Epithelioid Hemangioendothelioma

The term "hemangioendothelioma" is used for endothelial neoplasms of intermediate malignancy and describes a group of rare neoplasms having biologic behavior falling in between that of the benign hemangiomas and fully malignant angiosarcomas. The hemangioendotheliomas fall into several specific, clinicopathologically and genetically distinct entities, specifically epithelioid hemangioendothelioma, kaposiform hemangioendothelioma, papillary intralymphatic angioendothelioma and retiform hemangioendothelioma (hobnailed hemangioendothelioma), pseudomyogenic hemangioendothelioma, composite hemangioendothelioma, and YAP1::TFE3-fused hemangioendothelioma...

Epithelioid hemangioendothelioma (EHE) is a rare vascular malignant tumor that comprises less than 1% of all vascular tumors. Cutaneous involvement in EHE can occur either by spreading from underlying bone or rarely could be limited to the skin and mostly presents as solitary well-circumscribed mass to an ill-defined infiltrative lesion. We present a case of rapidly progressive and debilitating EHE presenting multiple vascular papules and nodules. Histopathology showed an ill-circumscribed nodular proliferation of epithelioid and spindled cells in the dermis that extended into the subcutaneous tissue...

Pseudomyogenic hemangioendothelioma (PMHE), a rare vascular neoplasm, was first described in 1992 asa fibroma-like variant of epithelioid sarcoma, and would be termed as epithelioid sarcoma-like hemangioendothelioma a decade later due to its significant histologic overlap with epithelioid sarcoma and diffuse cytokeratin expression. PHME is currently defined as a distinct, potentially intermediate malignant, rarely metastasizing neoplasm with vascular/endothelial differentiation. It is characterized by young age (typically less than 40 years old), extremity location (approximately ~80%), and t(7:19) SERPINE1::FOSB fusion as the most common molecular alteration...

PURPOSE: Disease control and survival following percutaneous ablation of hepatic epithelioid hemangioendothelioma (EHE) was studied retrospectively. METHODS: Six patients underwent 16 image-guided ablation procedures to treat 35 liver tumors from 2015 to 2022 (17 microwave ablation, 9 irreversible electroporation, 8 cryoablation, and 1 radiofrequency ablation). Technical success, local progression, intrahepatic progression, distant progression, overall survival, and adverse events were assessed...

Primary vascular tumors of bone are a heterogeneous group of neoplasms, ranging from benign hemangiomas to frankly malignant epithelioid hemangioendotheliomas and angiosarcomas. Over the years, their classification has been a matter of discussion, due to morphologic similarities and uncertainty regarding biologic behavior. Over the past decade, with the development of next-generation sequencing, there has been a significant improvement in the molecular characterization of these lesions. The integration of their morphologic, immunohistochemical and molecular features has led to a better stratification, with important prognostic and therapeutic implications...

BACKGROUND: The missing requirement for resection for the majority of hepatic hemangiomas (HH) and tissue scarcity for rare diseases such as hepatic epithelioid hemangioendotheliomas (HEHE) complicate the characterization of the spatial immunovascular niche of these benign and malignant vascular neoplastic diseases. METHODS: Two tissue cohorts containing 98 HHs and 13 HEHEs were used to study entity-specific and disease stage-specific endothelial cell (EC) phenotype and immune cell abundance...

Despite significant advances in their molecular pathogenesis, skeletal vascular tumors remain diagnostically challenging due to their aggressive radiologic appearance and significant morphologic overlap. Within the epithelioid category and at the benign end of the spectrum, recurrent FOS/FOSB fusions have defined most epithelioid hemangiomas, distinguishing them from epithelioid hemangioendothelioma and angiosarcoma. More recently, the presence of EWSR1/FUS :: NFATC1/2 fusions emerged as the genetic hallmark of a novel group of unusual vascular proliferations, often displaying epithelioid morphology, with alternating vasoformative and solid growth, variable atypia, reminiscent of composite hemangioendothelioma...

BACKGROUND: Hepatic epithelioid hemangioendothelioma (EHE) is an extremely rare tumor, and no standard therapy has been established yet. OBJECTIVES: The aim of this study was to investigate the short-term results of combined therapy with sirolimus and interferon-alpha 2b (IFN-a 2b) (SI therapy). METHODS: From January 2022 to April 2023, 40 patients histologically diagnosed with hepatic EHE and progressive disease received SI therapy. All patients were regularly evaluated for the safety and efficacy of the SI therapy...

The diagnosis or rare mesenchymal malignant lesions of the liver may be a challenge owing to the rarity of the disease and is usually made by histological confirmation. An ultrasound examination with, if required, color Doppler sonography and contrast-enhanced ultrasound, taking into account the clinical background of the patient, may help to focus the differential diagnosis. In this review, we describe the pathological and ultrasound features of several rare mesenchymal malignant liver lesions which include undifferentiated sarcoma of the liver, leiomyosarcoma, angiosarcoma, fibrosarcoma, liposarcoma, and epithelioid hemangioendothelioma...

PURPOSE: To investigate and compare the ultrasonic features of hepatic epithelioid hemangioendothelioma (HEHE) and other common hepatic malignancies, such as hepatocellular carcinoma (HCC), intrahepatic cholangiocarcinoma (ICC) and hepatic metastatic tumor (HMT). METHODS: A total of 37 patients with pathologically proven HEHE, 37 HCC cases, 37 ICC cases, and 37 HMT cases were enrolled from single hospital. The clinical characteristics and ultrasonic features of all cases were summarized and statistically analyzed...

Malignant focal liver lesions (FLLs) represent various kinds of epithelial and mesenchymal tumors. In pediatric patients, the understanding of pediatric liver diseases and associated imaging manifestations is essential for making accurate diagnosis and differential diagnosis. This paper will discuss the latest knowledge of the common pediatric malignant FLLs, including undifferentiated embryonal sarcoma, rhabdomyosarcoma, epithelioid hemangioendothelioma, angiosarcoma, and malignant rhabdoid tumor. Medical imaging features are not only helpful for clinical diagnosis, but can also be useful in the evaluation and follow-up of pre- and post-treatment...

Femoral epithelioid hemangioendothelioma with cervical lymph node metastasis is rare. We report the FDG PET/CT findings of cervical lymph node metastasis from left femoral epithelioid hemangioendothelioma in a 50-year-old woman with painless enlargement of the left cervical lymph nodes as the initial presentation. Ultrasound and MRI revealed multiple enlarged lymph nodes in the left cervical sheath area. PET/CT showed strong radioactive uptake in the left cervical lymph nodes, and there was additional lesion with increased FDG uptake in the left femur, which was later confirmed as cervical lymph nodes metastasis from left femoral epithelioid hemangioendothelioma by pathological examination...

Epithelioid hemangioendothelioma of the prostate is a rare malignant vasogenic tumor. We report a case of epithelioid hemangioendothelioma of the prostate in a 65-year-old man with lymph nodes and lung metastases on 18F-FDG PET/CT imaging. The patient presented with symptoms of frequent and urgent urination. On 18F-FDG PET/CT, intense FDG uptake was observed in the prostate mass along with multiple FDG-avid lesions involving the lung and lymph nodes. Histopathological examination confirmed epithelioid hemangioendothelioma in both the prostate mass and lung nodule...

Our case highlights the 18F-fluorodeoxyglucose positron emission tomography/computed tomography (18F-FDG PET/CT) scan findings in a rare case of biopsy-proven epithelioid hemangioendothelioma (EHE) in a 66-year-old woman with multi-organ involvement (lung, liver, and bone) who was subsequently treated with palliative radiation therapy and oral pazopanib. Furthermore, follow-up 18F-FDG PET/CT findings are detailed. EHE is a rare malignant vascular neoplasm (<1% of all vascular tumors) with an epithelioid and histiocytoid appearance arising from the vascular endothelial and preendothelial cells...

Hepatic epithelioid hemangioendothelioma (EHE) is an uncommon vascular endothelial cell tumor of the liver with numerous symptoms and features. The median affected age is 41, and females are more frequently affected than men. In the following article, a 37-year-old nurse is presented who was referred to the hospital with severe right upper quadrant pain. She had been misdiagnosed with hepatic hemangioma for years, which finally turned out to be hepatic EHE. Liver transplantation has been recognized as the therapeutic method of choice due to the considerable extent of liver involvement and nonresponse to medications...

[This corrects the article DOI: 10.3389/fonc.2023.1109643.].

TAZ::CAMTA1 is a fusion protein found in over 90% of Epithelioid Hemangioendothelioma (EHE), a rare vascular sarcoma with an unpredictable disease course. To date, how TAZ::CAMTA1 initiates tumour formation remains unexplained. To study the oncogenic mechanism leading to EHE initiation, we developed a model system whereby TAZ::CAMTA1 expression is induced by doxycycline in primary endothelial cells. Using this model, we establish that upon TAZ::CAMTA1 expression endothelial cells rapidly enter a hypertranscription state, triggering considerable DNA damage...

Epithelioid hemangioendothelioma (EHE) is a rare sarcoma of vascular origin. It is frequently seen in the liver, lungs and bones. Cases with mastoid bone and intracranial extension are rarer. A 40 year old male, presented with a history of progressive headaches and a mass in the postauricular region for a duration of three months. Computer tomography of the temporal bone showed an approximately 40 × 30 mm soft tissue with osteolytic bony changes located in the mastoid bone. Epithelioid hemangioendothelioma is a rare sarcoma of vascular origin...

Vascular tumors of the liver are mesenchymal lesions from endothelial cells. They range from common benign lesions such as haemangioma, intermediate tumors like Kaposi sarcoma, and perivascular epithelioid cell tumor to malignant tumors such as hepatic epithelioid hemangioendothelioma and hepatic angiosarcoma in adults. Pediatric vascular tumors of the liver also include benign, locally aggressive, borderline, and malignant masses with haemangiomas being the most common benign tumors and epithelioid hemangioendothelioma being an uncommon pediatric malignancy...

Osseous vascular anomalies can be characterized as vascular tumors or malformations. Classification is vital for prognosis and treatment. Much remains unknown about conditions such as Gorham-Stout disease. Treatments target the proposed genetic pathways such as PI3KCA/AKT/mTOR pathway.