Bone and soft tissue sarcomas, tumors of the skinBone and soft tissue sarcomas, tumors of the skinСаркомы костей, мягких тканей и опухоли кожи2219-46142782-3687Publishing House ABV Press158SOFT TISSUE SARCOMASСАРКОМЫ МЯГКИХ ТКАНЕЙPEComas: Neoplasms with perivascular epithelioid cell differentiationПЭКомы: Опухоли из периваскулярных эпителиодных клетокBliznyukovO. P.БлизнюковО. П.
Russian Federation
Blisnukov@mail.ruN.N. Blokhin Russian Cancer Research Center, Russian Academy ofMedical SciencesРоссийский онкологический научный центр им. Н.Н. Блохина РАМН1103201022394711012022Copyright ©; 2010, Bliznyukov O.P.Copyright ©; 2010, Близнюков О.П.2010Bliznyukov O.P.Близнюков О.П.https://creativecommons.org/licenses/by/4.0https://sarbon.abvpress.ru/jour/article/view/158

PEComas, occasionally associated with tuberous sclerosis complex, are defined by the presence of perivascular epithelioid cells that coexpress muscle and melanocytic markers. The family of tumors includes angiomyolipoma, clear cell sugar tumor of the lung, limphangioleiomyomatosis and very rare tumors in the other locations, which natural history and prognostic features undefined. The majority of PEComas are benign. Clear criteria for malignancy in PEComas have not be elaborated, owning to their rarity. Histogenesis of PEComas is unknown.

Представлен обзор литературы, посвященный классификации, морфологии, гистогенезу и клиническому течению ПЭКом. ПЭКомы - редкая группа мезенхимальных опухолей, которые построены из периваскулярных эпителио- идных клеток с двойной - мышечной и меланоцитарной - дифференцировкой. К группе ПЭКом относятся разные по локализации и клиническому течению опухоли почек, печени, легких, матки, мягких тканей конечностей и кожи. Подавляющая часть ПЭКом - доброкачественные опухоли. Диагноз ПЭКомы возможен только после морфологического исследования биоптата опухоли с применением специальных методов исследования - иммуноморфологии и электронной микроскопии. Классификация ПЭКом носит топографический характер. Гистогенез ПЭКом и критерии злокачественности этих опухолей остаются неясными. Обсуждаются клинико-морфологические особенности ПЭКом разной локализации.

PEComaclassificationhistopathologyhistogenesisclinical featuresПЭКомыклассификацияморфологиягистогенезклиническое течениеAnderson А.Е., Yang X., Young R.H. Epithelioid angio- myolipoma of the ovary. Int. J. Gynecol. Pathol. 2002, v. 21, p. 69-73.Apitz K. Die Geschwulste und Gewebsmissbildungen der Niererinde. II Midteilung. Die mesenchymalen Neubildun- gen. VirchowsArch. 1943, v. 311, p. 306-327.Bonetti F., Pea M., Martingnoni G., Zamboni G. HTC and Sugar [Letter]. Am. J. Surg. Pathol. 1992, v. 16, p. 307-308.Bonetti F., Pea M., Martingnoni G. et al. Clear cell («sugar») tumor of the lung is a lesion strictly related to angiomyolipoma - the concept of a family of the lesions characterized by the presence of the perivascular epithelioid cells (PEC). Pathology. 1994, v. 26, p. 23-236.Bonetti F., Martingnoni G., Colato C. Abdominopelvic sarcoma of epithelioid cells. Mod. Pathol. 2001, v. 14, p. 563-568.Eble J.N., Amin M.B., Young R.H. Epithelioid angiomyolipoma of the kidney. Am. J. Surg. Pathol. 1997, v. 21, p. 1123-1130.Eble J.N. Angiomyolipoma of kidney. Semin. Diagn. Pathol. 1998, v. 15, p. 21-40.Folpe A.L., Goodman Z.D., Ishak K.G. et al. Clearcellmyo-melanocytic tumor of the falciform ligament/ligament teres. Am. J. Surg. Pathol. 2000, v. 24, p. 1239-1246.Folpe A.L. Neoplasms with perivascular epithelioid cell differentiation (PEComas). In: Fletcher C.D., Unni K.K., Mertens F., eds. WHO Classification of Tumors. Pathology and Genetics. Tumours of Soft Tissue and Bone. Lyon: IARC Press. 2002, p. 221-222.Folpe A., Mentzel T., Lehr H.-A. et al. Perivascular epithelioid cell neoplasms of soft tissue and gynecologic origin. Am. J. Surg. Pathol. 2005, v. 29, p. 1558-1575.Gyere K.A., Hart W.R., Kennedy A.W. Lymphangiomyo- matosis of the uterus associated with tuberous sclerosis and malignant neoplasia of the female genital tract: a report of two cases. Int. J. Gynecol. Pathol. 1995, v. 14, p. 344-351.Henske E.P., Scheithauer B.W., Short M.P Allelic loss is frequent in tuberous sclerosis kidney lesions but rare in brain lesions. Am. J. Hum. Genet. 1996, v. 59, p. 400-406.Ishak K.G. Mesenchimal tumors of the liver. In: Okuda K., Peter R.L., eds. Hepatocellular Carcinoma. NY John-Willey. 1976, p. 268-275.Jungbluth A.A., Iversen K., Coplan K. et al. Expression of melanocyte-associated markers gplOO and Melan-A/MART-1 in angiomyolipomas. Virchows Arch. 1999, v. 434, p. 429-435.Karbowniczek M., Yu J., Henske E.P. Renal angiomyolipomas from patients with sporadic lymphangiomyomatosis contain both neoplastic and non-neoplastic vascular structures. Am. J. Surg. Pathol. 2003, v. 162, p. 491-500.Liebow A.A., Castelman B. Benignclearcell («sugar») tumors of the lung. Am. J. Surg. Pathol. 1963, v. 43, p. 13a-14a.Martingnoni G., Amin M.B. Angiomyolipoma. In: Eble J.N., Sauter G., Epstein J.I., Sesterhenn I.A. eds. WHO Classification of Tumors. Pathology and Genetics. Tumors of the Urinary System and Male Genital Organs. Lyon: IARC Press. 2004, p. 221-222.Nicholson A.G. Clear cell tumor ofthelung. In: Travis W.D., Brambilla E., Muller-Hermlink H.K., Harris C. eds. WHO Classification of Tumors. Pathology and Genetics. Tumours of the Lung, Pleura, Thymus and Heart. Lyon: IARC Press. 2004, p. 118.Pan C.C., Yu I.T., Yang A.Y. et al. Clear cell myomelanocytic tumor of the urinary bladder. Am. J. Surg. Pathol. 2003, v. 27, p. 689-692.Pan C.C., Yang A.H., Chiang H. Malignant perivascular epithelioid cell tumor involving the prostate. Arch. Pathol. Lab. Med. 2003, v. 127, p. 96-98.Pea M., Martingnoni G., Zamboni G., et al. Perivascular epithelioid cell [Letter]. Am. J. Surg. Pathol. 1996, v. 20, p. 1149-1153.Sadeghi S., Krigman H., Maluf H. et al. Perivascular epithelioid cell tumor ofthe common bile duct. Am. J. Surg. Pathol. 2004. v.28,p. 1107-1110.Silva E.G., Deavers M.T., Bodurka D.C. et al. Uterine epithelioid leiomyosarcomas with clear cells: reactivity with HMB-45 and concept of the PEComa. Am. J. Surg. Pathol. 2004, v. 28, p. 244-249.Tazelaar H.D., Batts К.Р., Srigley J.R. Primary extrapulmonary sugar tumor (PEComa) of the vulva. Mod. Pathol. 2001, v. 14, p. 615-622.Tazelaar H., Henske E.P., Manabe T., Travis W.D. Lym- phangioleiomyomatosis. In: Travis W.D., Brambilla E., Muller-Hermlink H.K., Harris C. eds. WHO Classification of Tumors. Pathology and Genetics. Tumors of the Lung, Pleura, Thymus and Heart. Lyon: IARC Press. 2004, p. 107-108.Tsui W.M., Colombari R., Portmann B.C. et al. Hepatic angiomyolipoma: a clinicopathologic study of 30 cases and delineation of unusual morphologic variants. Am. J. Surg. Pathol. 1999, v. 23, p. 34-48.Vang R., Kempson R.L. Perivascular epithelioid cell tumor («PEComa») of the uterus. Am. J. Surg. Pathol. 2002, v. 26, p. 1-13.Zamboni G., Pea M., Martingnoni G. et al. Clear cell («sugar») tumor of the pancreas. Am. J. Surg. Pathol. 1996, v. 16, p. 307-308.