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 language | English (US) |
|---|---|
| Pages (from-to) | 252-258 |
| Number of pages | 7 |
| Journal | American Journal of Roentgenology |
| Volume | 202 |
| Issue number | 2 |
| DOIs | |
| State | Published - Feb 2014 |
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Keywords
- CT
- Malignant PEComa
- Malignant perivascular epithelioid tumors
- MRI
- Ultrasound
ASJC Scopus subject areas
- Radiology Nuclear Medicine and imaging
Cite this
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 journal › Article
}
TY - JOUR
T1 - Imaging features of primary and metastatic malignant perivascular epithelioid cell tumors
AU - Tirumani, Sree Harsha
AU - Shinagare, Atul B.
AU - Hargreaves, Jonathan B
AU - Jagannathan, Jyothi P.
AU - Hornick, Jason L.
AU - Wagner, Andrew J.
AU - Ramaiya, Nikhil H.
PY - 2014/2
Y1 - 2014/2
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.
AB - 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.
KW - CT
KW - Malignant PEComa
KW - Malignant perivascular epithelioid tumors
KW - MRI
KW - Ultrasound
UR - http://www.scopus.com/inward/record.url?scp=84892743914&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=84892743914&partnerID=8YFLogxK
U2 - 10.2214/AJR.13.10909
DO - 10.2214/AJR.13.10909
M3 - Article
C2 - 24450662
AN - SCOPUS:84892743914
VL - 202
SP - 252
EP - 258
JO - American Journal of Roentgenology
JF - American Journal of Roentgenology
SN - 0361-803X
IS - 2
ER -