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Lee, Lee, Choi, and Seo: A case of perivascular epithelioid cell tumor of the uterus
Case Report
Korean Journal of Obstetrics & Gynecology

Korean Journal of Obstetrics & Gynecology 2012; 55(6): 433-436.

Published online: 16 June 2012

DOI: https://doi.org/10.5468/KJOG.2012.55.6.433

A case of perivascular epithelioid cell tumor of the uterus

Yu Bin Lee, MD, Yoo Jin Lee, MD, Young Sik Choi, MD, Seok Kyo Seo, MD

Department of Obstetrics and Gynecology, Institute of Women's Life Medical Science, Gagnam Severance Hospital, Yonsei University College of Medicine, Seoul, Korea.

Corresponding author: Seok Kyo Seo, MD. Department of Obstetrics and Gynecology, Yonsei University College of Medicine, 50 Yonsei-ro, Seodaemun-gu, Seoul 120-749, Korea. Tel: +82-2-2228-2236, Fax: +82-2-313-8357, tudeolseo@yuhs.ac

Received 7 November 2011       Revised 21 April 2012       Accepted 3 May 2012

Copyright © 2012. Korean Society of Obstetrics and Gynecology

(open-access):

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Perivascular epithelioid cell tumor (PEComa) is a family of rare mesenchymal tumors that include angiomyolipoma, lymphangiomyomatosis and clear cell 'sugar' tumor of the lung, with distinctive histologic and immunohistochemical features. They can originate in any visceral organ such as kidney, lung, breast, urinary bladder, pancreas, prostate and gastrointestinal tract, and there are few reports of uterine PEComa. Histopathologically, the tumor consisted of nests or sheets of epithelioid cells with eosinophilic cytoplasm and positivity for melanocytic markers (especially HMB-45) plays an important role in the diagnosis of this tumor. Due to their rarity and varied sites and presentation, the biological behavior of PEComa has not yet been documented. We report a case of uterine PEComa confirmed by immunohistochemical staining and the histopathologic findings with a brief review of the literatures.

Keywords: Perivascular epithelioid cell tumor, Uterus, HMB-45

Figures and Tables

Fig. 1
Preoperative ultrasonography. Well marginated, oval shaped hypoechoic lesion is noted on the fundus of the uterus.
kjog-55-433-g001
Fig. 2
Operative findings shows moderate amount of hemoperitoneum (A) and 2×2 cm sized uterine mass on uterine fundus (B).
kjog-55-433-g002
Fig. 3
Basic characteristic features of perivascular epithelioid cells include abundant clear to lightly eosinophilic granular cytoplasm, normochromatic ovoid nuclei (H&E, ×400) (A) with an immunoprofile of HMB-45 positivity (B).
kjog-55-433-g003

References

1. Bonetti F, Pea M, Martignoni G, Zamboni G. PEC and sugar. Am J Surg Pathol. 1992. 16:307–308.
2. Bonetti F, Martignoni G, Colato C, Manfrin E, Gambacorta M, Faleri M, 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.
3. Bonetti F, Pea M, Martignoni G, Doglioni C, Zamboni G, Capelli P, 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.
4. Martignoni G, Pea M, Rigaud G, Manfrin E, Colato C, Zamboni G, et al. Renal angiomyolipoma with epithelioid sarcomatous transformation and metastases: demonstration of the same genetic defects in the primary and metastatic lesions. Am J Surg Pathol. 2000. 24:889–894.
5. 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.
6. 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. 29:1558–1575.
7. Pan CC, Yu IT, Yang AH, Chiang H. Clear cell myomelanocytic tumor of the urinary bladder. Am J Surg Pathol. 2003. 27:689–692.
8. Kwon HJ, Choi SJ, Kim KS, Hwang SO, Song ES, Lee BI, et al. A case of perivascular epithelioid cell tumor (PEComa) at the uterus. Korean J Obstet Gynecol. 2007. 50:1428–1432.
9. Folpe AL, Kwiatkowski DJ. Perivascular epithelioid cell neoplasms: pathology and pathogenesis. Hum Pathol. 2010. 41:1–15.
10. Fadare O. Perivascular epithelioid cell tumors (PEComas) and smooth muscle tumors of the Uterus. Am J Surg Pathol. 2007. 31:1454–1455.
11. Pea M, Bonetti F, Zamboni G, Martignoni G, Fiore-Donati L, Doglioni C. Clear cell tumor and angiomyolipoma. Am J Surg Pathol. 1991. 15:199–202.
12. Pea M, Bonetti F, Zamboni G, Martignoni G, Riva M, Colombari R, et al. Melanocyte-marker-HMB-45 is regularly expressed in angiomyolipoma of the kidney. Pathology. 1991. 23:185–188.
13. 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.
14. Wagner AJ, Malinowska-Kolodziej I, Morgan JA, Qin W, Fletcher CD, Vena N, et al. Clinical activity of mTOR inhibition with sirolimus in malignant perivascular epithelioid cell tumors: targeting the pathogenic activation of mTORC1 in tumors. J Clin Oncol. 2010. 28:835–840.
15. 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.
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