Imaging features of primary and metastatic malignant perivascular epithelioid cell tumors

Sree Harsha Tirumani, Atul B. Shinagare, Jonathan B Hargreaves, Jyothi P. Jagannathan, Jason L. Hornick, Andrew J. Wagner, Nikhil H. Ramaiya

Research output: Contribution to journalArticle

28 Citations (Scopus)

Abstract

OBJECTIVE. The objective of our study was to describe the imaging features of primary and metastatic malignant perivascular epithelioid cell tumors (PEComas). MATERIALS AND METHODS. In this retrospective study, 36 patients (26 women, 10 men; mean age, 53.1 years; age range, 35-77 years) with a histopathologically confirmed diagnosis of malignant PEComa who were seen at our institute between January 2007 and December 2012 were included. Pretreatment imaging of the primary tumor in 17 patients (CT, n = 13; MRI, n = 9; ultrasound, n = 5) and follow-up imaging in all 36 patients (CT, n = 36; MRI, n = 7) were reviewed by three radiologists in consensus. RESULTS. The most common site of presentation of malignant PEComas was the retroperitoneum (38.9%) followed by the female genital tract (27.8%). Kidneys (8/36) and uterus (8/36) were the organs most frequently involved. The mean largest dimension was 11.01 cm (range, 4.5-25 cm). Primary tumors were well-circumscribed masses (16/17), were heteroechoic on ultrasound (5/5), were hypodense (2/6) to isodense (4/6) enhanced significantly (11/12) and compared with the paraspinal muscles on CT, were hypointense to isointense compared with skeletal muscle on T1-weighted imaging (8/9), were heterogeneously hyperintense compared with skeletal muscle on T2-weighted imaging (9/9), and showed significant post-gadolinium enhancement (7/7) on MRI. Of the 17 primary tumors, necrosis was seen in seven tumors, hemorrhage in three tumors, and calcification in three tumors. Twenty-six of 36 (72%) patients developed metastases, most commonly to the lung (21.6%), liver (17.6%), and peritoneum (10.8%). CONCLUSION. Malignant PEComas in our study were large tumors that most often arise in the kidneys and uterus and metastasize to lungs, liver, or peritoneum. In our experience malignant PEComas should be considered in the differential diagnosis of large well-circumscribed renal and uterine tumors.

Original languageEnglish (US)
Pages (from-to)252-258
Number of pages7
JournalAmerican Journal of Roentgenology
Volume202
Issue number2
DOIs
StatePublished - Feb 2014

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Perivascular Epithelioid Cell Neoplasms
Neoplasms
Peritoneum
Kidney
Uterus
Skeletal Muscle
Paraspinal Muscles
Lung
Liver
Gadolinium
Differential Diagnosis
Necrosis
Retrospective Studies
Hemorrhage
Neoplasm Metastasis

Keywords

  • CT
  • Malignant PEComa
  • Malignant perivascular epithelioid tumors
  • MRI
  • Ultrasound

ASJC Scopus subject areas

  • Radiology Nuclear Medicine and imaging

Cite this

Tirumani, S. H., Shinagare, A. B., Hargreaves, J. B., Jagannathan, J. P., Hornick, J. L., Wagner, A. J., & Ramaiya, N. H. (2014). Imaging features of primary and metastatic malignant perivascular epithelioid cell tumors. American Journal of Roentgenology, 202(2), 252-258. https://doi.org/10.2214/AJR.13.10909

Imaging features of primary and metastatic malignant perivascular epithelioid cell tumors. / Tirumani, Sree Harsha; Shinagare, Atul B.; Hargreaves, Jonathan B; Jagannathan, Jyothi P.; Hornick, Jason L.; Wagner, Andrew J.; Ramaiya, Nikhil H.

In: American Journal of Roentgenology, Vol. 202, No. 2, 02.2014, p. 252-258.

Research output: Contribution to journalArticle

Tirumani, SH, Shinagare, AB, Hargreaves, JB, Jagannathan, JP, Hornick, JL, Wagner, AJ & Ramaiya, NH 2014, 'Imaging features of primary and metastatic malignant perivascular epithelioid cell tumors', American Journal of Roentgenology, vol. 202, no. 2, pp. 252-258. https://doi.org/10.2214/AJR.13.10909
Tirumani, Sree Harsha ; Shinagare, Atul B. ; Hargreaves, Jonathan B ; Jagannathan, Jyothi P. ; Hornick, Jason L. ; Wagner, Andrew J. ; Ramaiya, Nikhil H. / Imaging features of primary and metastatic malignant perivascular epithelioid cell tumors. In: American Journal of Roentgenology. 2014 ; Vol. 202, No. 2. pp. 252-258.
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abstract = "OBJECTIVE. The objective of our study was to describe the imaging features of primary and metastatic malignant perivascular epithelioid cell tumors (PEComas). MATERIALS AND METHODS. In this retrospective study, 36 patients (26 women, 10 men; mean age, 53.1 years; age range, 35-77 years) with a histopathologically confirmed diagnosis of malignant PEComa who were seen at our institute between January 2007 and December 2012 were included. Pretreatment imaging of the primary tumor in 17 patients (CT, n = 13; MRI, n = 9; ultrasound, n = 5) and follow-up imaging in all 36 patients (CT, n = 36; MRI, n = 7) were reviewed by three radiologists in consensus. RESULTS. The most common site of presentation of malignant PEComas was the retroperitoneum (38.9{\%}) followed by the female genital tract (27.8{\%}). Kidneys (8/36) and uterus (8/36) were the organs most frequently involved. The mean largest dimension was 11.01 cm (range, 4.5-25 cm). Primary tumors were well-circumscribed masses (16/17), were heteroechoic on ultrasound (5/5), were hypodense (2/6) to isodense (4/6) enhanced significantly (11/12) and compared with the paraspinal muscles on CT, were hypointense to isointense compared with skeletal muscle on T1-weighted imaging (8/9), were heterogeneously hyperintense compared with skeletal muscle on T2-weighted imaging (9/9), and showed significant post-gadolinium enhancement (7/7) on MRI. Of the 17 primary tumors, necrosis was seen in seven tumors, hemorrhage in three tumors, and calcification in three tumors. Twenty-six of 36 (72{\%}) patients developed metastases, most commonly to the lung (21.6{\%}), liver (17.6{\%}), and peritoneum (10.8{\%}). CONCLUSION. Malignant PEComas in our study were large tumors that most often arise in the kidneys and uterus and metastasize to lungs, liver, or peritoneum. In our experience malignant PEComas should be considered in the differential diagnosis of large well-circumscribed renal and uterine tumors.",
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AU - Hornick, Jason L.

AU - Wagner, Andrew J.

AU - Ramaiya, Nikhil H.

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N2 - OBJECTIVE. The objective of our study was to describe the imaging features of primary and metastatic malignant perivascular epithelioid cell tumors (PEComas). MATERIALS AND METHODS. In this retrospective study, 36 patients (26 women, 10 men; mean age, 53.1 years; age range, 35-77 years) with a histopathologically confirmed diagnosis of malignant PEComa who were seen at our institute between January 2007 and December 2012 were included. Pretreatment imaging of the primary tumor in 17 patients (CT, n = 13; MRI, n = 9; ultrasound, n = 5) and follow-up imaging in all 36 patients (CT, n = 36; MRI, n = 7) were reviewed by three radiologists in consensus. RESULTS. The most common site of presentation of malignant PEComas was the retroperitoneum (38.9%) followed by the female genital tract (27.8%). Kidneys (8/36) and uterus (8/36) were the organs most frequently involved. The mean largest dimension was 11.01 cm (range, 4.5-25 cm). Primary tumors were well-circumscribed masses (16/17), were heteroechoic on ultrasound (5/5), were hypodense (2/6) to isodense (4/6) enhanced significantly (11/12) and compared with the paraspinal muscles on CT, were hypointense to isointense compared with skeletal muscle on T1-weighted imaging (8/9), were heterogeneously hyperintense compared with skeletal muscle on T2-weighted imaging (9/9), and showed significant post-gadolinium enhancement (7/7) on MRI. Of the 17 primary tumors, necrosis was seen in seven tumors, hemorrhage in three tumors, and calcification in three tumors. Twenty-six of 36 (72%) patients developed metastases, most commonly to the lung (21.6%), liver (17.6%), and peritoneum (10.8%). CONCLUSION. Malignant PEComas in our study were large tumors that most often arise in the kidneys and uterus and metastasize to lungs, liver, or peritoneum. In our experience malignant PEComas should be considered in the differential diagnosis of large well-circumscribed renal and uterine tumors.

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