Clinical characteristics of epithelioid hemangioendothelioma: a single-center retrospective study

Wu, Xin; Li, Binglu; Zheng, Congyi; Hong, Tao; He, Xiaodong

doi:10.1186/s40001-019-0375-8

Cited by 28 publications

(30 citation statements)

References 27 publications

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“…Although EHE is often reported to be heterogeneous [7,24], the present study is the first to demonstrate this diversity by identification of three symptom clusters. Previous data are limited to prevalence of symptoms rather than examining the symptom burden of individual patients [2,3,25]. In the present study, 87% of patients reported one or more symptoms, compared to 45-72% symptomatic EHE patients in previous studies [2,3,25].…”

Section: Discussioncontrasting

confidence: 51%

Health-related quality of life and symptom burden of epithelioid hemangioendothelioma patients: a global patient-driven Facebook study in a very rare malignancy

et al. 2020

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Purpose: Epithelioid hemangioendothelioma (EHE) is an ultra-rare vascular sarcoma with unique clinical features. EHE is characterized by an unpredictable, often protracted, clinical course and highly variable clinical presentation. Due to difficulty recruiting ultra-rare cancer patients, health-related quality of life (HRQoL) of EHE patients has not yet been studied. The aim of this study was to assess EHE symptom burden and its impact on HRQoL and psychological distress. Methods: The study was initiated after EHE patients' foundations approached our research group to study HRQoL. Patients were recruited from the international EHE Facebook group from May through October 2018. Data were collected using the online PROFILES registry. Latent class cluster analysis was performed to identify groups based on frequently reported symptoms. Differences in HRQoL (EORTC-QLQ-C30) and psychological distress (Hospital Anxiety and Depression Scale) between symptom-based clusters were examined. Results: Among 115 EHE patients from 20 countries, three clusters were identified, with low-, intermediate-and high-symptom burden, respectively. Highly symptomatic patients (33%) had clinically relevantly lower scores on HRQoL compared to the other two groups (p < 0.001). These patients suffered mostly from pain, insomnia and fatigue. Symptom burden significantly correlated with reduced daily functioning and high levels of psychological distress. Only for highly symptomatic patients, HRQoL and symptom levels were worse compared to healthy individuals. Conclusion: For the first time, we studied HRQoL in a large international cohort of ultra-rare cancer patients with distinct clinical characteristics, enabled by collaboration with patients and use of social media. We showed a considerable number of EHE patients were highly symptomatic, with a significant impact on HRQoL and psychological distress.

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Section: Discussioncontrasting

confidence: 51%

Health-related quality of life and symptom burden of epithelioid hemangioendothelioma patients: a global patient-driven Facebook study in a very rare malignancy

et al. 2020

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“…Surgery is the treatment of choice in local disease. There is a risk of metastatic disease (synchronic or metachronic) and a significant mortality rate (up to 45%) [58][59][60].…”

Section: Epithelioid Hemangioendothelioma (Ehe)mentioning

confidence: 99%

Soft Tissue Special Issue: Perivascular and Vascular Tumors of the Head and Neck

Flucke

Karanian

Broek

et al. 2020

Head and Neck Pathol

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Perivascular and vascular neoplasms of the head and neck are a rare group of tumors comprising a spectrum of clinical/ biologic and histological features. They are frequently diagnostically challenging, due to their morphologic and immunohistochemical overlap. In this review, we summarize the pathology of these neoplasms, discussing morphology, immunohistochemistry, associated genetic findings, and the differential diagnoses.

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“…Patients with unresectable primary tumor or distant metastases were diagnosed via biopsy and did not undergo resection. Such patients showed high mortality [ 19 ].…”

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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Clinical characteristics of epithelioid hemangioendothelioma: a single-center retrospective study

Cited by 28 publications

References 27 publications

Health-related quality of life and symptom burden of epithelioid hemangioendothelioma patients: a global patient-driven Facebook study in a very rare malignancy

Health-related quality of life and symptom burden of epithelioid hemangioendothelioma patients: a global patient-driven Facebook study in a very rare malignancy

Soft Tissue Special Issue: Perivascular and Vascular Tumors of the Head and Neck

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

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