Journal of clinical and experimental hepatology · 2024
Clinical, Histopathological, and Immunophenotypic Spectrum of Hepatic Epithelioid Hemangioendothelioma: Eight Years' Data of a Tertiary Care Center from North India.
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Kaur Gunjangeet, Mitra Suvradeep, Barwad Adarsh, Chatterjee Debajyoti, Dey Treshita, Khosla Divya, Saikia Uma N, Kaman Lileshwar, Dutta Usha, Duseja Ajay, Das Ashim
Аннотация
BACKGROUND/AIMS: Epithelioid hemangioendothelioma (EHE) is an uncommon vascular tumor that commonly affects the liver. Hepatic EHE (HEHE) presents with variable clinical and histopathological features. We describe detailed clinico-histopathological features, differential diagnosis, and treatment outcomes of the cases of HEHE diagnosed in our center.
METHODS: All cases of HEHE diagnosed in our institute in the last eight years (2016-2023) were reviewed (n = 8; 11 samples) (total 36 cases of EHE; 22.2%). The clinical features, radiology, histopathology, immunophenotype, molecular features, and treatment outcomes of all cases were evaluated.
RESULTS: The median age of presentation was 49.5 years with a female: male ratio of 7:1. Abdominal pain was the commonest presentation. Approximately two-thirds of the patients had multifocal lesions. Histopathology showed purely epithelioid, predominantly epithelioid, and predominantly spindle-cell morphology in 50%, 25%, and 25%, respectively. All cases showed typical myxohyaline/fibrous stroma and organized thrombi of the portal/central veins. CD31 was the most commonly used immunostain with positivity in all cases. CAMTA1 break-apart fluorescence in situ hybridization was positive in 75% of cases, while none showed TFE3 immunopositivity. Chemotherapy was the most commonly employed therapy (n = 5) followed by surgery (n = 2). The median duration of follow-up was 26 months. Five patients were alive with disease (two patients ≥3 years), one patient died of sudden cardiac death, and two patients were lost to follow-up. Two patients developed metastatic disease at follow-up.
CONCLUSIONS: We describe the clinico-histopathological features and differential diagnosis of HEHE. This appears to be the largest case series of HEHE from India.
METHODS: All cases of HEHE diagnosed in our institute in the last eight years (2016-2023) were reviewed (n = 8; 11 samples) (total 36 cases of EHE; 22.2%). The clinical features, radiology, histopathology, immunophenotype, molecular features, and treatment outcomes of all cases were evaluated.
RESULTS: The median age of presentation was 49.5 years with a female: male ratio of 7:1. Abdominal pain was the commonest presentation. Approximately two-thirds of the patients had multifocal lesions. Histopathology showed purely epithelioid, predominantly epithelioid, and predominantly spindle-cell morphology in 50%, 25%, and 25%, respectively. All cases showed typical myxohyaline/fibrous stroma and organized thrombi of the portal/central veins. CD31 was the most commonly used immunostain with positivity in all cases. CAMTA1 break-apart fluorescence in situ hybridization was positive in 75% of cases, while none showed TFE3 immunopositivity. Chemotherapy was the most commonly employed therapy (n = 5) followed by surgery (n = 2). The median duration of follow-up was 26 months. Five patients were alive with disease (two patients ≥3 years), one patient died of sudden cardiac death, and two patients were lost to follow-up. Two patients developed metastatic disease at follow-up.
CONCLUSIONS: We describe the clinico-histopathological features and differential diagnosis of HEHE. This appears to be the largest case series of HEHE from India.