The First Case of Composite Hemangioendothelioma in The Heart
DOI:
https://doi.org/10.1532/hsf.4587Keywords:
Composite hemangioendothelioma, Heart tumor, Surgical treatmentAbstract
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.
References
Aydingoz IE, Demirkesen C, Serdar ZA, et al. 2009. Composite haemangioendothelioma with lymph-node metastasis: an unusual presentation at an uncommon site. Clin Exp Dermatol. 34(8): p. e802-6.
Bhat A, Chowdappa V. 2016. Composite Hemangioendothelioma: Report of a Rare Case. J Clin Diagn Res. 10(10): p. ED01-ED03.
Biagioli M, Sbano P, Miracco C, et al. 2005. Composite cutaneous haemangioendothelioma: case report and review of the literature. Clin Exp Dermatol. 30(4): p. 385-7.
Chen YL, Chen WX, Wang J, et al. 2012. Composite hemangioendothelioma on the neck. Kaohsiung J Med Sci. 28(10): p. 564-5.
Fasolis M, Iaquinta C, Montesco MC, et al. 2008. Composite hemangioendothelioma of the oral cavity: case report and review of the literature. Head Neck. 30(7): p. 974-9.
Hisaoka M, Kouho H, Aoki T, et al. 1995. DNA flow cytometric and immunohistochemical analysis of proliferative activity in spindle cell haemangioendothelioma. Histopathology. 27(5): p. 451-6.
Leen SL, Clarke PM, Chapman J, et al. 2015. Composite Hemangioendothelioma of the Submandibular Region. Head Neck Pathol. 9(4): p. 519-24.
Li WW, Liang P, Zhao HP, et al. 2021. Composite hemangioendothelioma of the spleen with multiple metastases: CT findings and review of the literature. Medicine (Baltimore). 100(21): p. e25846.
Mahmoudizad R, Samrao A, Bentow JJ, et al. 2014. Composite hemangioendothelioma: An unusual presentation of a rare vascular tumor. Am J Clin Pathol. 141(5): p. 732-6.
Miyamoto E, Seki K, Katsuragawa H, et al. 2021. Thoracic composite hemangioendothelioma with neuroendocrine marker expression. Surg Case Rep. 7(1): p. 249.
Nayler SJ, Rubin BP, Calonje E, et al. 2000. Composite hemangioendothelioma: a complex, lowgrade vascular lesion mimicking angiosarcoma. Am J Surg Pathol. 24(3): p. 352-61.
Rokni GR, Montazer F, Sharifian M, et al. 2017. Composite hemangioendothelioma of the forehead and right eye; a case report. BMC Dermatol. 17(1): p. 15.
Tsai JW, Huang HY, Lee JC, et al. 2011. Composite haemangioendothelioma: report of four cases with emphasis on atypical clinical presentation. Pathology. 43(2): p. 176-80.
Zhang J, Wu B, Zhou GQ, et al. 2013. Composite hemangioendothelioma arising from the kidney: a case report with review of the literature. Int J Clin Exp Pathol. 6(9): p. 1935-41.
