Hepatic epithelioid hemangioendothelioma with metastasis to the mesentery of the small intestine: A case report

Nakamura, Daichi; Adachi, Yasushi; Nakashima, Yasuaki; Sakaida, Noriko; Narahara, Hiroyuki; Shi, Ming; Tsubono, Michihiko; Ikehara, Susumu

doi:10.3892/mco.2017.1402

Cited by 3 publications

(4 citation statements)

References 16 publications

(20 reference statements)

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“…One case in our series metastasized to the omentum and colon. Nakamura et al published a case of hepatic EHE which metastasized to the mesentery of the small intestine [ 24 ]. Shah et al reported a case of multifocal hepatic EHE developing in the background of Budd- Chiari syndrome with secondary cirrhosis.…”

Section: Discussionmentioning

confidence: 99%

Hepatic epithelioid hemangioendothelioma: case series of a rare vascular tumor mimicking metastases

Din¹,

Rahim²,

Asghari³

et al. 2020

Diagn Pathol

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Background Hepatic epithelioid hemangioendothelioma is an extremely rare malignant vascular tumor which is often multifocal and, in many cases, discovered incidentally. Here, we describe the clinicopathological features of hepatic epithelioid hemangioendothelioma cases seen in our practice and present a detailed review of the published literature. Methods All cases of hepatic epithelioid hemangioendothelioma diagnosed in Department of Pathology and Laboratory Medicine, Aga Khan University Hospital between January 1, 2006 and December 31, 2019 were included in the study. Slides were reviewed and follow up was obtained. Results Seven cases were reported during the study period. There were 4 females and 3 males. Age range was 20 to 77 years, mean age was 45 years. Three patients presented with right upper abdominal pain; 1 patient presented with jaundice while 3 patients were asymptomatic. In all 7 cases, lesions were identified on imaging studies. In 5 cases, liver lesions were multifocal. Clinical differential diagnosis in all cases was metastatic carcinoma and multifocal hepatocellular carcinoma. Liver function tests were normal in 5 cases. In 1 patient, tumor had already metastasized to the right lung. On histological examination of liver core biopsies performed in all 7 cases, classic histological features of epithelioid hemangioendothelioma were seen. Tumor cells expressed positivity for vascular markers (CD 34, CD31 and ERG) and were negative for cytokeratins, Hep par 1 and Glypican 3. Surgical resection was not performed in any of the 7 cases and all patients were treated by chemotherapy. Follow up was available in 5 cases. Of these, 3 patients died of disease and another patient was alive with metastases in both lungs, omentum and colon. Conclusion Clinicopathological features of the 7 cases in our series and detailed review of published literature is presented. Prognosis was bad in our cases most likely due to fact that surgical resection could not be performed in any of the cases owing to lack of surgical expertise for liver tumor surgery in most parts of the country.

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Section: Discussionmentioning

confidence: 99%

Hepatic epithelioid hemangioendothelioma: case series of a rare vascular tumor mimicking metastases

Din¹,

Rahim²,

Asghari³

et al. 2020

Diagn Pathol

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“…PHMVT is prone to pulmonary metastasis and is easily misdiagnosed clinically [6]. To date, only a few cases of PHMVT have been described worldwide [1][2][3][4][5][6][7][8][9][10]. Thus, we designed the present study to explore the CT manifestations of PHMVT to improve the understanding of this disease.…”

Section: Discussionmentioning

confidence: 99%

“…The solid components are significantly enhanced in the arterial phase. EHE, MHP and IHE present with heterogeneous and persistent enhancement without filling, while PHA shows persistent and complete filling in the portal venous phase [8][9][10]. Imaging findings should be differentiated from hepatic hemangioma, hepatocellular carcinoma and hepatoblastoma [13][14][15][16][17][18][19][20].…”

Section: Discussionmentioning

confidence: 99%

“…Certainly, different pathological types are also associated with different imaging features. Few systematic reports have characterized the imaging features of PHMVT according to pathological type [ 8 – 10 ]. Furthermore, previous case reports and literature reviews have not systematically summarized imaging manifestations and clinical features.…”

Section: Introductionmentioning

confidence: 99%

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Primary hepatic malignant vascular tumors: a follow-up study of imaging characteristics and clinicopathological features

et al. 2020

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Background: Owing to its low incidence, there is insufficient clinical awareness and diagnostic experience with primary hepatic malignant vascular tumors (PHMVTs). The aim of our study was to investigate the imaging and clinicopathological features of patients with PHMVTs and analyze the clinicopathological correlations. Methods: We retrospectively analyzed 42 patients who had pathologically confirmed PHMVT during the period from June 2012 to December 2019 and enrolled them in our study. The computed tomography (CT) and magnetic resonance (MR) images and pathological findings of each patient were recorded. Results: There were more female (29/42) than male patients. The imaging features of primary hepatic angiosarcoma (PHA) (n = 11) included ill-defined margins (11/11, 100%), necrosis (5/11, 45%), calcification (3/11, 27%) and "slow inslow out" centripetal enhancement (7/11, 64%). Patients with epithelioid hemangioendothelioma (EHE) (n = 15) presented with ill-defined margins (15/15, 100%), necrosis (6/15, 40%), calcification (2/15, 13%), "fast in-slow out" centripetal enhancement (10/15, 67%), halo sign (15/15, 100%), pseudocapsule sign (4/15, 27%), lollipop sign (2/15, 13%) and capsule retraction sign (2/15, 13%). Patients with malignant hemangiopericytoma (MHP) (n = 3) showed illdefined margins (3/3, 100%), necrosis (3/3, 100%) and "fast in-slow out" progressive enhancement (3/3, 100%). Infantile hemangioendotheliomas (IHEs) (n = 13) were defined by ill-defined margins (7/13, 54%), necrosis (8/13, 62%), calcification (5/13, 38%) and "fast in-slow out" centripetal enhancement (13/13, 100%). Immunohistochemistry showed strong positive expression of CD31, CD34, ERG, FaVIII and FLI-1. Patients with IHE (96 months) and EHE (88 months) had the longest survival times, followed by those with MHP (23 months), while patients with PHA (15 months) had the shortest survival time. Conclusion: On CT and MR images, most PHMVTs were ill-defined, heterogeneous, hypervascular masses with centripetal progressive enhancement and possibly calcification, especially in female patients. The prognosis of patients with PHMVT was associated with the pathological type of the tumor.

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Shortness of Breath in a 38-Year-Old Woman With Pulmonary and Hepatic Nodules

Ido

Purdy

Harrison

et al. 2021

Chest

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Hepatic epithelioid hemangioendothelioma with metastasis to the mesentery of the small intestine: A case report

Cited by 3 publications

References 16 publications

Hepatic epithelioid hemangioendothelioma: case series of a rare vascular tumor mimicking metastases

Hepatic epithelioid hemangioendothelioma: case series of a rare vascular tumor mimicking metastases

Primary hepatic malignant vascular tumors: a follow-up study of imaging characteristics and clinicopathological features

Shortness of Breath in a 38-Year-Old Woman With Pulmonary and Hepatic Nodules

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