Neoplasia de células epitelioides perivasculares subtipo angiomiolipoma de localización retroperitoneal

Gustavo Nari; Eduardo Brandan Recalde; Gisela German; Sandra Rojo; María Carreras; David Lasso Molina

doi:10.56969/oc.v24i2.43

Autores/as

Gustavo Nari Servicio de Cirugía General, Hospital Tránsito Cáceres de Allende
Eduardo Brandan Recalde Anatomía Patológica, Hospital Tránsito Cáceres de Allende
Gisela German Servicio de Oncología Clínica, Hospital Oncológico Provincial, Córdoba
Sandra Rojo Servicio de Oncología Clínica, Hospital Oncológico Provincial, Córdoba
María Carreras Servicio de Oncología Clínica, Hospital Oncológico Provincial, Córdoba
David Lasso Molina Servicio de Oncología Clínica, Hospital Oncológico Provincial, Córdoba https://orcid.org/0000-0002-4357-8094

DOI:

https://doi.org/10.56969/oc.v24i2.43

Palabras clave:

angiomiolipoma, neoplasias de células epitelioides perivasculares, angiomyolipoma, neoplasms of perivascular epithelioid cells

Resumen

La Organización Mundial de la Salud define PEComa como "un tumor mesenquimal compuesto por células epitelioides perivasculares histológicamente e inmunohistoquímicamente distintivas". Esta familia de tumores incluye a: angiomiolipoma (AML), tumor de pulmón de células claras, linfangioleiomiomatosis y tumores muy raros en otros lugares. Los AML se clasificaron previamente como hamartomas; sin embargo, ahora se considera que los AML pertenecen a la familia de los PEComa. Se localizan principalmente en el riñón y son poco frecuentes las localizaciones extra-renales, raramente la ubicación es retroperitoneal. Presentamos el caso clínico de un varón de 30 años, en quien se halló a través de estudios por imágenes una masa retroperitoneal intercavo- aórtica de 7 cm aproximadamente, que se resecó quirúrgicamente y el estudio anatomopatológico confirmó el diagnóstico de PEComa sub-tipo AML.

Citas

Folpe AL, Fletcher CDM, Unni KK, Epstein J, Mertens F (eds). Neoplasm with perivascular epithelioid cell differentiation (PEComas).Pathology and genetics of tumours of soft tissue and bone. WHO Classification of Tumours. Lyon: IARC Press; 2002, pp 221-2.

Fletcher CDM, Bridge JA, Hogendoorn P, Mertens F (eds). World Health Organization. IARC WHO Classification of Tumours of Soft Tissue and Bone. Lyon: IARC Press, 2013.

Ferenczi K, Lastra RR, Farkas T, et al. MUM-1 expression differentiates tumors in the PEComa family from clear cell sarcoma and melanoma. Int J Surg Pathol 2012; 20:29-36. DOI: https://doi.org/10.1177/1066896911418204

Harknett EC, Chang WYC, Byrnes S, et al. Use of variability in national and regional data to estimate the prevalence of lymphangioleiomyomatosis. QJM 2011; 104:971-9. DOI: https://doi.org/10.1093/qjmed/hcr116

Folpe AL, Mentzel T, Lehr H-A, 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; 29:1558-75. DOI: https://doi.org/10.1097/01.pas.0000173232.22117.37

Thway K, Fisher C. PEComa: morphology and genetics of a complex tumor family. Ann Diagn Pathol 2015; 19:359-68. DOI: https://doi.org/10.1016/j.anndiagpath.2015.06.003

Hussain T, Al-Hamali S. Pathophysiology and management aspects of adrenal angiomyolipomas. Ann R Coll Surg Engl 2012; 94:224-6. DOI: https://doi.org/10.1308/003588412X13171221498541

Strahan A, King J, McClintock S. Retroperitoneal angiomyolipoma: a case report and review of the literature. Case Rep Radiol 2013; 2013:457383. DOI: https://doi.org/10.1155/2013/457383

Venyo AK. A Review of the literature on extrarenal retroperitoneal angiomyolipoma. Int J Surg Oncol 2016; 2016:6347136. DOI: https://doi.org/10.1155/2016/6347136

Lau SK, Marchevsky AM, McKenna RJ Jr, Luthringer DJ. Malignant monotypic

epithelioid angiomyolipoma of the retroperitoneum. Int J Surg Pathol 2003; 11:223-8. DOI: https://doi.org/10.1177/106689690301100313

Ziadie M. Kidney tumor-cysts, children, adult benign Benign (usually) adult tumors angiomyolipoma. PathologyOutlines. com, 2015. En: http://www.pathologyoutlines.com/topic/ kidneytumorangiomyolipoma.html; consultado 2/3/1019.

Tan G, Liu L, Qiu M, Chen L, Cao J, Liu J. Clinicopathologic features of renal epithelioid angiomyolipoma: report of one case and review of literatures. Int J Clin Exp Pathol 2015; 8:1077-80.

Minja EJ, Pellerin M, Saviano N, Chamberlain RS. Retroperitoneal extrarenal angiomyolipomas: an evidence- based approach to a rare clinical entity. Case Rep Nephrol 2012; 2012:374107. DOI: https://doi.org/10.1155/2012/374107

Murphy DP, Glazier DB, Chenven ES, Principato R, Diamond SM. Extrarenal retroperitoneal angiomyolipoma: nonoperative management. J Urol 2000; 163:234-5. DOI: https://doi.org/10.1016/S0022-5347(05)68015-9

Peces R, Peces C, Cuesta-López E, et al. Low-dose rapamycin reduces kidney volume angiomyolipomas and prevents the loss of renal function in a patient with tuberous sclerosis complex. Nephrol Dial Transplant 2010; 25:3787-91. DOI: https://doi.org/10.1093/ndt/gfq456

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; 7: 595-8. DOI: https://doi.org/10.1080/15384047.2016.1167290