Cardiology>>>>>Genetics, Lipids And Tumours
Question 29#

A 57-year-old male patient is discovered to have an echogenic mass arising from the insertion point of the anterior mitral valve leaflet. He undergoes uneventful surgical resection. The mass is described as reddish and nodular. Frequent areas of calcification are identified by naked eye. Histologically, it is composed of oval CD34, CD31, and factor VIII positive cells consistent with epithelioid haemangioendothelioma. 

Which one of the following five statements regarding this tumour is true?

A. It is rare in females, with a male:female ratio of 20:1
B. It is usually presented by the presence of metastases
C. It typically occurs from the intra-atrial septum
D. The differential diagnosis of adenocarcinoma is excluded by the presence of CD31, CD34, and factor VIII positive cells
E. It often presents with complete heart block

Correct Answer is D

Comment:

Previously considered a low-grade or borderline malignant vascular lesion, epitheloid haemangioendothelioma is classified as a malignant tumour, along with angiosarcoma, because of its local aggressiveness and metastasizing potential. It is a very rare tumour.

There is a spectrum of disease with benign epithelioid haemangiomas at one end and the highly malignant epithelioid angiosarcoma at the other. The epithelioid haemangioendothelioma sits in the middle. Systemic metastasis is reported in approximately 20% of cases described in the medical literature (Lisy M). Treatment is radical surgical resection. There is limited value in radiotherapy and chemotherapy (Moulai N).

Originating from the subendocardium, these tumours can occur at any location within the heart. The expression of vascular endothelial markers, such as von Willebrand factor, CD31, and CD34, rules out metastatic adenocarcinoma or melanoma. The prognosis is unpredictable and typically poor