Residents' Teaching Files

Ignacio, Elizabeth A.; Palmer, Kathryn M.; Mathur, Sharad C.; Schwartz, Arnold M.; Olan, Wayne J.

doi:10.1148/radiographics.19.2.g99mr11531

Cited by 43 publications

(3 citation statements)

References 3 publications

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“…When EHE involves bones, the axial skeleton and lower limbs are the most common sites. 51 Bone lesions are predominantly poorly demarcated and lytic, some with a sclerotic rim at initial presentation. 5 , 7 Expansible mixed density lesions are also recognized.…”

Section: Radiologymentioning

confidence: 99%

Epithelioid hemangioendothelioma, an ultra-rare cancer: a consensus paper from the community of experts

et al. 2021

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Epithelioid hemangioendothelioma (EHE) is an ultra-rare, translocated, vascular sarcoma. EHE clinical behavior is variable, ranging from that of a low-grade malignancy to that of a high-grade sarcoma and it is marked by a high propensity for systemic involvement. No active systemic agents are currently approved specifically for EHE, which is typically refractory to the antitumor drugs used in sarcomas. The degree of uncertainty in selecting the most appropriate therapy for EHE patients and the lack of guidelines on the clinical management of the disease make the adoption of new treatments inconsistent across the world, resulting in suboptimal outcomes for many EHE patients. To address the shortcoming, a global consensus meeting was organized in December 2020 under the umbrella of the European Society for Medical Oncology (ESMO) involving >80 experts from several disciplines from Europe, North America and Asia, together with a patient representative from the EHE Group, a global, disease-specific patient advocacy group, and Sarcoma Patient EuroNet (SPAEN). The meeting was aimed at defining, by consensus, evidence-based best practices for the optimal approach to primary and metastatic EHE. The consensus achieved during that meeting is the subject of the present publication.

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Section: Radiologymentioning

confidence: 99%

Epithelioid hemangioendothelioma, an ultra-rare cancer: a consensus paper from the community of experts

et al. 2021

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“…There is evidence that most patients with this malignancy are males in their second decade [3] and other reports that most cases are in their fifth decade and female [4,5]. One reference even states there is no gender preference [6]. Moreover, the clinical presentation in some reports suggests that the majority of patients present symptomatically with pain [3] or pulmonary symptoms, and other reports suggest most cases are incidental findings in asymptomatic individuals [7].…”

Section: Discussionmentioning

confidence: 99%

Epithelioid Hemangioendothelioma: A Rare Case of an Aggressive Vascular Malignancy

et al. 2019

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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.

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“…Although EHE more commonly arises from soft tissues, it may rarely occur in bone 1,2 . Considering most EHE patients present with multiple lesions and more than one organ involved, 3,4 PET/CT represents a useful imaging modality for evaluation of EHE. In previous studies, EHE demonstrates variable, but most commonly moderate 18 F-FDG uptake on PET/CT 5,6 .…”

mentioning

confidence: 99%

68Ga-FAPI Versus 18F-FDG PET/CT in the Evaluation of Epithelioid Hemangioendothelioma of Bone

Cai²,

Meng³

et al. 2023

Clin Nucl Med

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Epithelioid hemangioendothelioma (EHE) is a rare type of vascular tumor that may arise in bone. Here we presented a 62-year-old man with suspected bone metastases who underwent PET/CT to detect the primary tumor. He underwent 18 F-FDG and 68 Ga-FAPI PET/CT under the prospective trial NCT04416165. Radiotracer uptake was much higher with 68 Ga-FAPI PET/CT than with 18 F-FDG PET/CT in most of bone lesions. No intense uptake likely presenting the primary tumor was observed. Subsequent bone biopsy confirmed the diagnosis of EHE of bone. This case suggested that 68 Ga-FAPI PET/CT might be a useful tool for evaluation of EHE.

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Residents' Teaching Files

Cited by 43 publications

References 3 publications

Epithelioid hemangioendothelioma, an ultra-rare cancer: a consensus paper from the community of experts

Epithelioid hemangioendothelioma, an ultra-rare cancer: a consensus paper from the community of experts

Epithelioid Hemangioendothelioma: A Rare Case of an Aggressive Vascular Malignancy

68Ga-FAPI Versus 18F-FDG PET/CT in the Evaluation of Epithelioid Hemangioendothelioma of Bone

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