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Published in: Diagnostic Pathology 1/2015

Open Access 01-12-2015 | Case Report

Malignant perivascular epithelioid cell tumor (PEComa) of the femur: a case report and literature review

Authors: I Weng Lao, Lin Yu, Jian Wang

Published in: Diagnostic Pathology | Issue 1/2015

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Abstract

Background

We describe a case of malignant perivascular epithelial cell tumor (PEComa) arising primarily in the distal left femur of a 47-year-old male.

Case presentation

The patient presented with pain accompanied by progressive swelling of his left thigh. Computed tomography (CT) scan and magnetic resonance imaging (MRI) revealed an osteolytic lesion. Curettage of the lesion was reported as a clear cell tumor with recommendation for exclusion of a metastatic clear cell carcinoma. However, thorough examinations did not find any primary site elsewhere, apart from the presence of bilateral pulmonary metastases. Evaluation of the submitted H & E slides identified a malignant PEComa which was further confirmed by subsequent immunohistochemical study.

Conclusions

The occurrence of PEComa as a primary bone lesion is extremely rare. We present here a malignant PEComa of the distal left femur, and summarize the clinicopathological characteristics of this rare entity with literature review.

Virtual slides

The virtual slide (s) for this article can be found here: http://​www.​diagnosticpathol​ogy.​diagnomx.​eu/​vs/​5729035221600545​.
Literature
1.
go back to reference Folpe AL, Mentzel T, Lehr HA, Fisher C, Balzer BL, Weiss SW. Perivascular epithelioid cell neoplasms of soft tissue and gynecologic origin: a clinicopathologicstudy of 26 cases and review of the literature. Am J Surg Pathol. 2005;29(12):1558–75.PubMed Folpe AL, Mentzel T, Lehr HA, Fisher C, Balzer BL, Weiss SW. Perivascular epithelioid cell neoplasms of soft tissue and gynecologic origin: a clinicopathologicstudy of 26 cases and review of the literature. Am J Surg Pathol. 2005;29(12):1558–75.PubMed
2.
go back to reference Insabato L, De Rosa G, Terracciano LM, Fazioli F, Di Santo F, Rosai J. Primary monotypic epithelioid angiomyolipoma of bone. Histopathology. 2002;40(3):286–90.CrossRefPubMed Insabato L, De Rosa G, Terracciano LM, Fazioli F, Di Santo F, Rosai J. Primary monotypic epithelioid angiomyolipoma of bone. Histopathology. 2002;40(3):286–90.CrossRefPubMed
3.
go back to reference Lian DW, Chuah KL, Cheng MH, Yap WM. Malignant perivascular epithelioid cell tumour of the fibula: a report and a short review of bone perivascular epithelioid cell tumour. J Clin Pathol. 2008;61(10):127–1129. Lian DW, Chuah KL, Cheng MH, Yap WM. Malignant perivascular epithelioid cell tumour of the fibula: a report and a short review of bone perivascular epithelioid cell tumour. J Clin Pathol. 2008;61(10):127–1129.
4.
go back to reference Righi A, Dimosthenous K, Rosai J. PEComa: another member of the MiT tumor family? Int J Surg Pathol. 2008;16(1):16–20.PubMed Righi A, Dimosthenous K, Rosai J. PEComa: another member of the MiT tumor family? Int J Surg Pathol. 2008;16(1):16–20.PubMed
5.
go back to reference Torii I, Kondo N, Takuwa T, Matsumoto S, Okumura Y, Sato A, Tanaka F, Nishigami T, Hasegawa S, Tsujimura T. Perivascular epithelioid cell tumor of the rib. Virchows Arch. 2008;452(2):697–702. Torii I, Kondo N, Takuwa T, Matsumoto S, Okumura Y, Sato A, Tanaka F, Nishigami T, Hasegawa S, Tsujimura T. Perivascular epithelioid cell tumor of the rib. Virchows Arch. 2008;452(2):697–702.
6.
go back to reference Yamashita K, Fletcher CD. PEComa presenting in bone: clinicopathologic analysis of 6 cases and literature review. Am J Surg Pathol. 2010;34(11):1622–9.PubMed Yamashita K, Fletcher CD. PEComa presenting in bone: clinicopathologic analysis of 6 cases and literature review. Am J Surg Pathol. 2010;34(11):1622–9.PubMed
7.
go back to reference Kazzaz D, Khalifa M, Alorjan M, Shaw M, Rezajooi K, Saifuddin A. Malignant PEComa of the lumbar vertebra: a rare bone tumour. Skeletal Radiol. 2012;41(11):1465–8.PubMed Kazzaz D, Khalifa M, Alorjan M, Shaw M, Rezajooi K, Saifuddin A. Malignant PEComa of the lumbar vertebra: a rare bone tumour. Skeletal Radiol. 2012;41(11):1465–8.PubMed
8.
go back to reference Desy NM, Bernstein M, Nahal A, Aziz M, Kenan S, Turcotte RE, et al. Primary perivascular epithelioid cell neoplasm (PEComa) of bone: report of two cases and review of the literature. Skeletal Radiol. 2012;41(11):1469–74.PubMed Desy NM, Bernstein M, Nahal A, Aziz M, Kenan S, Turcotte RE, et al. Primary perivascular epithelioid cell neoplasm (PEComa) of bone: report of two cases and review of the literature. Skeletal Radiol. 2012;41(11):1469–74.PubMed
9.
go back to reference Rakheja R, Abikhzer G, Alabed YZ, Nahal A, Lisbona R. The appearance of osseous PEComa on F-18 FDG PET/CT. Clin Nucl Med. 2012;37(2):190–2.PubMed Rakheja R, Abikhzer G, Alabed YZ, Nahal A, Lisbona R. The appearance of osseous PEComa on F-18 FDG PET/CT. Clin Nucl Med. 2012;37(2):190–2.PubMed
10.
go back to reference Young LR, Franz DN, Nagarkatte P, Fletcher CD, Wikenheiser-Brokamp KA, Galsky MD, et al. Utility of _18F_2-fluoro-2- deoxyglucose-PET in sporadic and tuberous sclerosis-associated lymphangioleiomyomatosis. Chest. 2009;136(3):926–33.PubMedCentralPubMed Young LR, Franz DN, Nagarkatte P, Fletcher CD, Wikenheiser-Brokamp KA, Galsky MD, et al. Utility of _18F_2-fluoro-2- deoxyglucose-PET in sporadic and tuberous sclerosis-associated lymphangioleiomyomatosis. Chest. 2009;136(3):926–33.PubMedCentralPubMed
11.
go back to reference Zarbis N, Barth TF, Blumstein NM, Schelzig H. Pecoma of the lung: a benign tumor with extensive 18 F-2-deoxy-D-glucose uptake. Interact Cardiovasc Thorac Surg. 2007;6(5):676–8.PubMed Zarbis N, Barth TF, Blumstein NM, Schelzig H. Pecoma of the lung: a benign tumor with extensive 18 F-2-deoxy-D-glucose uptake. Interact Cardiovasc Thorac Surg. 2007;6(5):676–8.PubMed
12.
go back to reference Pang LJ, Chang B, Zou H, Qi Y, Jiang JF, Li HA, Hu WH, Chen YZ, Liu CX, Zhang WJ, Li F.Alveolar soft part sarcoma: a bimarker diagnostic strategy using TFE3 immunoassay and ASPL-TFE3 fusion transcripts in paraffin-embedded tumor tissues. Diagn Mol Pathol. 2008;17(4):245-52. Pang LJ, Chang B, Zou H, Qi Y, Jiang JF, Li HA, Hu WH, Chen YZ, Liu CX, Zhang WJ, Li F.Alveolar soft part sarcoma: a bimarker diagnostic strategy using TFE3 immunoassay and ASPL-TFE3 fusion transcripts in paraffin-embedded tumor tissues. Diagn Mol Pathol. 2008;17(4):245-52.
13.
go back to reference 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(9):1361–71.PubMedCentralPubMed 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(9):1361–71.PubMedCentralPubMed
14.
go back to reference 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(5):835–40.PubMed 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(5):835–40.PubMed
Metadata
Title
Malignant perivascular epithelioid cell tumor (PEComa) of the femur: a case report and literature review
Authors
I Weng Lao
Lin Yu
Jian Wang
Publication date
01-12-2015
Publisher
BioMed Central
Published in
Diagnostic Pathology / Issue 1/2015
Electronic ISSN: 1746-1596
DOI
https://doi.org/10.1186/s13000-015-0292-2

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