Онкопедиатрия. 2016; 3: 267-276
ПЕКОМЫ
https://doi.org/10.15690/onco.v3i4.1630Аннотация
В 2002 г. в Международной классификации опухолей костей и мягких тканей было выделено семейство мезенхимальных опухолей, обозначенных общим термином «пекомы» (PEComas). Обзор литературных данных подтверждает трудности в диагностике и отсутствие прогресса в лечении больных. Несмотря на достижения в области изучения пеком, гистологическая природа этих неоплазий остается неясной. Требуется дальнейшее изучение прогностических факторов для проведения адекватного лечения. Больные, получившие лечение по поводу пеком различных локализаций, требуют тщательного динамического наблюдения независимо от потенциала опухоли, так как возможно развитие поздних рецидивов и метастазов.
Список литературы
1. Conlon N, Soslow RA, Murali R. Perivascular epithelioid tumors (PEComas) of the gynaecological tract. J Clin Pathol. 2015;68:418–426.
2. Pea M, Bonetti F, Zamboni G, et al. Clear cell tumors and angiomyolipoma. Am J Surg Pathol. 1991;15:199–202.
3. Pea M, Bonetti F, Zamboni G, et al. Melanocytemarker HMB-45 is regularly expressed in angiomyolipoma of kidney. Pathology. 1991;23:185–188.
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7. Martignoni G, Pea M, Reghellin D, et al. PEComas: the past, the present and the future. Virchows Arch. 2008;452:119–132.
8. Bonetti F, Pea M, Martignoni G, et al. PEC and sugar. Am J Surg Pathol. 1992;16:307–308.
9. Zamboni G, Pea M, Martignoni G, et al. Clear cell ‘sugar’ tumor of pancreas. A novel member of the family of lesions characterised by the presence of perivascular epithelioid cells. Am J Surg Pathol. 1996;20:722–730.
10. Fletcher CD, Unni KK, Mertens F. World health organization classification of tumors: pathology and genetics of tumors of soft tissue and bone. Lyon: IARC Press, 2002.
11. Hornick JL, Fletcher CD. PEComa: what do we know so far? Histopathology. 2006;48, N 1:75–82.
12. Bonetti F., Pea M., Martignoni G, et al. Clear cell (‘sugar’) tumor of the lung is a lesion strictly related to angiomyolipoma — the concept of a family of lesions characterized by the presence of the perivascular epithelioid cells (PEC). Pathology. 1994;26:230–236.
13. Fernandez-Flores A. Evidence on the neural crest origin of PEComas. Rom J Morphol Embryol. 2011;52:7–13.
14. Armah HB, Parwani AV. Perivascular epithelioid cell tumor. Arch Pathol Lab Med. 2009;133(4):648–654.
15. Thway K, Fisher C. PEComa: morphology and genetics of a complex tumor family. Ann Diagn Pathol. 2015. pii: S1092-9134(15)00090-8.doi: 10.1016/j.anndiagpath.2015.06.003.
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21. Pan C-C, Chung M-Y, Ng K-F, et al. Constant allelic alteration on chromosome 16p (TSC2 gene) in perivascular epithelioid tumor (PEComa): genetic evidence for the relationship of PEComa with angiomyolipoma. J Pathol. 2008;214:387–393.
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27. Schoolmeester JK, Dao LN, Sukov WR, et al. TFE3 translocation-associated perivascular epithelioid cell neoplasm (PEComa) of the gynecologic tract: morphology, immunophenotype, differential diagnosis. Am J Surg Pathol. 2015;39(3):394–404.
28. Bonetti F, Pea M, Martignoni G, et al. The perivascular epithelioid cell and related lesions. Adv Anat Pathol. 1997;4:343–358.
29. Stone CH, Lee MW, Amin MB, et al. Renal angiomyolipoma: further immunophenotypic characterization of an expanding morphologic spectrum. Arch Pathol Lab Med. 2001;125:751–758.
30. Whale CI, Johnson SR, Phillips KG, et al. Lymphangioleiomyomatosis: a case-control study of perinatal and early life events. Thorax. 2003;58:979– 982.
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32. Logginidou H, Ao H, Russo I, Henske EP. Frequent estrogen and progesterone receptor immunoreactivity in renal angiomyolipomas from women with pulmonary lymphangioleiomyomatosis. Chest. 2000;117:25–30.
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35. Korytnaya E, Liu J, Camelo-Piragua S, et al. Ectopic prolactin secretion from a perivascular epithelioid cell tumor (PEComa). J Clin Endocrinol Metab. 2014;99(11):3960–3964.
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90. Wu JH, Zhou JL, Cui Y, et al. Malignant perivascular epithelioid cell tumor of the retroperitoneum. Int J Clin Exp Pathol. 2013;6(10):2251–2256.
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93. Musella A, De Felice F, Kyriacou AK, et al. Perivascular epithelioid cell neoplasm (PEComa) of the uterus: a systematic review. Int J Surg. 2015;19:1–5.
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95. Pirson Y. Tuberous sclerosis complex-associated kidney angiomyolipoma: from contemplation to action. Nephrol Dial Transplant. 2013;28:1680–1685.
96. Benson C, Vitfell-Rasmussen J, Maruzzo M, Fisher C, 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):3663–3668.
97. Bissler JJ, McCormack FX, Young LR, et al. Sirolimus for angiomyolipoma in tuberous sclerosis complex or lymphangioleiomyomatosis. N Engl J Med. 2008;358(2):140–151.
98. Le P, Garg A, Brandao G, et al. Hormonal manipulation with letrozole in treatment of metastatic malignant pecoma. Curr Oncol. 2014;21(3):e518–520.
99. 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. Diagnostic Pathology. 2007;2:45. doi: 10.1186/1746-1596-2-45.
Oncopediatrics. 2016; 3: 267-276
PEComa
https://doi.org/10.15690/onco.v3i4.1630Abstract
The family of mesenchymal tumors called PEComas (Perivascular Epithelioid Cell Tumors) has been included in the International Classification of Soft Tissue and Bone Tumors since 2002. Literature review confirms difficulties of timely diagnosis and lack of progress in treatment. Despite advances in the study of PEComas, histology of this type of tumors remains elusive. Further research of prognostic factors is required to improve treatment approaches and techniques. It is strongly recommended to provide remote monitoring for patients with PEComas of different localization who underwent treatment course, irrespectively of malignant potential, because late recurrence and metastases can occur.
References
1. Conlon N, Soslow RA, Murali R. Perivascular epithelioid tumors (PEComas) of the gynaecological tract. J Clin Pathol. 2015;68:418–426.
2. Pea M, Bonetti F, Zamboni G, et al. Clear cell tumors and angiomyolipoma. Am J Surg Pathol. 1991;15:199–202.
3. Pea M, Bonetti F, Zamboni G, et al. Melanocytemarker HMB-45 is regularly expressed in angiomyolipoma of kidney. Pathology. 1991;23:185–188.
4. Bonetti F, Pea M, Martignoni G, et al. Cellular heterogeneity in lymphangiomyomatosis of the lung. Hum Pathol. 1991;22:727–728.
5. Chan JK, Tsang WY, Pau MY, et al. Lymphangiomyomatosis and angiomyolipoma: closely related entities characterised by hamartomatous proliferation of HMB-45-positive smooth muscle. Histopathology. 1993;23:445–455.
6. Argani P, Aulmann S, Illei PB, et al. A distinctive subset of PEComas harbors TFE3 gene fusions. Am J Surg Pathol. 2010;34(10):1395–1406.
7. Martignoni G, Pea M, Reghellin D, et al. PEComas: the past, the present and the future. Virchows Arch. 2008;452:119–132.
8. Bonetti F, Pea M, Martignoni G, et al. PEC and sugar. Am J Surg Pathol. 1992;16:307–308.
9. Zamboni G, Pea M, Martignoni G, et al. Clear cell ‘sugar’ tumor of pancreas. A novel member of the family of lesions characterised by the presence of perivascular epithelioid cells. Am J Surg Pathol. 1996;20:722–730.
10. Fletcher CD, Unni KK, Mertens F. World health organization classification of tumors: pathology and genetics of tumors of soft tissue and bone. Lyon: IARC Press, 2002.
11. Hornick JL, Fletcher CD. PEComa: what do we know so far? Histopathology. 2006;48, N 1:75–82.
12. Bonetti F., Pea M., Martignoni G, et al. Clear cell (‘sugar’) tumor of the lung is a lesion strictly related to angiomyolipoma — the concept of a family of lesions characterized by the presence of the perivascular epithelioid cells (PEC). Pathology. 1994;26:230–236.
13. Fernandez-Flores A. Evidence on the neural crest origin of PEComas. Rom J Morphol Embryol. 2011;52:7–13.
14. Armah HB, Parwani AV. Perivascular epithelioid cell tumor. Arch Pathol Lab Med. 2009;133(4):648–654.
15. Thway K, Fisher C. PEComa: morphology and genetics of a complex tumor family. Ann Diagn Pathol. 2015. pii: S1092-9134(15)00090-8.doi: 10.1016/j.anndiagpath.2015.06.003.
16. Weinreb I, Howard D, Latta E, et al. 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.
17. Han JM, Lee KH, Kim SJ, et al. A case of lymphangioleiomyomatosis originated in the pelvic cavity. J Gynecol Oncol. 2008;19(3):195–198.
18. Crino PB, Nathanson KL, Henske EP. The tuberous sclerosis complex. N Engl J Med. 2006;355:1345– 1356.
19. Al-Saleem T, Wessner LL, Scheithauer BW, et al. Malignat tumors of kidney, brain and soft tissues in children and young adults with the tuberous sclerosis complex. Cancer. 1998;83:2208–2216.
20. Pan CC, Jong YJ, Chai CY, et al. 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(5):606–612.
21. Pan C-C, Chung M-Y, Ng K-F, et al. Constant allelic alteration on chromosome 16p (TSC2 gene) in perivascular epithelioid tumor (PEComa): genetic evidence for the relationship of PEComa with angiomyolipoma. J Pathol. 2008;214:387–393.
22. Knuutila S, Aalto Y, Autio K, et al. DNK copy number losses in human neoplasms. Am J Pathol. 1999;155:683–694.
23. Lee SE, Choi YL, Cho J, et al. Ovarian perivascular epithelioid cell tumor not otherwise specified with transcription factor E3 gene rearrangement: a case report and review of the literature. Hum Pathol. 2012;43(7):1126–1130.
24. LeGallo RD, Stelow EB, Sukov WR, et al. Melanotic Xp11.2 neoplasm of the ovary: report of a unique case. Am J Surg Pathol. 2012;36(9):1410–1414.
25. Cho HY, Chung DH, Khurana H, et al. The role of TFE3 in REComa. Histopathology. 2008;53(2):236– 249.
26. Schoolmeester JK, Howitt BE, Hirsch MS, et al. Perivascular epithelioid cell neoplasm (PEComa) of the gynecologic tract: clinicopathologic and immunohistochemical characterization of 16 cases. Am J Surg Pathol. 2014;32(2):176–188.
27. Schoolmeester JK, Dao LN, Sukov WR, et al. TFE3 translocation-associated perivascular epithelioid cell neoplasm (PEComa) of the gynecologic tract: morphology, immunophenotype, differential diagnosis. Am J Surg Pathol. 2015;39(3):394–404.
28. Bonetti F, Pea M, Martignoni G, et al. The perivascular epithelioid cell and related lesions. Adv Anat Pathol. 1997;4:343–358.
29. Stone CH, Lee MW, Amin MB, et al. Renal angiomyolipoma: further immunophenotypic characterization of an expanding morphologic spectrum. Arch Pathol Lab Med. 2001;125:751–758.
30. Whale CI, Johnson SR, Phillips KG, et al. Lymphangioleiomyomatosis: a case-control study of perinatal and early life events. Thorax. 2003;58:979– 982.
31. Matsui K, Takeda K, Yu Z-H, Valencia J, et al. Downregulation of estrogen and progesterone receptors in abnormal smooth muscle cells in pulmonary lymphangioleiomyomatosis following therapy. Am J Respir Crit Care Med. 2000;161:1002–1009.
32. Logginidou H, Ao H, Russo I, Henske EP. Frequent estrogen and progesterone receptor immunoreactivity in renal angiomyolipomas from women with pulmonary lymphangioleiomyomatosis. Chest. 2000;117:25–30.
33. Bosincu L, Rocca PC, Martignoni G, et al. Perivascular epithelioid cell (PEC) tumors of the uterus: clinicopathologic study of two cases with aggressive features. Mod Pathol. 2005;18:1336–1342.
34. Folpe AL, Kwiatkowski DJ. Perivascular epithelioid cell neoplasms: pathology and pathogenesis. Hum Pathol. 2010;41:1–15.
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