T, et al. PEComa of the colon resistant to sirolimus but responsive to doxorubicin/ifosfamide. World J Gastroenterol. 2013;19(10):1657-60. doi: 10.3748/wjg.v19.i10.1657.
  36. Anti-PD-1 therapy with nivolumab in the treatment of metastatic malignant PEComa. The Sarcoma J. 2018;2(1):7-9.
  37. Le P., Garg A., Brandao G., et al. Hormonal manipulation with letrozole in the treatment of metastatic malignant pecoma. Curr Oncol. 2014;21(3):e518-20. Doi: 10.3 747/co.21.1849.
  38. Lattanzi M., Deng F.M., Chiriboga L.A., et al. Durable response to anti-PD-1 immunotherapy in epithelioid angiomyolipoma: a report on the successful treatment of a rare malignancy. J Immunother Cancer. 2018;6(1):97. doi: 10.1186/s40425-018-0415-x.

Arquivos suplementares

Arquivos suplementares
Ação
1. JATS XML
Baixar

Declaração de direitos autorais © Bionika Media, 2019

Este site utiliza cookies

Ao continuar usando nosso site, você concorda com o procedimento de cookies que mantêm o site funcionando normalmente.

Informação sobre cookies