Epithelioid Hemangioendothelioma: A Rare Case of an Aggressive Vascular Malignancy

Abstract

Patient: Female, 46 Final Diagnosis: Epithelioid hemangioendothelioma Symptoms: Pain Medication: — Clinical Procedure: — Specialty: Oncology Objective: Unusual clinical course Background: Epithelioid hemangioendothelioma (EHE) is a rare vascular malignancy that occurs in multiple organs and tissues with a predilection for the extremities, bone, liver, and lung. It is often characterized by a clinically indolent course, delayed diagnosis, and unestablished standardized treatment options. Case Report: A 46-year-old female presented with a 2-month history of right shoulder and arm pain. Imaging revealed involvement of the humerus, lung, liver; and brain and biopsies of both the lung and humerus were performed. A diagnosis of epithelioid hemangioendothelioma was confirmed and the patient received radiation therapy to the right humerus and brain stereotactic radiosurgery. She was scheduled to begin palliative chemotherapy with doxorubicin but developed complications and never received chemotherapeutic agents. She died 5.5 months from her first admission with widespread metastasis. Conclusions: Epithelioid hemangioendothelioma (EHE) is typically a low-to-intermediate grade vascular malignancy, but, as seen in this case, can be aggressive. In the future, diagnosis, prognosis, and treatment may improve using genetic or immune therapy considering a structural chromosomal translocation has been identified.

Massive Calcified Epithelioid Hemangioendothelioma With Multifocal Involvement: An Imaging Diagnosis Dilemma and a Rare Case Report

“…The age at diagnosis ranges from 3 years to 86 years, with a peak incidence in middle-aged adults and a mild female predilection (male: female: 1:4) (5,6). Most patients have nonspecific symptoms on initial presentation with generally indolent clinical activity, but can sometimes demonstrate aggressive clinical behavior (7). histologic, immunohistochemical, and molecular characteristics since there is nonspecific imaging and the differential diagnosis for EHE is broad.…”

Long-Term Observation and Treatment of Epithelioid Haemangioendothelioma of the Mediastinum: A Case Report

“…The preoperative misdiagnosis of EHE is usually due to its low incidence, lack of specific oncological markers, and unclear pathogenesis (1,13). However, the improvement of medical imaging equipment and diagnostic technology has made it possible for medical imaging, including CT, MRI, and F-FDG-PET to show certain pathological characteristics of EHE (1,(6)(7)(8). Mediastinal EHE is typically expressed as a soft-tissue mass with a well-defined margin on CT and MRI, and it is less likely to invade the surrounding tissues due to its relatively indolent clinical course compared to EHE originating from liver, lung, and soft tissue (7,8,18).…”

“…However, EHE was described as a malignant vascular tumour distinct from epithelioid angiosarcoma in the fifth revision of the WHO classification (5). The primary tumour can occur anywhere in the body, and it arises in the lung, bone, and liver in more than 65% of all cases (1,6), while the mediastinum is a rather rare site (7,8). Due to its rarity, atypical symptoms, and non-specific laboratory indicators, preoperative misdiagnosis is usual.…”

Intracranial Epidermoid Cyst Mimics Musculoskeletal Shoulder Disease: Findings from a Case Report in Physiotherapy Clinical Practice

“…Notably, shoulder pain can be caused by many non-neuro-musculoskeletal disorders [ 3 , 6 , 7 , 8 , 9 ]. In this context, some rare intracranial pathologies, in particular, can affect the shoulder complex in terms of pain and disability [ 6 , 10 ]; they are often characterized by compressive space-occupying masses [ 7 ] and/or ischemia in areas that are functionally connected to the shoulder [ 10 ]. Specifically, most of these compression phenomena may affect structures that contribute to the innervation of the shoulder muscles, causing motor deficits and decreased functional capacity.…”