The First Case of Composite Hemangioendothelioma in The Heart

Abstract

Composite hemangioendothelioma (CHE) is an extremely rare vascular neoplasm that is characterized by an admixture of benign, low-grade malignant, and malignant vascular components. It is usually located superficially in the dermis and subcutis of the extremities, and other sites involved include the head and neck region, oral mucosa, and viscera of the kidney and spleen. CHE has a low-grade malignant potential because it is locally aggressive. Here, we report a case of CHE in the heart in a 46-year-old man, who presented with a palpable mass arising from his right ventricle. Echocardiogram imaging revealed a 13.3 × 14.2 mm mass with high-signal intensity. Excision was performed, and microscopic examination revealed a heterogeneous mixture of vascular components, consisting of spindle-cell hemangioma, retiform hemangioendothelioma, and epithelioid-like hemangioendothelioma areas. To our knowledge, this is the first report on the behavior of this distinctive vascular neoplasm occurring in the right ventricle. Due to the unclear biological behavior of CHE in the heart and the paucity of cases, no further therapy was undertaken despite the risk of local recurrence and distant metastasis. The result of a six-month follow-up after surgery was disease-free.

Composite haemangioendothelioma in the heart: a case report

“…In 2022, two cases of cardiac CHE were reported. 16 , 17 Details on these cases and comparison with the present case are listed in Table 1 .…”

Dural composite hemangioendothelioma: The first intracranial case

“…Less than 100 cases have been reported in the literature [Table 1]. [4,5,8,[11][12][13]15,[18][19][20][21]25,28,31,32,36,39,41,46,[48][49][50][51][52]55,56,58,60,61,69,74,75,77,80,81,87,88,90,91,93,100,102,103] Two patients with primary spinal CHE have been reported. [58,60] e reported median age at diagnosis is 42.5 years, which is nearly identical to our patient's age.…”

Primary cardiac composite hemangioendothelioma with calcification mimicking a right atrial myxoma: A rare entity

“…CHE is a rare vascular neoplasm of intermediate malignant potential 1 . CHE is usually located on the surface of the dermis and subcutaneous tissue of the extremities, and reports of origin in the heart are very rare 2 . Immunohistochemical and molecular testing can facilitate the correct diagnosis of CHE.…”