Epithelioid Hemangioendothelioma

Cousin, Sophie; Loarer, François Le; Crombé, Amandine; Karanian, Marie; Minard, Véronique; Penel, Nicolas

doi:10.1007/978-3-030-24697-6_7

Cited by 2 publications

(1 citation statement)

References 65 publications

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“…These features are not considered low risk. They reported a 5-year disease-specific survival rate of 59% and a metastasis rate of 32% for the high-risk group ( 1 , 11 ).…”

Section: Discussionmentioning

confidence: 99%

A case report of parotid gland epithelioid hemangioendothelioma

Wang,

Hu,

Wen

et al. 2024

Front. Surg.

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Epithelioid hemangioendothelioma (EHE) is a rare low-grade malignant vascular tumor. It mainly occurs in the liver, lungs, bones, and other parts of the body. Reports of epithelioid hemangioendothelioma in the parotid gland are rare in both domestic and international literature. Here, we present a case report of a parotid gland epithelioid hemangioendothelioma, including its complete clinical course and imaging findings, to improve the diagnosis and treatment of this unusual disease.Case presentationThe patient, a 75-year-old female, presented with a swelling around the right ear for 2 months and pain for 20 days. Enhanced MRI of the parotid gland revealed a well-defined, round mass with homogeneous signal intensity. The mass showed low signal intensity on T1-weighted imaging, high signal intensity on T2-weighted imaging, nodular low signal intensity within, significant high signal intensity on DWI sequence, low signal intensity on ADC sequence, and heterogeneous enhancement in the arterial phase after intravenous injection of Gd-DTPA. Nodular non-enhancing low signal intensity was observed internally, and slight clearance was seen in the venous phase. The initial diagnosis before surgery was a benign lesion, but after histopathological and immunohistochemical examination, it was confirmed as epithelioid hemangioendothelioma.InterventionComplete tumor resection was performed.ResultsThe patient experienced a favorable recovery, with meticulous follow-up conducted for up to 1 year revealing no signs of recurrence or metastasis. Continued patient surveillance is ongoing to substantiate and validate the long-term efficacy of the treatment.ConclusionDue to the extreme rarity of parotid gland epithelioid hemangioendothelioma, it often leads to a high misdiagnosis rate. The most common misdiagnosis is salivary gland lymphoma, followed by epithelioid hemangiosarcoma. When the lesion is multifocal, fusiform, with internal necrosis, and shows punctate low signal intensity on T2-weighted imaging, significant enhancement in the arterial phase, particularly with more pronounced peripheral enhancement, and persistent enhancement in the venous and delayed phases, epithelioid hemangioendothelioma should be considered. However, the current clinical diagnosis of epithelioid hemangioendothelioma still primarily relies on immunohistochemical methods.

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“…These features are not considered low risk. They reported a 5-year disease-specific survival rate of 59% and a metastasis rate of 32% for the high-risk group ( 1 , 11 ).…”

Section: Discussionmentioning

confidence: 99%

A case report of parotid gland epithelioid hemangioendothelioma

Wang,

Hu,

Wen

et al. 2024

Front. Surg.

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Subcapital Femoral Neck Fracture in a Professionally Active Patient Undergoing Palliative Treatment for Endothelial Cell-Derived Epithelioid Haemangioendothelioma (EHE)

Kluszczyk,

Tobiasz,

Szumilas

et al. 2024

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Background and Clinical Significance: Femoral neck fracture frequently occurs in the elderly population but may also present in patients diagnosed with primary cancer or bone metastases. A pathological, oligosymptomatic fracture associated with epithelioid haemangioendothelioma (EHE), a rare endothelial cell-derived sarcoma, is uncommon. Case Presentation: A 44-year-old patient underwent biopsy procedures three times (2010, 2012, 2013) for a focal lesion of the left ischium, none confirming its malignant nature. The last biopsy revealed a neoplastic tissue with features of discrete dysplasia. The lesion did not undergo medical follow-up for seven consecutive years. In August 2020, the patient presented with right lower limb pain. A CT scan, PET/CT scan, and biopsy confirmed EHE with spindle/sarcomatous features. In November 2020, chemotherapy (5xADIC) started (PET/CT confirmed a partial response). After its completion in July 2021, bone progression occurred and sirolimus-based therapy was started. After 3 months, a small liver metastasis was visualized on PET/CT, which did not result in the termination of treatment. In December 2021, pamidronate-based antiresorptive therapy was started. Liver metastasis remained stable in follow-up CT scans. Due to pelvic and spinal lesions, the patient was assisted by elbow crutches and underwent radiotherapy, remaining professionally active. The patient did not report any trauma, but in August 2023, a subsequent CT scan revealed a subcapital fracture of the left femoral neck in the fusion phase. Due to pelvic changes and the stable nature of the fracture, surgical treatment was abandoned. Conclusions: An oligosymptomatic femoral neck fracture, not requiring medical intervention is considered a rare complication of bone cancer.

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Epithelioid Hemangioendothelioma

Cited by 2 publications

References 65 publications

A case report of parotid gland epithelioid hemangioendothelioma

A case report of parotid gland epithelioid hemangioendothelioma

Subcapital Femoral Neck Fracture in a Professionally Active Patient Undergoing Palliative Treatment for Endothelial Cell-Derived Epithelioid Haemangioendothelioma (EHE)

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