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Abdominal perivascular epithelioid cell tumor (PEComa) without visible fat: a clinicopathologic and radiological analysis of 16 cases

  • ABDOMINAL RADIOLOGY
  • Published:
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Abstract

Objective

To retrospectively review the clinicopathological features and computed tomography (CT) and magnetic resonance imaging (MRI) findings of abdominal perivascular epithelioid cell tumor without visible fat (PEComawvf).

Materials and methods

Sixteen patients with surgically and pathologically confirmed perivascular epithelioid cell tumor without visible fat were enrolled. Their clinicopathological data and imaging findings were retrospectively reviewed. The CT and MRI features, including location, size, shape, margin, density, calcification, cystic necrosis and enhancement pattern, were analyzed.

Results

There were 4 males and 12 females (median age, 46 years; range, 21–65 years) in this study. All 16 patients were diagnostic asymptomatic unenhanced CT or MRI and revealed a well-defined (n = 13), oval (n = 10), mass with heterogeneous (n = 6) or homogeneous density/signal intensity (n = 7), calcification and hemorrhage was no found in any cases. On enhanced CT/MRI, markedly enhancement patterns (n = 14) were observed. The “peripheral enhancement” sign was observed in 13 cases. One in 16 cases recurrence was discovered during the follow-up period.

Conclusions

Dynamic CT, MRI and pathology of PEComawvf had some characteristics of non-aggressive pattern of performance, and MRI would provide beneficial detection of microscopic fat. Enhanced imaging showed PEComawvf is characterized by a “peripheral enhancement” with a marked enhancement pattern. Knowing these characteristics could contribute to improving the understanding abdominal PEComawvf and related palliative care.

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References

  1. Folpe AL, Fletcher CDM, Unni KK et al (2002) Neoplasms with perivascular epithelioid cell differentiation (PEComas). World Health Organization classification of tumours. Pathology and genetics of tumours of soft tissue and bone. IARC Press, Lyon, pp 221–222

  2. Thway K, Fisher C (2015) PEComa: morphology and genetics of a complex tumor family. Ann Diagn Pathol 19(5):359–368

    Article  Google Scholar 

  3. Baez JC, Landry JM, Saltzman JR, Qian X, Zinner MJ, Mortelé KJ (2009) Pancreatic PEComa (sugar tumor): MDCT and EUS Features. J Pancreas 6(10):679–682

    Google Scholar 

  4. Tirumani SH, Shinagare AB, Hargreaves J, Jagannathan JP, Hornick JL, Wagner AJ, Ramaiya NH (2014) Imaging features of primary and metastatic malignant perivascular epithelioid cell tumors. Am J Roentgenol 202(2):252–258

    Article  Google Scholar 

  5. Musella A, De Felice F, Kyriacou AK, Barletta F, Di Matteo FM, Marchetti C, Izzo L, Monti M, Panici PB, Redler A, D’Andrea V (2015) Perivascular epithelioid cell neoplasm (PEComa) of the uterus: a systematic review. Int J Surg 19:1–5

    Article  Google Scholar 

  6. Acosta AM, Adley BP (2017) Predicting the behavior of perivascular epithelioid cell tumors of the uterine corpus. Arch Pathol Lab Med 141:463–469

    Article  Google Scholar 

  7. Liu Z, Qi Y, Wang C, Zhang X, Wang B (2015) Hepatic perivascular epithelioid cell tumor: five case reports and literature review. Asian J Surg 1(38):58–63

    Article  Google Scholar 

  8. Chang Z-G, Zhang J-M, Ying J-Q, Ge Y-P (2011) Characteristics and treatment strategy of hepatic Angiomyolipoma: a series of 94 patients collected from four institutions. J Gastrointest Liver Dis 20(1):65–69

    Google Scholar 

  9. Yen C-N, Chen M-F, Hung C-F, Chen T-C, Chao T-C (2001) Angiomyolipoma of the liver. J Surg Oncol 77:195–200

    Article  Google Scholar 

  10. Tsui WMS, Colomban R, Portmann BC, Bonetti F, Thung SN, Ferrell LD, Nakanuma Y, Snover DC, Bioulac-Sage P, Dhillon AP (1999) Hepatic angiomyolipoma: a clinicopathologic study of 30 cases and delineation of unusual morphologic variants. Am J Surg Pathol 1(23):34–48

    Article  Google Scholar 

  11. Tan Y, Zhang H, Xiao E-H (2013) Perivascular epithelioid cell tumour: dynamic CT, MRI and clinicopathological characteristics—analysis of 32 cases and review of the literature. Clin Radiol 68(6):555–561

    Article  CAS  Google Scholar 

  12. Wei S, Henderson-Jackson E, Qian X, Bui MM (2017) Soft tissue tumor immunohistochemistry update illustrative examples of diagnostic pearls to avoid pitfalls. Arch Pathol Lab Med 141:387–405

    Article  Google Scholar 

  13. Utpatel K, Calvisi DF, Köhler G, Kühnel T, Niesel A, Verloh N, Vogelhuber M, Neu R, Hosten N, Schildhaus H-U, Dietmaier W, Evert M (2019) Complexity of PEComas: diagnostic approach, molecular background, clinical management. Der Pathologe. https://doi.org/10.1007/s00292-019-0612-5

    Article  PubMed  PubMed Central  Google Scholar 

  14. Ye HY, Chen JG, Luo DL, Jiang ZM, Chen ZH (2012) Perivascular epithelioid cell tumor(PEComa) of gynecologic origin: a clinicopathological study of three cases. Eur J Gynaecol Oncol 1(33):105–108

    Google Scholar 

  15. Fadare O (2008) Perivascular epithelioid cell tumor (PEComa) of the uterus: an outcome-based clinicopathologic analysis of 41 reported cases. Adv Anat Pathol 2(15):63–75

    Article  Google Scholar 

  16. Schoolmeester JK, Howitt BE, Hirsch MS, Dal Cin P, Quade BJ, Nucci MR (2014) Perivascular epithelioid cell neoplasm (pecoma) of the gynecologic tract: clinicopathologic and immunohistochemical characterization of 16 cases. Am J Surg Pathol 2(38):176–188

    Article  Google Scholar 

  17. Fang C-L, Lin Y-H, Chen Wei-Yu (2012) Microscopic endometrial perivascular epithelioid cell nodules: a case report with the earliest presentation of a uterine perivascular epithelioid cell tumor. Diagn Pathol 7(1):117

    Article  Google Scholar 

  18. Park SH, Ro JY, Kim HS, Lee ES (2003) Perivascular epithelioid cell tumor of the uterus: immunohistochemical, ultrastructural and molecular study. Pathol Int 53(11):800–805

    Article  Google Scholar 

  19. Takahashi N, Leng S, Kitajima K, Gomez-Cardona D, Thapa P, Carter RE, Leibovich BC, Sasiwimonphan K, Sasaguri K, Kawashima A (2015) Small (< 4 cm) renal masses: differentiation of angiomyolipoma without visible fat from renal cell carcinoma using unenhanced and contrast-enhanced CT. AJR Am J Roentgenol 205(6):1194–1202

    Article  Google Scholar 

  20. Liu Z, Qi Y, Wang C, Zhang X, Wang B (2015) Hepatic perivascular epithelioid cell tumor: five case reports and literature review. Asian J Surg 38(1):58–63

    Article  Google Scholar 

  21. Zhao LJ, Yang YJ, Wu H, Huang SM, Liu K (2013) Perivascular epithelioid cell tumor of the liver: a case report and literature review. Eur Rev Med Pharmacol 17(12):1665–1668

    Google Scholar 

  22. Tan Y, Xiao E-h (2012) Hepatic perivascular epithelioid cell tumor (PEComa): dynamic CT, MRI, ultrasonography, and pathologic features—analysis of 7 cases and review of the literature. Abdom Radiol 37(5):781–787

    Article  Google Scholar 

  23. Yan F, Zeng M, Zhou K, Shi W, Zheng W, Da R, Fan J, Ji Y (2002) Hepatic angiomyolipoma: various appearances on two-phase contrast scanning of spiral CT. Eur J Radiol 41(1):12–18

    Article  Google Scholar 

  24. Ding H, Wei H, Liu H, Chen Y, Xue X, Weng H (2017) The histopathological features and CT/MRI imaging performances in hepatic angiomyolipoma patients. Ann Hepatol 16(5):759–764

    Article  Google Scholar 

  25. Serdar S, Senturk E, Kuman N, Pabuscu E, Kacar F (2009) PEComa (clear cell “sugar” tumor) of the lung: a benign tumor that presented with thrombocytosis. Ann Thorac Surg 88(6):2013–2015

    Article  Google Scholar 

  26. Catherineh P, Keraliya A, Shinagare A, Ramaiya N, Tirumani S (2016) Update on the imaging of malignant perivascular epithelioid cell tumors (PEComas). Abdom Radiol 41(2):368–376

    Article  Google Scholar 

  27. Annej K, Verver D, Janki S, Bramer W, Doukas M, Dwarkasing R, de Man R, IJzermans J (2017) Management of hepatic angiomyolipoma: a systematic review. Liver Int 37(9):1272–1280

    Article  Google Scholar 

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Authors and Affiliations

  1. Department of Radiology, Xiangya Hospital, Central South University, Changsha, 410011, Hunan, China

    Jiaxi Hu, Wenguang Liu, Simin Xie, Mengsi Li & Wenzheng Li

  2. Department of Gastrointestinal Surgery, Xiangya Hospital, Central South University, Changsha, 410011, Hunan, China

    Kangtao Wang

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  1. Jiaxi Hu
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Correspondence to Wenzheng Li.

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Hu, J., Liu, W., Xie, S. et al. Abdominal perivascular epithelioid cell tumor (PEComa) without visible fat: a clinicopathologic and radiological analysis of 16 cases. Radiol med 126, 189–199 (2021). https://doi.org/10.1007/s11547-020-01241-8

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  • DOI: https://doi.org/10.1007/s11547-020-01241-8

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