Uterine PEComa: appraisal of a controversial and increasingly reported mesenchymal neoplasm

Bonetti F, Pea M, Martignoni G, Doglioni C, Zamboni G, Capelli P, Rimondi P, Andrion A: 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: 230-6. 10.1080/00313029400169561.

Pea M, Bonetti F, Zamboni G, Martignoni G, Fiore-Donati L: Clear cell tumor and angiomyolipoma [Letter]. Am J Surg Pathol. 1991, 15: 199-201.

Bonetti F, Pea M, Martignoni G, Zamboni G: PEC and Sugar [Letter]. Am J Surg Pathol. 1992, 16: 307-308. 10.1097/00000478-199203000-00013.

Apitz K: Die Geschwulste und Gewebsmissbildungen der Nierenrinde. II Midteilung. Die mesenchymalen Neubildungen. Virchows Arch. 1943, 311: 306-327.

Fadare O, Parkash V, Yilmaz Y, Mariappan MR, Ma L, Hileeto D, Qumsiyeh MB, Hui P: Perivascular epithelioid cell tumor (PEComa) of the uterine cervix associated with intraabdominal "PEComatosis": A clinicopathological study with comparative genomic hybridization analysis. World J Surg Oncol. 2004, 2: 35-10.1186/1477-7819-2-35.

Fadare O: Perivascular Epithelioid Cell Tumors and Smooth Muscle Tumors of the Uterus [Letter]. Am J Surg Pathol. 2007, 31: 1454-5. 10.1097/PAS.0b013e318039b218.

Fadare O, Hileeto D, Mariappan MR, Hui P: Perivascular Epithelioid Cell Tumor Not Otherwise Specified (PEComa-NOS) – A Review of 43 Reported Cases. [abstract]. Am J Clin Pathol. 2004, 122: 625-661.

Fadare O: Perivascular epithelioid cell tumor [PEComa] of the uterus: an outcome-based clinicopathologic analysis of 41 reported cases. Adv Anat Pathol. 2008, 15: 63-75. 10.1097/PAP.0b013e31816613b0.

Fadare O, Ghofrani M, Stamatakos MD, Tavassoli FA: Mesenchymal lesions of the uterine cervix. Pathol Case Reviews. 2006, 11: 140-152. 10.1097/01.pcr.0000217868.84282.77.

Folpe AL: Neoplasms with perivascular epithelioid cell differentiation (PEComas). World Health Organization Classification of Tumors: Pathology and Genetics of Tumors of Soft Tissue and Bone. Edited by: Fletcher CDM, Unni KK, Mertens F. 2002, Lyon: IARC Press, 221-222.

Martignoni G, Pea M, Reghellin D, Zamboni G, Bonetti F: Perivascular epithelioid cell tumor (PEComa) in the genitourinary tract. Adv Anat Pathol. 2007, 14: 36-41. 10.1097/PAP.0b013e31802e0dc4.

Hornick JL, Fletcher CD: PEComa: what do we know so far?. Histopathology. 2006, 48: 75-82. 10.1111/j.1365-2559.2005.02316.x.

Folpe AL, Mentzel T, Lehr HA, 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. 10.1097/01.pas.0000173232.22117.37.

Bonetti F, Pea M, Martignoni G, Zamboni G, Manfrin E, Colombari R, Mariuzzi GM: The perivascular epithelioid cell and related lesions. Adv Anat Pathol. 1997, 4: 343-358. 10.1097/00125480-199711000-00001.

Martignoni G, Pea M, Reghellin D, Zamboni G, Bonetti F: PEComas: the past, the present and the future. Virchows Arch. 2007 Dec 14.

Zamboni G, Pea M, Martignoni G, Zancanaro C, Faccioli G, Gilioli E, Pederzoli P, Bonetti F: 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: 722-30. 10.1097/00000478-199606000-00010.

Hendrickson MR, Tavassoli FA, Kempson RL, McCluggage WG, Haller U, Kubik-Huch RA: Mesenchymal tumors and related lesions. World Health Organization Classification of Tumors. Pathology and Genetics of Tumors of the Breast and Female Genital Organs. Edited by: Tavassoli FA, Devilee P. 2003, Lyon: IARC Press, 233-244.

Folpe AL, Goodman ZD, Ishak KG, Paulino AF, Taboada EM, Meehan SA, Weiss SW: 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: 1239-46. 10.1097/00000478-200009000-00007.

Pea M, Martignoni G, Zamboni G, Bonetti F: Perivascular epithelioid cell. Am J Surg Pathol. 1996, 20: 1149-53. 10.1097/00000478-199609000-00012.

Gan MF, Yu CK, Jin M, Lu HS, Li HM: Perivascular epithelioid cell tumor of the uterus: report of three cases. Chin Med J (Engl). 2007, 120: 526-8.

Rammeh Rommani S, Trabelsi A, Attia L, Msakni I, Koubaa A, Ben Jilani S, Zermani R: Perivascular epithelioid cell tumor of the uterus: a case report. Pathologica. 2006, 98: 649-51.

Azad NS, Aziz AB, Pervez S, Kayani N: Uterine perivascular epithelioid cell tumour presenting as a cervical mass. J Pak Med Assoc. 2006, 56: 83-4.

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: 681-4. 10.1097/01.mph.0000193475.06870.d5.

Bosincu L, Rocca PC, Martignoni G, Nogales FF, Longa L, Maccioni A, Massarelli G: Perivascular epithelioid cell (PEC) tumors of the uterus: a clinicopathologic study of two cases with aggressive features. Mod Pathol. 2005, 18: 1336-42. 10.1038/modpathol.3800433.

Fukunaga M: Perivascular epithelioid cell tumor of the uterus: report of four cases. Int J Gynecol Pathol. 2005, 24: 341-6. 10.1097/01.pgp.0000168515.83557.89.

Fukunaga M: Perivascular epithelioid cell tumor of the uterus: a case report. Int J Gynecol Pathol. 2004, 23: 287-91. 10.1097/01.pgp.0000130448.30412.79.

Bernardo Vega R, Camino FV, Frenandez MDC, Segura GQ, Gonzalez JM, Gonzalez-Tejero C: The PEComa Tumor: Could it be considered an independent neoplastic entity. J Gynecol Surg. 2005, 21: 161-166. 10.1089/gyn.2005.21.161.

Gao Z, Bhuiya T, Anderson A: Perivascular epithelioid cell tumour (PEComa) of the uterus associated with malignant neoplasm of the female genital tract. J Obstet Gynaecol. 2004, 24: 600-4. 10.1080/01443610410001722905.

Darai E, Bazot M, Barranger E, Detchev R, Cortez A: Epithelioid angiomyolipoma of the uterus: a case report. J Reprod Med. 2004, 49: 578-81.

Greene LA, Mount SL, Schned AR, Cooper K: Recurrent perivascular epithelioid cell tumor of the uterus (PEComa): an immunohistochemical study and review of the literature. Gynecol Oncol. 2003, 90: 677-81. 10.1016/S0090-8258(03)00325-1.

Park SH, Ro JY, Kim HS, Lee ES: Perivascular epithelioid cell tumor of the uterus: immunohistochemical, ultrastructural and molecular study. Pathol Int. 2003, 53: 800-5. 10.1046/j.1440-1827.2003.01557.x.

Dimmler A, Seitz G, Hohenberger W, Kirchner T, Faller G: Late pulmonary metastasis in uterine PEComa. J Clin Pathol. 2003, 56: 627-8. 10.1136/jcp.56.8.627.

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. 10.1097/00000478-200201000-00001.

Bonetti F, Martignoni G, Colato C, Manfrin E, Gambacorta M, Faleri M, Bacchi C, Sin VC, Wong NL, Coady M, Chan JK: Abdominopelvic sarcoma of perivascular epithelioid cells. Report of four cases in young women, one with tuberous sclerosis. Mod Pathol. 2001, 14: 563-8. 10.1038/modpathol.3880351.

Michal M, Zamecnik M: Hyalinized Uterine Mesenchymal Neoplasms with HMB-45-Positive Epithelioid Cells: Epithelioid Leiomyomas or Angiomyolipomas?. Report of Four Cases. 2000, 8: 323-328.

Zamecnik M, Michal M: HMB45+ hyalinized epithelioid tumor of the uterus is linked to epithelioid leiomyoma rather than to PEC-omas. Int J Surg Pathol. 2001, 9: 341-3. 10.1177/106689690100900415.

D'Andrea V, Lippolis G, Biancari F, Ruco LP, Marzullo A, Wedard BM, Di Matteo FM, Sarmiento R, Dibra A, De Antoni E: A uterine pecoma: a case report. G Chir. 1999, 20: 163-4.

Ruco LP, Pilozzi E, Wedard BM, Marzullo A, D'Andrea V, De Antoni E, Silvestrini G, Bonetti F: Epithelioid lymphangioleiomyomatosis-like tumour of the uterus in a patient without tuberous sclerosis: a lesion mimicking epithelioid leiomyosarcoma. Histopathology. 1998, 33: 91-3. 10.1046/j.1365-2559.1998.0415g.x.

Hornick JL, Fletcher CD: Sclerosing PEComa: Clinicopathologic Analysis of a Distinctive Variant With a Predilection for the Retroperitoneum. Am J Surg Pathol. 2008 Jan 24.

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. Diagn Pathol. 2007, 2: 45-10.1186/1746-1596-2-45.

Liang SX, Pearl M, Liu J, Hwang S, Tornos C: "Malignant" uterine perivascular epithelioid cell tumor, pelvic lymph node lymphangioleiomyomatosis, and gynecological pecomatosis in a patient with tuberous sclerosis: a case report and review of the literature. Int J Gynecol Pathol. 2008, 27: 86-90.

Cil AP, Haberal A, Hucumenoglu S, Kovalak EE, Gunes M: Angiomyolipoma of the uterus associated with tuberous sclerosis: case report and review of the literature. Gynecol Oncol. 2004, 94: 593-6. 10.1016/j.ygyno.2004.05.015.

Laffargue F, Giacalone PL, Charpin C, Lachard A: Uterine angiomyolipoma associated with pregnancy. Gynecol Oncol. 1993, 50: 357-60. 10.1006/gyno.1993.1225.

Braun HL, Wheelock JB, Amaker BH, Seeds JW: Sonographic evaluation of a uterine angiolipoleiomyoma. J Clin Ultrasound. 2002, 30: 241-4. 10.1002/jcu.10057.

Yaegashi H, Moriya T, Soeda S, Yonemoto Y, Nagura H, Sasano H: Uterine angiomyolipoma: case report and review of the literature. Pathol Int. 2001, 51: 896-901. 10.1046/j.1440-1827.2001.01289.x.

Shintaku M: Lipoleiomyomatous tumors of the uterus: a heterogeneous group? Histopathological study of five cases. Pathol Int. 1996, 46: 498-502.

Wang X, Kumar D, Seidman JD: Uterine lipoleiomyomas: a clinicopathologic study of 50 cases. Int J Gynecol Pathol. 2006, 25: 239-42. 10.1097/01.pgp.0000192273.66931.29.

Gyure KA, Hart WR, Kennedy AW: Lymphangiomyomatosis 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, 14: 344-51.

Torres VE, Björnsson J, King BF, Kumar R, Zincke H, Edell ES, Wilson TO, Hattery RR, Gomez MR: Extrapulmonary lymphangioleiomyomatosis and lymphangiomatous cysts in tuberous sclerosis complex. Mayo Clin Proc. 1995, 70: 641-8.

Longacre TA, Hendrickson MR, Kapp DS, Teng NN: Lymphangioleiomyomatosis of the uterus simulating high-stage endometrial stromal sarcoma. Gynecol Oncol. 1996, 63: 404-10. 10.1006/gyno.1996.0344.

Prasad SR, Sahani DV, Mino-Kenudson M, Narra VR, Humphrey PA, Menias CO, Chintapalli KN: Neoplasms of the perivascular epithelioid cell involving the abdomen and the pelvis: cross-sectional imaging findings. J Comput Assist Tomogr. 2007, 31: 688-96.

Lendvay TS, Marshall FF: The tuberous sclerosis complex and its highly variable manifestations. J Urol. 2003, 169: 1635-42. 10.1097/01.ju.0000058253.40352.60.

Pan CC, Jong YJ, Chai CY, Huang SH, Chen YJ: 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: 606-12. 10.1016/j.humpath.2006.01.008.

Kurman RJ, Norris HJ: Mesenchymal tumors of the uterus. VI. Epithelioid smooth muscle tumors including leiomyoblastoma and clear-cell leiomyoma: a clinical and pathologic analysis of 26 cases. Cancer. 1976, 37: 1853-65. 10.1002/1097-0142(197604)37:4<1853::AID-CNCR2820370433>3.0.CO;2-E.

Prayson RA, Goldblum JR, Hart WR: Epithelioid smooth-muscle tumors of the uterus: a clinicopathologic study of 18 patients. Am J Surg Pathol. 1997, 21: 383-91. 10.1097/00000478-199704000-00003.

Oliva E, Nielsen PG, Clement PB, Young RH, Scully RE: Epithelioid smooth muscle tumors of the uterus. A clinicopathologic study of 80 cases. [abstract]. Mod Pathol. 1997, 10: 107A-

Atkins K, Bell S, Kempson R, Hendrickson M: Epithelioid smooth muscle tumors of the uterus [abstract]. Mod Pathol. 2001, 14: 132A-

Silva EG, Deavers MT, Bodurka DC, Malpica A: Uterine epithelioid leiomyosarcomas with clear cells: reactivity with HMB-45 and the concept of PEComa. Am J Surg Pathol. 2004, 28: 244-9. 10.1097/00000478-200402000-00013.

Silva EG, Bodurka DC, Scouros MA, Ayala A: A uterine leiomyosarcoma that became positive for HMB45 in the metastasis. Ann Diagn Pathol. 2005, 9: 43-5. 10.1053/j.anndiagpath.2004.10.011.

Silva EG, Tornos C, Ordonez NG, Morris M: Uterine leiomyosarcoma with clear cell areas. Int J Gynecol Pathol. 1995, 14: 174-8.

Kempson RL, Hendrickson MR: Smooth muscle, endometrial stromal, and mixed Müllerian tumors of the uterus. Mod Pathol. 2000, 13: 328-42. 10.1038/modpathol.3880055.

Smolarek TA, Bejarano PA, Heffelfinger S, Menon AG: HMB-45 immunoreactivity is present in both uterine leiomyomas and normal myometrium [abstract]. Mod Pathol. 1999, 12: 125-

Williams ME, Gainey TW, Lamb T, Bach D, Kornstein MJ, Burks RT: HMB-45 immunoreactivity in uterine mesenchymal tumors [abstract]. Mod Pathol. 2000, 13: 120-10.1038/modpathol.3880121.

Oliva E, Wang WL, Branton P, Logani S, Zannoni GF, Linkov I, Asher M, Soslow R: Expression of melanocytic ("PEComa") markers in smooth muscle tumors of the uterus: an immunohistochemical analysis of 86 cases [abstract]. Mod Pathol. 2006, 86: 191A-

Simpson KW, Albores-Saavedra J: HMB-45 reactivity in conventional uterine leiomyosarcomas. Am J Surg Pathol. 2007, 31: 95-8. 10.1097/01.pas.0000213346.57391.70.

Hurrell DP, McCluggage WG: Uterine leiomyosarcoma with HMB45+ clear cell areas: report of two cases. Histopathology. 2005, 47: 540-2. 10.1111/j.1365-2559.2005.02147.x.

Barcus ME, Williams RI, Gainey TW: HMB-45 immunoreactivity in uterine mesenchymal tumors. Mod Pathol. 2000, 13: 120A-

Magro CM, Crowson AN, Mihm MC: Unusual variants of malignant melanoma. Mod Pathol. 2006, 19 (Suppl 2): S41-70. 10.1038/modpathol.3800516.

Martin PC, Pulitzer DR, Reed RJ: Pigmented myomatous neurocristoma of the uterus. Arch Pathol Lab Med. 1989, 113: 1291-5.

Etchevers HC, Couly G, Le Douarin NM: Morphogenesis of the branchial vascular sector. Trends Cardiovasc Med. 2002, 12: 299-304. 10.1016/S1050-1738(02)00178-0.

Etchevers HC, Vincent C, Le Douarin NM, Couly GF: The cephalic neural crest provides pericytes and smooth muscle cells to all blood vessels of the face and forebrain. Development. 2001, 128: 1059-1068.

Boot MJ, Steegers-Theunissen RP, Poelmann RE, Van Iperen L, Lindemans J, Gittenberger-de Groot AC: Folic acid and homocysteine affect neural crest and neuroepithelial cell outgrowth and differentiation in vitro. Dev Dyn. 2003, 227: 301-308. 10.1002/dvdy.10303.

Shah NM, Groves AK, Anderson DJ: Alternative neural crest cell fates are instructively promoted by TGFbeta superfamily members. Cell. 1996, 85: 331-43. 10.1016/S0092-8674(00)81112-5.

Jain MK, Layne MD, Watanabe M, Chin MT, Feinberg MW, Sibinga NE, Hsieh CM, Yet SF, Stemple DL, Lee ME: In vitro system for differentiating pluripotent neural crest cells into smooth muscle cells. J Biol Chem. 1998, 273: 5993-5996. 10.1074/jbc.273.11.5993.

Rizeq MN, van de Rijn M, Hendrickson MR, Rouse RV: A comparative immunohistochemical study of uterine smooth muscle neoplasms with emphasis on the epithelioid variant. Hum Pathol. 1994, 25: 671-7. 10.1016/0046-8177(94)90300-X.

Oliva E, Young RH, Amin MB, Clement PB: An immunohistochemical analysis of endometrial stromal and smooth muscle tumors of the uterus: a study of 54 cases emphasizing the importance of using a panel because of overlap in immunoreactivity for individual antibodies. Am J Surg Pathol. 2002, 26: 403-12. 10.1097/00000478-200204000-00001.

Hyde KE, Geisinger KR, Marshall RB, Jones TL: The clear-cell variant of uterine epithelioid leiomyoma. An immunohistologic and ultrastructural study. Arch Pathol Lab Med. 1989, 113: 551-3.

Mazur MT, Priest JB: Clear cell leiomyoma (leiomyoblastoma) of the uterus: ultrastructural observations. Ultrastruct Pathol. 1986, 10: 249-55. 10.3109/01913128609032224.

Everitt JI, Wolf DC, Howe SR, Goldsworthy TL, Walker C: Rodent model of reproductive tract leiomyomata. Clinical and pathological features. Am J Pathol. 1995, 146: 1556-67.

Pan CC, Chung MY, Ng KF, Liu CY, Wang JS, Chai CY, Huang SH, Chen PH, Ho D: 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: 387-93. 10.1002/path.2289.

Adachi Y, Horie Y, Kitamura Y, Nakamura H, Taniguchi Y, Miwa K, Fujioka S, Nishimura M, Hayashi K: CD1a expression in PEComas. Pathol Int. 2008, 58: 169-173. 10.1111/j.1440-1827.2007.02206.x.

Kenerson H, Folpe AL, Takayama TK, Yeung RS: Activation of the mTOR pathway in sporadic angiomyolipomas and other perivascular epithelioid cell neoplasms. Hum Pathol. 2007, 38: 1361-71. 10.1016/j.humpath.2007.01.028.