Skip to main content
Key Specialties
Cardiology Diabetology Endocrinology Gastroenterology Geriatrics and Gerontology Gynecology and Obstetrics Hematology Infectious Disease Internal Medicine Nephrology Neurology Oncology Pediatrics Respiratory Medicine Rheumatology Surgery
Congress Coverage
2024 ACC Congress 2023 ESMO Congress 2023 EASD Congress 2023 ERS Congress 2023 ESC Congress
Clinical Collections
Advances in Alzheimer’s

Keynote webinar | Spotlight on medication adherence

LIVE: Thursday 27th June 2024, 18:00-19:30 (CEST)
Join our expert panel to discover why you need to understand the drivers of non-adherence in your patients, and how you can optimize medication adherence in your clinics to drastically improve patient outcomes.
ADVANCED SEARCH
Log in
Top
World Journal of Surgical Oncology
Published in: World Journal of Surgical Oncology 1/2004

Open Access 01-12-2004 | Review

Perivascular epithelioid cell tumor (PEComa) of the uterine cervix associated with intraabdominal "PEComatosis": A clinicopathological study with comparative genomic hybridization analysis

Authors: Oluwole Fadare, Vinita Parkash, Yesim Yilmaz, M Rajan Mariappan, Linglei Ma, Denise Hileeto, Mazin B Qumsiyeh, Pei Hui

Published in: World Journal of Surgical Oncology | Issue 1/2004

Login to get access

Abstract

Background

The World Health Organization recently recognized a family of neoplasms showing at least partial morphological or immunohistochemical evidence of a putative perivascular epithelioid cell (PEC) differentiation. These tumors include angiomyolipoma (AML), clear cell "sugar" tumors of the lung (CCST), lymphangioleiomyomatosis (LAM), clear cell myomelanocytic tumors of the falciform ligament and distinctive clear cell tumors at various other anatomic sites.

Case presentation & methods

A 41-year old gravida-1 para-1 with tuberous sclerosis presented with an incidentally identified 2.2 cm mass. The morphology and immunohistochemical profile was consistent with PEComa. Distinct aggregates of HMB-45 epithelioid cells were present in an occasionally distinctive perivascular distribution in the myometrium, small bowel lamina propria and ovarian hila. These distinctive aggregates, for which we propose the designation "PEComatosis" based on their intraabdominal distribution, did not display cytological atypia, mitotic activity or necrosis. CGH and DNA ploidy analysis showed a balanced chromosomal profile and diploid nuclei, respectively. There was no recurrence or metastases at 35 months' follow-up. Fifty-one previously reported cases of non-AML, LAM and CCST PEComas [perivascular epithelioid cell tumors- not otherwise specified (PEComa-NOS)] are reviewed.

Conclusions

The lesions may be a reflection of tumor multicentricity, in which each may be a potential nidus for the development of future more well-developed tumors. Alternatively, they may be a manifestation of a poorly understood "field effect", in which there is an increased propensity to develop tumors of this type throughout the abdomen. Finally, and least likely in our opinion, they may represent tumor spread from its primary site.
Appendix
Available only for authorised users
Literature
1.
Adachi S, Hanada M, Kobayashi Y, Tsutahara K, Fukuhara S, Mori N, Hara T, Mukai H, Shimasaku E, Kawai M, Kishikawa N, Yamaguchi S: Heavily melanotic perivascular epithelioid cell tumor of the kidney. Pathol Int. 2004, 54: 261-265. 10.1111/j.1440-1827.2004.01617.x.CrossRefPubMed
2.
Bonetti F, Pea M, Martignoni G, Zamboni G: The perivascular epithelioid cell and related lesions. Adv Anat Pathol. 1997, 4: 343-358.CrossRef
3.
Nuciforo PG, O'Hara CD, 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. And Tazelaar HD, Batts KP, Srigley JR: Primary extrapulmonary sugar tumor (PEST): a report of four cases. Mod Pathol 2001, 14:615–22. Mod Pathol 2002, 15:87–90[MP1].CrossRef
4.
5.
Bonetti F, Pea M, Martignoni G, Doglioni C, Zamboni G, Capelli P, Rimondi P, Andrion A: Clear cell ("sugar") tumor of the lung is strictly related to angiomyolipoma: the concept of a family of lesions characterized by the presence of the perivascular epithelioid cell (PEC). Pathology. 1994, 26: 230-236.CrossRefPubMed
6.
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: 563-568. 10.1038/modpathol.3880351.CrossRefPubMed
7.
Crowson AN, Taylor JR, Magro CM: Cutaneous clear cell myomelanocytic tumor-perivascular epithelioid cell tumor: first reported case [Abstract]. Mod Pathol. 2003, 16: 90A-
8.
Fukunaga M: Perivascular epithelioid cell tumor of the uterus: a case report. Int J Gynecol Pathol. 2004, 23: 287-291. 10.1097/01.pgp.0000130448.30412.79.CrossRefPubMed
9.
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: 163-164.PubMed
10.
Diment J, Colecchia M: Myomelanocytic tumor of the thigh. Am J Surg Pathol. 2003, 27: 1288-10.1097/00000478-200309000-00019.CrossRefPubMed
11.
Dimmler A, Seitz G, Hohenberger W, Kirchner T, Faller G: Late pulmonary metastases in uterine PEComa. J Clin Pathol. 2002, 56: 627-628. 10.1136/jcp.56.8.627.CrossRef
12.
Fukunaga M: Perivascular epithelioid cell tumor (PEComa) of soft tissue: case report with ultrastructural study. APMIS. 2004, 112: 98-104. 10.1111/j.1600-0463.2004.apm1120203.x.CrossRefPubMed
13.
Folpe AL: Neoplasms with perivascular epithelioid cell differentiation (PEComas). In: 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.
14.
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.CrossRefPubMed
15.
Folpe AL, McKenney JK, Li Z, Smith SJ, Weiss SW: Clear cell myomelanocytic tumor of the thigh. Am J Surg Pathol. 2002, 26: 809-812. 10.1097/00000478-200206000-00018.CrossRefPubMed
16.
Gaffey MJ, Zarbo RJ, Weiss LM, Bonetti F, Pea M, Martignoni G, Zamboni G: PEC and Sugar. Am J Surg Pathol. 1992, 16: 307-308.PEC and sugar.,CrossRef
17.
Gaffey MJ, Mills SE: Clear cell tumor and angiomyolipoma [Letter]. Am J Surg Pathol. 1991, 15: 201-202.CrossRef
18.
Gaffey MJ, Mills SE, Zarbo RJ, Weiss LM: Clear cell tumor of the lung. Immunohistochemical and ultrastructural evidence of melanogenesis. Am J Surg Pathol. 1991, 15: 644-653.CrossRefPubMed
19.
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: 677-681. 10.1016/S0090-8258(03)00325-1.CrossRefPubMed
20.
Govender D, Sabaratnam RM, Essa AS: Clear cell "sugar" tumor of the breast. Am J Surg Pathol. 2002, 26: 670-675. 10.1097/00000478-200205000-00014.CrossRefPubMed
21.
Hendrickson MR, Tavassoli FA, Kempson RL, McCluggage WG, Haller U, Kubik-Huch RA: Mesenchymal tumors and related lesions. In: 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.
22.
Lehman NL: Malignant PEComa of the skull base. Am J Surg Pathol. 2004, 28: 1230-1232. 10.1097/01.pas.0000128668.34934.81.CrossRefPubMed
23.
Kung M, Landa JF, Lubin J: Benign clear cell tumor ("sugar") tumor of the trachea. Cancer. 1984, 54: 517-551.CrossRefPubMed
24.
Manganaro L, Ballesio L, Angeli ML, Bertini L, Di Seri M: US CT and MR findings in pecoma metastases. A case report. Radiol Med (Torino). 2002, 103: 433-436.
25.
Michal M, Zamecnik M: Hyalinized uterine mesenchymal neoplasms with HMB-45 positive epithelioid cells: epithelioid leiomyomas or angiomyolipomas?. Int J Surg Pathol. 2000, 8: 323-328.CrossRefPubMed
26.
Nuciforo PG, O'Hara CD: Correspondence. Mod Pathol. 2002, 15: 87-88. 10.1038/modpathol.3880495. Re: 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 and Tazelaar HD, Batts KP, Srigley JR: Primary extrapulmonary sugar tumor (PEST): a report of four cases. Mod Pathol 2001, 14:615–22.CrossRefPubMed
27.
Pan CC, Yu IT, Yang AH, Chiang H: Clear cell myomelanocytic tumor of the urinary bladder. Am J Surg Pathol. 2003, 27: 689-692. 10.1097/00000478-200305000-00013.CrossRefPubMed
28.
Pan CC, Yang AH, Chiang H: Malignant perivascular epithelioid cell tumor involving the prostate. Arch Pathol Lab Med. 2003, 127: e96-e98.PubMed
29.
Panizo-Santos A, Sola I, De Alava E, Lozano MD, Idoate MA, Pardo FJ: Angiomyolipoma and PEComa are immunoreactive for MyoD1 in cell cytoplasmic staining pattern. Appl Immunohistochem Mol Morphol. 2003, 11: 156-160.CrossRefPubMed
30.
Panizo A, Sola JJ, de Alava E, Idoate M, Toledo G, Pardo J: Angiomyolipoma and PEComa are immunoreactive for MyoD1 in cell cytoplasmic staining pattern [Abstract]. Mod Pathol. 2003, 16: 165A-10.1038/modpathol.3880736.CrossRef
31.
Park SH, Ro JY, Kim HS, Lee ES: Perivascular epithelioid cell tumor of the uterus: immunohistochemical, ultrastructural and molecular study. Pathol Int. 2003, 53: 800-805. 10.1046/j.1440-1827.2003.01557.x.CrossRefPubMed
32.
Pea M, Martignoni G, Zamboni G, Bonetti F: Perivascular epithelioid cell. Am J Surg Pathol. 1996, 20: 1149-1153. 10.1097/00000478-199609000-00012.CrossRefPubMed
33.
Pea M, Bonneti F, Zamboni G, Martignoni G, Fiore-Donati L, Doglioni C: Clear cell tumor and angiomyolipoma. Am J Surg Pathol. 1991, 15: 199-202.CrossRefPubMed
34.
Ruco LP, Pilozzi E, Wedard BM, Marzullo A, D'Andrea V, De Antoni E, Silvestrini G, Bonetti F: Epithelioid lymphangioleiomyomatosis-like tumor of the uterus in a patient without tuberous sclerosis: a lesion mimicking epithelioid leiomyosarcoma. Histopathology. 1998, 33: 91-93. 10.1046/j.1365-2559.1998.0415g.x.CrossRefPubMed
35.
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: 244-249. 10.1097/00000478-200402000-00013.CrossRefPubMed
36.
Sola JJ, Gomez-Roman J, Panizo A, Idoate MA, Lozano MD, Val-Bernal JF, Pardo J: Poor correlation between IHC and RT-PCR detection of tyrosinase in tumors derived from perivascular epithelioid cells (PEC tumors) [Abstract]. Mod Pathol. 2003, 16: 172A-
37.
Tanaka Y, Ijiri R, Kato K, Kato Y, Nakatani Y, Hara M: HMB-45/Melan-A and smooth muscle actin-positive clear-cell epithelioid tumor arising in the ligament teres hepatis: additional example of clear cell "sugar" tumors. Am J Surg Pathol. 2000, 24: 1295-1299. 10.1097/00000478-200009000-00015.CrossRefPubMed
38.
Tazelaar HD, Batts KP, Srigley JR: Primary extrapulmonary sugar tumor (PEST): a report of four cases. Mod Pathol. 2001, 14: 615-622. 10.1038/modpathol.3880360.CrossRefPubMed
39.
Tazelaar HD, Batts KP: In Reply. Re: Nuciforo PG, O'Hara CD: Mod Pathol. 2002, 15: 87-88. Mod Pathol 2002, 15:89–90.
40.
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.CrossRefPubMed
41.
Yanai H, Matsuura H, Sonobe H, Shiozaki S, Kawabata K: Perivascular epithelioid cell tumor of the jejunum. Pathol Res Pract. 2003, 199: 47-50.CrossRefPubMed
42.
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: 722-730. 10.1097/00000478-199606000-00010.CrossRefPubMed
43.
Sadeghi S, Krigman H, Maluf H: Perivascular epithelioid clear cell tumor of the common bile duct. Am J Surg Pathol. 2004, 28: 1107-1110. 10.1097/01.pas.0000116831.81882.d0.CrossRefPubMed
44.
Bhalla R, Dail D, Kozlowski P, Corman J: Multiple bilateral perivascular epithelioid cell tumor (PEComa) of the kidneys. Can J Urol. 2004, 11: 2296-2298.PubMed
45.
Fink D, Mardsen DE, Edwards L, Camaris C, Hacker NF: Malignant perivascular epithelioid cell tumor (PEComa) arising in the broad ligament. Int J Gynecol Cancer. 2004, 14: 1036-1039. 10.1111/j.1048-891X.2004.014549.x.CrossRefPubMed
46.
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: 600-604. 10.1080/01443610410001722905.CrossRefPubMed
47.
Garcia TR, Mestre de Juan MJ: Angiomyolipoma of the liver and lung: a case explained by the presence of perivascular epithelioid cells. Pathol Res Pract. 2002, 198: 363-367.CrossRefPubMed
48.
Cummings RJ, O'Grady A, Sabah M, Kay E, Leader M: Comparative genomic hybridization: emphasis on use of formalin-fixed paraffin-embedded tissue. Curr Diagn Pathol. 2003, 9: 173-178. 10.1016/S0968-6053(02)00097-2.CrossRef
49.
Hui P, Riba A, Pejovic T, Johnson T, Baergen RN, Ward D: Comparative genomic hybridization study of placental site trophoblastic tumor: a report of 4 cases. Mod Pathol. 2004, 17: 248-251. 10.1038/modpathol.3800025.CrossRefPubMed
50.
Fadare O, Bifulco C, Carter D, Parkash V: Cartilaginous differentiation in peritoneal tissues: a report of two cases and a review of the literature. Mod Pathol. 2002, 15: 777-780. 10.1097/01.MP.0000017565.19341.63.CrossRefPubMed
51.
Robboy SJ, Scully RE: Ovarian teratoma with glial implants on the peritoneum: an analysis of 12 cases. Hum Pathol. 1970, 1: 643-653.CrossRefPubMed
52.
Rosai J: Peritoneum, retroperitoneum, and related structures. In: Ackerman's Surgical Pathology. Edited by: Rosai J. 1996, St Louis: Mosby, 2: 2135-2172. 8
53.
Hironaka M, Fukayama M: Regional proliferation of HMB-45-positive clear cells of the lung with lymphangioleiomyomatosis-like distribution, replacing the lobes with multiple cysts and a nodule. Am J Surg Pathol. 1999, 23: 1288-1293. 10.1097/00000478-199910000-00016.CrossRefPubMed
54.
Chuah KL, Tan PH: Multifocal micronodular pneumocyte hyperplasia, lymphangioleiomyomatosis and clear cell micronodules of the lung in a Chinese female patient with tuberous sclerosis. Pathology. 1998, 30: 242-246.CrossRefPubMed
55.
Chiodera PL, Pea M, Martignoni G, Bonetti F: Pulmonary lymphangioleiomyomatosis associated with miliariform 'sugar' tumor. Int J Surg Pathol. 1995, 2 (Suppl): 483-486.
56.
Pileri SA, Cavazza A, Schiavina M, Zompatori M, Pederzoli M, Goldfischer M, Sabattini E, Ascani S, Pasquinelli G, Bonetti F, Colby TV: Clear-cell proliferation of the lung with lymphangioleiomyomatosis-like change. Histopathology. 2004, 44: 156-163.CrossRefPubMed
57.
Henske EP: Metastasis of benign tumor cells in tuberous sclerosis complex. Genes Chromosomes Cancer. 2003, 38: 376-381. 10.1002/gcc.10252.CrossRefPubMed
58.
Silva EG, Deavers MT, Bodurka D, Malpica A: Uterine epithelioid leiomyosarcomas with clear cells or malignant PEComas? [Abstract]. Mod Pathol. 2003, 16: 211A-10.1097/01.MP.0000057236.96797.07.CrossRef
59.
Zamecnik M, Michal M: HMB-45+ hyalinized epithelioid tumor of the uterus is linked to epithelioid leiomyoma rather than to PEC-omas. Int J Surg Pathol. 2001, 9: 341-343.CrossRefPubMed
60.
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-
61.
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.CrossRef
Metadata
Title
Perivascular epithelioid cell tumor (PEComa) of the uterine cervix associated with intraabdominal "PEComatosis": A clinicopathological study with comparative genomic hybridization analysis
Authors
Oluwole Fadare
Vinita Parkash
Yesim Yilmaz
M Rajan Mariappan
Linglei Ma
Denise Hileeto
Mazin B Qumsiyeh
Pei Hui
Publication date
01-12-2004
Publisher
BioMed Central
Published in
World Journal of Surgical Oncology / Issue 1/2004
Electronic ISSN: 1477-7819
DOI
https://doi.org/10.1186/1477-7819-2-35

Other articles of this Issue 1/2004

World Journal of Surgical Oncology 1/2004 Go to the issue

Research

Metallopanstimulin as a marker for head and neck cancer

Case report

Pseudomyxoma extraperitonei occurring 35 years after appendicectomy: a case report and review of literature

Case report

Recurrence of primary extramedullary plasmacytoma in breast both simulating primary breast carcinoma

Case report

Concurrent pleomorphic adenoma in parapharyngeal space and submandibular gland

Review

Barrett's oesophagus and adenocarcinoma

Case report

Rupture of totally implantable central venous access devices (Intraports) in patients with cancer: report of four cases