A Case of Malignant Perivascular Epithelioid Cell Tumor of the Retroperitoneum with Multiple Metastases

Hye Won Oh; Tae Hyo Kim; Ra Ri Cha; Na Young Kim; Hyun Jin Kim; Woon Tae Jung; Ok Jae Lee; Jeong Hee Lee

doi:10.4166/kjg.2014.64.5.302

Journal List > Korean J Gastroenterol > v.64(5) > 1007302

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Oh, Kim, Cha, Kim, Kim, Jung, Lee, and Lee: A Case of Malignant Perivascular Epithelioid Cell Tumor of the Retroperitoneum with Multiple Metastases

Case Report

Korean J Gastroenterol 2014;64(5):302-306.

Published online: 4 October 2014

DOI: https://doi.org/10.4166/kjg.2014.64.5.302

A Case of Malignant Perivascular Epithelioid Cell Tumor of the Retroperitoneum with Multiple Metastases

Hye Won Oh^1,³, Tae Hyo Kim^1,³, Ra Ri Cha^1,³, Na Young Kim^1,³, Hyun Jin Kim^1,³, Woon Tae Jung^1,³, Ok Jae Lee^1,³, Jeong Hee Lee^2,³

¹Department of Internal Medicine, Gyeongsang National University School of Medicine, Jinju, Korea

²Department of Pathology, Gyeongsang National University School of Medicine, Jinju, Korea

³Institute of Health, Gyeongsang National University School of Medicine, Jinju, Korea

Correspondence to: Tae Hyo Kim, Department of Internal Medicine, Gyeongsang National University Hospital, 79 Gangnam-ro, Jinju 660-702, Korea. Tel: +82-55-750- 8726, Fax: +82-55-755-9078, E-mail: kimthy@medimail.co.kr

Received 7 February 201417 March 2014 Accepted 18 March 2014

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 tumors (PEComas) are unusual mesenchymal neoplasms composed of histologically and immunohistochemically distinct perivascular epithelioid cells (PECs). Although PEComas have the potential to behave in a malignant fashion, malignant PEComas arising from the retroperitoneum are extremely rare. A 68-year-old woman presented with a painful palpable mass in her left upper abdomen. Computed tomography of the abdomen showed a 9 cm sized heterogeneous mass in left para-aortic space and multiple hypervascular nodules in the liver. ¹⁸ F-fludeoxyglucose-PET/CT showed multifocal hyper-metabolic lesions in retroperitoneum, liver, and skeletal bones. Percutaneous needle biopsies were done on the retroperitoneal and hepatic mass. Both specimens were positive for human melanoma black-45 (HMB-45) on histological and immunohistochemical staining which was compatible with PEComas. Herein, we report a rare case of retroperitoneal PEComa with multiple metastases involving liver and bone at initial diagnosis that exhibited aggressive behavior and resulted in a devastating prognosis.

Keywords: Perivascular epithelioid cell tumor, Metastasis, Retroperitoneal, Liver, Bone

References

1. Bonetti F, Pea M, Martignoni G, Zamboni G. PEC and sugar. Am J Surg Pathol. 1992; 16:307–308.

2. Pea M, Martignoni G, Zamboni G, Bonetti F. Perivascular epithelioid cell. Am J Surg Pathol. 1996; 20:1149–1153.

3. Folpe AL, Kwiatkowski DJ. Perivascular epithelioid cell neoplasms: pathology and pathogenesis. Hum Pathol. 2010; 41:1–15.

4. Folpe AL, Mentzel T, Lehr HA, Fisher C, Balzer BL, Weiss SW. Perivascular epithelioid cell neoplasms of soft tissue and gyne-cologic origin: a clinicopathologic study of 26 cases and review of the literature. Am J Surg Pathol. 2005; 29:1558–1575.

5. Hornick JL, Fletcher CD. Sclerosing PEComa: clinicopathologic analysis of a distinctive variant with a predilection for the retroperitoneum. Am J Surg Pathol. 2008; 32:493–501.

6. Ruco LP, Pilozzi E, Wedard BM, et al. Epithelioid lymphangioleio-myomatosis-like tumour of the uterus in a patient without tuber-ous sclerosis: a lesion mimicking epithelioid leiomyosarcoma. Histopathology. 1998; 33:91–93.

7. Hornick JL, Fletcher CD. PEComa: what do we know so far? Histopathology. 2006; 48:75–82.

8. Yokoo H, Isoda K, Nakazato Y, et al. Retroperitoneal epithelioid angiomyolipoma leading to fatal outcome. Pathol Int. 2000; 50:649–654.

9. Lau SK, Marchevsky AM, McKenna RJ Jr, Luthringer DJ. Malignant monotypic epithelioid angiomyolipoma of the retroperitoneum. Int J Surg Pathol. 2003; 11:223–228.

10. Gupta C, Malani AK, Gupta V, Singh J, Ammar H. Metastatic retroperitoneal epithelioid angiomyolipoma. J Clin Pathol. 2007; 60:428–431.

11. Lans TE, van Ramshorst GH, Hermans JJ, den Bakker MA, Tran TC, Kazemier G. Perivascular epithelioid cell tumor of the retroperitoneum in a young woman resulting in an abdominal chyloma. J Gastrointest Surg. 2009; 13:389–392.

12. Subbiah V, Trent JC, Kurzrock R. Resistance to mammalian tar-get of rapamycin inhibitor therapy in perivascular epithelioid cell tumors. J Clin Oncol. 2010; 28:e415.

13. de León DC, Pérez-Montiel D, Bandera A, Villegas C, Gonzalez- Conde E, Vilchis JC. Perivascular epithelioid cell tumor of abdominal origin. Ann Diagn Pathol. 2010; 14:173–177.

14. Wagner AJ, Malinowska-Kolodziej I, Morgan JA, 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. Wu JH, Zhou JL, Cui Y, Jing QP, Shang L, Zhang JZ. Malignant perivascular epithelioid cell tumor of the retroperitoneum. Int J Clin Exp Pathol. 2013; 6:2251–2256.

16. Scheppach W, Reissmann N, Sprinz T, Schippers E, Schoettker B, Mueller JG. PEComa of the colon resistant to sirolimus but responsive to doxorubicin/ifosfamide. World J Gastroenterol. 2013; 19:1657–1660.

17. Koenig AM, Quaas A, Ries T, et al. Perivascular epitheloid cell tumour (PEComa) of the retroperitoneum - a rare tumor with uncertain malignant behaviour: a case report. J Med Case Rep. 2009; 3:62.

18. Gennatas C, Michalaki V, Kairi PV, Kondi-Paphiti A, Voros D. Successful treatment with the mTOR inhibitor everolimus in a patient with perivascular epithelioid cell tumor. World J Surg Oncol. 2012; 10:181.

Fig. 1.

Abdominal enhanced computer tomography shows multiple hypervascular nodules in the liver (A) and 9 cm sized heterogenous enhancing mass in left para-aortic space (B).

Fig. 2.

PET/CT scan shows ¹⁸ F-fludeoxyglucose hot uptakes in the liver (A) and left para-aortic space mass (B).

Fig. 3.

(A) The tumor shows nested epithelioid tumor cells with round nucleus and granular eosinophilic or clear cytoplasm (H&E, ×200). The tumor cells are positive for HMB-45 (B, ×200), but negative for smooth muscle actin (C, ×200) and S-100 (D, ×200).

Table 1.

Proposed Classification of Perivascular Epithelioid Cell Tumors by Folpe et al.^3,4

Risk features	Risk category
Size >5 cm	Benign; <2 high risk features and size <5 cm
Infiltrative growth pattern	Uncertain malignant potential; size >5 cm with no other high risk features
High nuclear grade and cellularity	or nuclear pleomorphism/multinucleated giant cells only
Mitotic rate >1/50 HPF	Malignant; 2 or more high risk features
Necrosis
Vascular invasion

Table 2.

Summary of Previously Reported Cases of Malignant Retroperitoneal Perivascular Epithelioid Cell Tumors

Author (year)	Age (yr)/sex	Presenting symptoms	Size (cm)	Location of tumor	Metastasis at initial diagnosis	Treatment	Outcome
Yokoo et al. (2000)⁸	38/F	Lumbar pain	30	Near the left kidney	Regional lymph node	Surgery, CTx, RT	Peritoneum metastasis after 1 year; dead after 4 years
Lau et al. (2003)⁹	29/M	Dyspepsia	20	Near the splenic flexure	None	Surgery	Liver metastases after 9 years; thymus and lung metastases after 17 years
Gupta et al. (2007)¹⁰	80/F	Abdominal pain	15	Abutting left kidney	Liver	Surgery	Bone metastasis after 1 year
Lans et al. (2009)¹¹	28/F	LLQ pain	15	Posterior to the left broad ligament	None	Surgery	NED at 1 year
Subbiah et al. (2010)¹²	58/F	RUQ mass	17	Above the right kidney	None	Surgery, adjuvant CTx	Liver metastasis after 2 years
de León et al. (2010)¹³	38/F	Abdominal pain	7	Near the renal hilum	None	Surgery	Dead after 18 months
Wagner et al. (2010)¹⁴	65/M	Not available	20	Not available	Peritoneal seeding	Surgery	Recur after 3 months
Wu et al. (2013)¹⁵	55/F	RLQ discomfort	7.5	Front of the right psoas muscle	None	Surgery	Liver metastasis after 7 months

CTx, chemotherapy; RT, radiation therapy; LLQ, left lower quadrant; RUQ, right upper quadrant; RLQ, right lower quadrant; NED, no evidence of disease.

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