Seminars in Diagnostic Pathology
Volume 26, Issue 3 , Pages 123-130 , August 2009

PEComas: a review with emphasis on cutaneous lesions

  • Sarah N. Walsh, MD

      Affiliations

    • Cutaneous Pathology, WCP Laboratories, St. Louis, Missouri
    • Corresponding Author InformationAddress reprint requests and correspondence: Sarah N. Walsh, MD, Cutaneous Pathology, WCP Laboratories, Inc, 2326 Millpark Dr, St. Louis, MO 63043
    • ,
  • Omar P. Sangüeza, MD

      Affiliations

    • Wake Forest University School of Medicine, Winston-Salem, North Carolina

References 

  1. Bourneville DM, Brissard E. Encephalite ou sclerose tuberouse des circonvolutions cerebrals. Arch Neurol. 1880;1:297
  2. Pea M, Bonetti F, Zamboni G, et al. Melanocyte-marker HMB-45 is regularly expressed in angiomyolipoma of the kidney. Pathology. 1991;23:185–188
  3. Liebow AA, Castleman B. Benign clear cell (“sugar”) tumors of the lung. Am J Pathol. 1963;43:13a–14a
  4. Pea M, Bonetti F, Zamboni G, et al. Clear cell tumor and angiomyolipoma. Am J Surg Pathol. 1991;15:199–202
  5. Gaffey MJ, Mills SE, Zarbo RJ, et al. Clear cell tumor of the lung (Immunohistochemical and ultrastructural evidence of melanogenesis). Am J Surg Pathol. 1991;15:644–653
  6. Frack MD, Simon L, Dawson BH. The lymphangiomyomatosis syndrome. Cancer. 1968;22:428–437
  7. Valensi QJ. Pulmonary lymphangiomyoma, a probable forme frust of tuberous sclerosis: A case report and survey of the literature. Am Rev Respir Dis. 1973;108:1411–1415
  8. Morteforte WJ, Kohnen PW. Angiomyolipomas in a case of lymphangiomyomatosis syndrome: Relationships to tuberous sclerosis. Cancer. 1974;34:317–321
  9. Bonetti F, Pea M, Martignoni G, et al. Cellular heterogeneity in lymphangiomyomatosis of the lung. Hum Pathol. 1991;22:727–728
  10. Fukuda Y, Kawamoto M, Yamamoto A, et al. Role of elastic fiber degradation in emphysema-like lesions of pulmonary lymphangiomyomatosis. Hum Pathol. 1990;21:1252–1261
  11. Mukai M, Torikata C, Iri H, et al. Crystalloids in angiomyolipoma. 1. A previously unnoticed phenomenon of renal angiomyolipoma occurring at a high frequency. Am J Surg Pathol. 1992;16:1–10
  12. Bonetti F, Chiodera PL, Pea M, et al. Transbronchial biopsy in lymphangiomyomatosis of the lung (HMB45 for diagnosis). Am J Surg Pathol. 1993;17:1092–1102
  13. Bonetti F, Pea M, Martignoni G, et al. PEC and sugar. Am J Surg Pathol. 1992;16:307–308
  14. Zamboni G, Pea M, Martignoni G, et al. Clear cell “sugar” tumor of the pancreas (A novel member of the family of lesions characterized by the presence of perivascular epithelioid cells). Am J Surg Pathol. 1996;20:722–730
  15. Folpe AL, Goodman ZD, Ishak KG, et al. Clear cell myomelanocytic tumor of the falciform ligament/ligamentum teres: A novel member of the perivascular epithelioid clear cell family of tumors with a predilection for children and young adults. Am J Surg Pathol. 2000;24:1239–1246
  16. Apitz K. Die Geschwülste und Gewebsmissbildungen der Nierenrinde (II Midteilung). Virchows Arch. 1943;311:306–327
  17. Martignoni G, Pea M, Reghellin D, et al. PEComas: The past, the present and the future. Virchows Arch. 2008;452:119–132
  18. Weeks DA, Malott RL, Arnesen M, et al. Hepatic angiomyolipoma with striated granules and positivity with melanoma—Specific antibody (HMB-45): A report of two cases. Ultrastruct Pathol. 1991;15:563–571
  19. Fetsch PA, Fetsch JF, Marincola FM, et al. Comparison of melanoma antigen recognized by T cells (Mart 1) to HMB-45: Additional evidence to support a common lineage for angiomyolipoma, lymphangiomyomatosis, and clear cell sugar tumor. Mod Pathol. 1998;11:699–703
  20. 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
  21. Folpe AL, Mentzel T, Lehr HA, et al. 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;29:1558–1575
  22. Adachi Y, Horie Y, Kitamura Y, et al. CD1a expression in PEComas. Pathol Int. 2008;58:169–173
  23. Weinreb I, Howarth 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
  24. Hornick JL, Fletcher CD. PEComa: What do we know so far?. Histopathology. 2006;48:75–82
  25. Beer TW. Cutaneous angiomyolipomas are HMB45 negative, not associated with tuberous sclerosis, and should be considered as angioleiomyomas with fat. Am J Dermatopathol. 2005;27:418–421
  26. Moss J, Avila NA, Barnes PM, et al. Prevalence and clinical characteristics of lymphangioleiomyomatosis (LAM) in patients with tuberous sclerosis complex. Am J Respir Crit Care Med. 2001;164:669–671
  27. 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;37:606–612
  28. Pan CC, Chung MY, Ng KF, et al. Constant allelic alteration on chromosome 16p (TSC2 gene) in perivascular epithelioid cell tumour (PEComa): Genetic evidence for the relationship of PEComa with angiomyolipoma. J Pathol. 2008;214:387–393
  29. 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;26:1–13
  30. Tazelaar HD, Batts KP, Srigley JR. Primary extrapulmonary sugar tumor (PEST): A report of four cases. Mod Pathol. 2001;14:615–622
  31. Govender D, Sabaratnam RM, Essa AS. Clear cell “sugar” tumor of the breast: Another extrapulmonary site and review of the literature. Am J Surg Pathol. 2002;26:670–675
  32. Tanaka Y, Ijiri R, Kato K, et al. HMB-45/Melan-A and smooth muscle actin-positive clear-cell epithelioid tumor arising in the ligamentum teres hepatis: Additional example of clear cell “sugar” tumors. Am J Surg Pathol. 2000;24:1295–1299
  33. Sadeghi S, Krigman H, Maluf H. Perivascular epithelioid clear cell tumor of the common bile duct. Am J Surg Pathol. 2004;28:1107–1110
  34. Pan CC, Yu IT, Yang AH, et al. Clear cell myomelanocytic tumor of the urinary bladder. Am J Surg Pathol. 2003;27:689–692
  35. Birkhaeuser F, Ackermann C, Flueckiger T, et al. First description of a PEComa (perivascular epithelioid cell tumor) of the colon: Report of a case and review of the literature. Dis Colon Rectum. 2004;47:1734–1737
  36. Pan CC, Yang AH, Chiang H. Malignant perivascular epithelioid cell tumor involving the prostate. Arch Pathol Lab Med. 2003;127:E96–E98
  37. Lehman NL. Malignant PEComa of the skull base. Am J Surg Pathol. 2004;28:1230–1232
  38. Righi A, Dimosthenous K, et al. Another member of the MiT tumor family?. Int J Surg Pathol. 2008;16:16–20
  39. Mahera H, Giamarelou N, Karabela-Bouropoulou V, et al. Soft tissue angiomyolipoma (A case report). Arch Anat Cytol Pathol. 1997;45:221–226
  40. Magro G, Lanzafame S. Sporadic subcutaneous angiomyolipoma with expression of estrogen and progesterone receptors. Virchows Arch. 2007;450:123–125
  41. Folpe AL, McKenney JK, Li Z, et al. Clear cell myomelanocytic tumor of the thigh: Report of a unique case. Am J Surg Pathol. 2002;26:809–812
  42. Hwang RC: Cutaneous angio(Lipo)leiomyoma. Tenth Annual Uniformed Services Dermatology Seminar, San Antonio, TX, May 15-17, 1985
  43. Fitzpatrick JE, Mellette JR, Hwang RJ, et al. Cutaneous angiolipoleiomyoma. J Am Acad Dermatol. 1990;23:1093–1098
  44. Argenyi ZB, Piette WW, Goeken JA. Cutaneous angiomyolipoma (A light-microscopic, immunohistochemical, and electron-microscopic study). J Cutan Pathol. 1986;13:434;(abstract)
  45. Argenyi ZB, Piette WW, Goeken JA. Cutaneous angiomyolipoma (A light-microscopic, immunohistochemical, and electron-microscopic study). Am J Dermatopathol. 1991;13:497–502
  46. Makino E, Yamada J, Tada J, et al. Cutaneous angiolipoleiomyoma. J Am Acad Dermatol. 2006;54:167–171
  47. Büyükbabani N, Tetikkurt S, Oztürk AS. Cutaneous angiomyolipoma: Report of two cases with emphasis on HMB-45 utility. J Eur Acad Dermatol Venereol. 1998;11:151–154
  48. Obata C, Murakami Y, Furue M, et al. Cutaneous angiomyolipoma. Dermatology. 2001;203:268–270
  49. Crowson AN, Taylor JR, Magro CM. Cutaneous clear cell myomelanocytic tumor-perivascular epithelioid cell tumor: First reported case. Mod Pathol. 2003;16:400
  50. de Saint Aubain Somerhausen N, Gomez Galdon M, Bouffioux B, et al. Clear cell “sugar” tumor (PEComa) of the skin: A case report. J Cutan Pathol. 2005;32:441–444
  51. Mentzel T, Reisshauer S, Rütten A, et al. Cutaneous clear cell myomelanocytic tumour: A new member of the growing family of perivascular epithelioid cell tumours (PEComas) (Clinicopathological and immunohistochemical analysis of seven cases). Histopathology. 2005;46:498–504
  52. Tan J, Peach AH, Merchant W. PEComas of the skin: More common in the lower limb? (Two case reports). Histopathology. 2007;51:135–136
  53. Liegl B, Hornick JL, Fletcher CD. Primary cutaneous PEComa: Distinctive clear cell lesions of skin. Am J Surg Pathol. 2008;32:608–614
  54. Bonetti F, Martignoni G, Colato C, et al. Abdominopelvic sarcoma of perivascular epithelioid cells (Report of four cases in young women, one with tuberous sclerosis). Mod Pathol. 2001;14:563–568
  55. Calder KB, Schlauder S, Morgan MB. Malignant perivascular epithelioid cell tumor (“PEComa”): A case report and literature review of cutaneous/subcutaneous presentations. J Cutan Pathol. 2008;35:499–503
  56. Chaplin A, Conrad DM, Tatlidil C, et al: Primary cutaneous PEComa. Am J Derm Pathol, in press
  57. Dimmler A, Seitz G, Hohenberger W, et al. Late pulmonary metastasis in uterine PEComa. J Clin Pathol. 2003;56:627–628
  58. Armah HB, Parwani AV. Perivascular epithelioid cell tumor. Arch Pathol Lab Med. 2009;133:648–654
  59. 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;27:681–684
  60. Parfitt JR, Bella AJ, Wehrli BM, et al. Primary PEComa of the bladder treated with primary excision and adjuvant interferon-alpha immunotherapy: A case report. BMC Urol. 2006;6:20
  61. Rigby H, Yu W, Schmidt MH, et al. Lack of response of a metastatic renal perivascular epithelial cell tumor (PEComa) to successive courses of DTIC based-therapy and imatinib mesylate. Pediatr Blood Cancer. 2005;45:202–206
  62. Ong LY, Hwang WS, Wong A, et al. Perivascular epithelioid cell tumour of the vagina in an 8 year old girl. J Pediatr Surg. 2007;42:564–566
  63. Cohen E. mTOR inhibitors. Clin Adv Hematol Oncol. 2006;4:38–39
  64. Kenerson H, Dundon TA, Yeung RS. Effects of rapamycin in the Eker rat model of tuberous sclerosis complex. Pediatr Res. 2005;57:67–75
  65. Lee L, Sudentas P, Donohue B, et al. Efficacy of a rapamycin analog (CCI-779) and IFN-gamma in tuberous sclerosis mouse models. Genes Chromosomes Cancer. 2005;42:213–227

PII: S0740-2570(09)00032-X

doi: 10.1053/j.semdp.2009.09.002

Seminars in Diagnostic Pathology
Volume 26, Issue 3 , Pages 123-130 , August 2009

Article Tools

* Image Usage & Resolution