Case of primary malignant hemangiopericytoma of the heart expressing basement membrane-degradable enzymes

An echocardiogram demonstrated a large tumor in the left atrium of a 47‐year‐old woman with sudden heart failure. Emergent cardiotomy showed that the tumor arose from the left atrial wall, and almost entirely occupied the left atrium. Grossly, the tumor had partly undergone necrosis. Histologically,...

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Bibliographic Details
Published in:Pathology international 2002-08, Vol.52 (8), p.544-550
Main Authors: Okada, Yoshikatsu, Mori, Hiroshi, Yoshii, Yasuyoshi, Tsuji, Motomu, Horimoto, Hitoshi, Sasaki, Shinjiro
Format: Article
Language:English
Subjects:
Basement Membrane - enzymology
Biomarkers, Tumor - biosynthesis
Echocardiography
Female
heart
Heart Neoplasms - diagnostic imaging
Heart Neoplasms - metabolism
Heart Neoplasms - pathology
hemangiopericytoma
Hemangiopericytoma - diagnostic imaging
Hemangiopericytoma - metabolism
Hemangiopericytoma - pathology
Hemangiopericytoma - secondary
Humans
Immunohistochemistry
Matrix Metalloproteinase 9 - biosynthesis
Middle Aged
Neoplasm Invasiveness
Pelvic Neoplasms - secondary
Radiography
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Summary:An echocardiogram demonstrated a large tumor in the left atrium of a 47‐year‐old woman with sudden heart failure. Emergent cardiotomy showed that the tumor arose from the left atrial wall, and almost entirely occupied the left atrium. Grossly, the tumor had partly undergone necrosis. Histologically, the tumor consisted predominantly of round to polygonal cells with ill‐defined cell borders surrounding ‘stag horn’‐shaped blood vessels. Mitosis was frequently seen with abnormal mitotic figures. Immunohistochemically, the tumor cells diffusely expressed vimentin and focally expressed factor XIIIa and human leukocyte antigen‐DR. A few tumor cells expressed S‐100 protein or α‐smooth muscle actin. Histopathological diagnosis was malignant hemangiopericytoma of the left atrium. Most tumor cells expressed matrix metalloproteinase (MMP)‐2 and MMP‐3, and several cells expressed MMP‐9, all of which are capable of degrading a major component of basement membrane (i.e. type IV collagen). Furthermore, tumor cells expressed membrane type 1 MMP and tissue inhibitor of metalloproteinase‐2, both of which are required for activation of proMMP‐2. Fourteen months after the surgical removal, she died of systemic recurrent tumors.
ISSN:1320-5463
1440-1827
DOI:10.1046/j.1440-1827.2002.01387.x