Dataset Information

Imaging Findings and Clinical Features of Primary Spinal Epithelioid Hemangioendothelioma.

ABSTRACT:

Rationale and objectives

Primary spinal epithelioid hemangioendothelioma (SEHE) is an extremely rare angiocentric vascular neoplasm with inherent metastatic potential, which pursues a clinical course intermediate between hemangioma and angiosarcoma. The present study sought to present computed tomography (CT) and magnetic resonance imaging (MRI) findings and clinical features of primary SEHE and review the literature.

Materials and methods

Clinical data of four patients with primary SEHE confirmed by pathology from January 2011 to May 2020 were analyzed retrospectively. Two cases underwent CT scan, while 3 cases underwent MRI scan. Image observation included the tumor location, bone changes, morphology, density/signal characteristics, and enhanced features.

Results

The four patients were all women who ranged in age from 25 to 74 years. Four cases of the lesions were in the vertebral body; among them, two cases involved the accessory of the vertebra. Soap bubble appearance was detected in 2 cases, honeycomb osteolytic appearance in 2 cases, a sclerotic margin in 3 cases, and accompanying vertebral compression fracture in 1 case. CT showed a slightly inhomogeneous low density with punctate high-density foci. MRI showed an inhomogeneous low signal on T₁-weighted image (T₁WI) and a high signal on T₂WI. Contrast-enhanced CT/MRI demonstrated moderate to intensive inhomogeneous enhancement of the lesions. The discs were normal. In one case, lesions presented a dural tail sign.

Conclusions

Primary SEHE is so rare in the clinic as an aggressive vascular tumor. The relatively typical clinical features and radiographic findings can help in preoperative diagnosis.

SUBMITTER: Liang P

PROVIDER: S-EPMC9190255 | biostudies-literature | 2022

REPOSITORIES: biostudies-literature

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Publications

Imaging Findings and Clinical Features of Primary Spinal Epithelioid Hemangioendothelioma.

Liang Pan P Zhu Bing-Bing BB Ren Xiu-Chun XC Gao Jian-Bo JB

Frontiers in oncology 20220530

<h4>Rationale and objectives</h4>Primary spinal epithelioid hemangioendothelioma (SEHE) is an extremely rare angiocentric vascular neoplasm with inherent metastatic potential, which pursues a clinical course intermediate between hemangioma and angiosarcoma. The present study sought to present computed tomography (CT) and magnetic resonance imaging (MRI) findings and clinical features of primary SEHE and review the literature.<h4>Materials and methods</h4>Clinical data of four patients with prima ...[more]

PMID: 35707348

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Project description:Epithelioid hemangioendothelioma is a low-grade malignant vascular tumor with an intermediate clinical behavior between benign hemangiomas and high-grade angiosarcomas. Pathologic or molecular factors to predict this clinical heterogeneity are not well defined. A WWTR1-CAMTA1 fusion is present in most classic epithelioid hemangioendothelioma, regardless of their clinical behavior, suggesting that additional genetic abnormalities might be responsible in driving a more aggressive biology. A small subset of cases show distinct morphology and are characterized genetically by a YAP1-TFE3 fusion. Two histologic grades have been described in classic epithelioid hemangioendothelioma of the soft tissue. However, proposed criteria do not apply to other clinical presentations and have not been assessed in the YAP1-TFE3 positive tumors. Furthermore, no previous studies have compared the survival of these two molecular subsets. In this study we investigate the clinicopathologic and molecular findings of a large cohort of 93 translocation-positive epithelioid hemangioendothelioma managed at our institution. Patient characteristics, histologic features, treatment outcomes, and genetic abnormalities were investigated and these factors were correlated with overall survival. In 18 patients (15 with WWTR1-CAMTA1 and 3 with YAP1-TFE3) Memorial Sloan Kettering-IMPACT targeted DNA sequencing was performed to identify secondary genetic alterations showing more than half of tumors had a genetic alteration beyond the disease-defining gene fusion. Patients with conventional epithelioid hemangioendothelioma with WWTR1-CAMTA1 fusion had a less favorable outcome compared with the YAP1-TFE3 subset, the 5-year overall survival being 59% versus 86%, respectively. Soft tissue epithelioid hemangioendothelioma were frequently solitary, followed an uneventful clinical course being often managed with curative surgery. Multifocality, pleural involvement, lymph node or distant metastases had a significantly worse outcome. Patients with pleural disease or lymph node metastases had an aggressive clinical course akin to high-grade sarcomas, with 22% and 30%, respectively, alive at 5 years, compared with >70% survival rate in patients lacking these two adverse factors.

Dataset Information

Imaging Findings and Clinical Features of Primary Spinal Epithelioid Hemangioendothelioma.

Rationale and objectives

Materials and methods

Results

Conclusions

Publications

Imaging Findings and Clinical Features of Primary Spinal Epithelioid Hemangioendothelioma.

Similar Datasets

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