Primary hepatic perivascular epithelioid cell tumors: imaging findings with histopathological correlation
Abstract Background Hepatic PEComas are very rare. Few systematic reports are available characterizing the imaging and pathological features of hepatic PEComa. The aim of this study was to investigate the imaging findings of primary hepatic perivascular epithelioid cell tumors (PEComa) and its corre...
| Main Authors: | , , , , , , |
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| Format: | Article |
| Language: | English |
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BMC
2019-06-01
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| Series: | Cancer Imaging |
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| Online Access: | http://link.springer.com/article/10.1186/s40644-019-0212-x |
| _version_ | 1818968503457153024 |
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| author | Pei Nie Jie Wu Hexiang Wang Ruizhi Zhou Lingling Sun Jingjing Chen Guangjie Yang |
| author_facet | Pei Nie Jie Wu Hexiang Wang Ruizhi Zhou Lingling Sun Jingjing Chen Guangjie Yang |
| author_sort | Pei Nie |
| collection | DOAJ |
| description | Abstract Background Hepatic PEComas are very rare. Few systematic reports are available characterizing the imaging and pathological features of hepatic PEComa. The aim of this study was to investigate the imaging findings of primary hepatic perivascular epithelioid cell tumors (PEComa) and its correlation with histopathological features. Methods The CT, MRI and ultrasound images and pathological findings of 22 patients with primary hepatic PEComa were retrospectively reviewed. Results More females (14/22) were affected with the mean age of 47.1 years. Most patients (17/22) were asymptomatic and the routine laboratory tests were normal. More tumors occurred in the right lobe (13/22) with a mean diameter of 76.7 mm. Surgery was performed in 21 patients, and biopsy was performed in 1 patient. Immunohistochemical studies showed the expression rate of HMB-45 and Melan A was 100% (22/22) and 86.4% (19/22) within the tumor cells. The pathology diagnoses were angiomyolipoma (n = 18), lymphangioleiomyoma (n = 2), clear-cell myomelanocytic tumor of falciform ligament/ligamentum teres (n = 1), and not otherwise specified (n = 1). Fifteen cases were classified as uncertain malignant potential (n = 13) or malignant (n = 2). CT, MRI and ultrasound features included well-defined margins (19/22), internal heterogeneity (20/22), arterial enhancement (20/22), dysmorphic vessels (17/22), fat (9/22), hemorrhage (3/22), necrosis (8/22), and calcification (2/22). The diagnostic accuracy was only 27.3% (6/22). No local recurrence or metastasis was found in the follow-up patients (12/22). Conclusions On CT, MRI and ultrasound images, most hepatic PEComas are well-defined, heterogeneous, arterial enhanced masses with dysmorphic vessels, with or without fat, especially in middle-aged females. With the potential to be malignant, timely surgical resection and long-term follow-up may be helpful for improving the prognosis. |
| first_indexed | 2024-12-20T14:05:44Z |
| format | Article |
| id | doaj.art-c386d3b837c443d09629e1c42746965a |
| institution | Directory Open Access Journal |
| issn | 1470-7330 |
| language | English |
| last_indexed | 2024-12-20T14:05:44Z |
| publishDate | 2019-06-01 |
| publisher | BMC |
| record_format | Article |
| series | Cancer Imaging |
| spelling | doaj.art-c386d3b837c443d09629e1c42746965a2022-12-21T19:38:16ZengBMCCancer Imaging1470-73302019-06-011911910.1186/s40644-019-0212-xPrimary hepatic perivascular epithelioid cell tumors: imaging findings with histopathological correlationPei Nie0Jie Wu1Hexiang Wang2Ruizhi Zhou3Lingling Sun4Jingjing Chen5Guangjie Yang6Department of Radiology, the Affiliated Hospital of Qingdao UniversityDepartment of Pathology, the Affiliated Hospital of Qingdao UniversityDepartment of Radiology, the Affiliated Hospital of Qingdao UniversityDepartment of Radiology, the Affiliated Hospital of Qingdao UniversityDepartment of Pathology, the Affiliated Hospital of Qingdao UniversityDepartment of Radiology, the Affiliated Hospital of Qingdao UniversityPET-CT Center, the Affiliated Hospital of Qingdao UniversityAbstract Background Hepatic PEComas are very rare. Few systematic reports are available characterizing the imaging and pathological features of hepatic PEComa. The aim of this study was to investigate the imaging findings of primary hepatic perivascular epithelioid cell tumors (PEComa) and its correlation with histopathological features. Methods The CT, MRI and ultrasound images and pathological findings of 22 patients with primary hepatic PEComa were retrospectively reviewed. Results More females (14/22) were affected with the mean age of 47.1 years. Most patients (17/22) were asymptomatic and the routine laboratory tests were normal. More tumors occurred in the right lobe (13/22) with a mean diameter of 76.7 mm. Surgery was performed in 21 patients, and biopsy was performed in 1 patient. Immunohistochemical studies showed the expression rate of HMB-45 and Melan A was 100% (22/22) and 86.4% (19/22) within the tumor cells. The pathology diagnoses were angiomyolipoma (n = 18), lymphangioleiomyoma (n = 2), clear-cell myomelanocytic tumor of falciform ligament/ligamentum teres (n = 1), and not otherwise specified (n = 1). Fifteen cases were classified as uncertain malignant potential (n = 13) or malignant (n = 2). CT, MRI and ultrasound features included well-defined margins (19/22), internal heterogeneity (20/22), arterial enhancement (20/22), dysmorphic vessels (17/22), fat (9/22), hemorrhage (3/22), necrosis (8/22), and calcification (2/22). The diagnostic accuracy was only 27.3% (6/22). No local recurrence or metastasis was found in the follow-up patients (12/22). Conclusions On CT, MRI and ultrasound images, most hepatic PEComas are well-defined, heterogeneous, arterial enhanced masses with dysmorphic vessels, with or without fat, especially in middle-aged females. With the potential to be malignant, timely surgical resection and long-term follow-up may be helpful for improving the prognosis.http://link.springer.com/article/10.1186/s40644-019-0212-xPerivascular epithelioid cell tumorsLiverCTMRIUltrasoundPathology |
| spellingShingle | Pei Nie Jie Wu Hexiang Wang Ruizhi Zhou Lingling Sun Jingjing Chen Guangjie Yang Primary hepatic perivascular epithelioid cell tumors: imaging findings with histopathological correlation Cancer Imaging Perivascular epithelioid cell tumors Liver CT MRI Ultrasound Pathology |
| title | Primary hepatic perivascular epithelioid cell tumors: imaging findings with histopathological correlation |
| title_full | Primary hepatic perivascular epithelioid cell tumors: imaging findings with histopathological correlation |
| title_fullStr | Primary hepatic perivascular epithelioid cell tumors: imaging findings with histopathological correlation |
| title_full_unstemmed | Primary hepatic perivascular epithelioid cell tumors: imaging findings with histopathological correlation |
| title_short | Primary hepatic perivascular epithelioid cell tumors: imaging findings with histopathological correlation |
| title_sort | primary hepatic perivascular epithelioid cell tumors imaging findings with histopathological correlation |
| topic | Perivascular epithelioid cell tumors Liver CT MRI Ultrasound Pathology |
| url | http://link.springer.com/article/10.1186/s40644-019-0212-x |
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