Bony and cartilaginous tumors of the heart and great vessels.Report of an osteosarcoma of the pulmonary artery

Abstract

An osteosarcoma of the pulmonary artery is reported, and 8 other cases of tumors of the heart and great vessels containing bony or cartilaginous components are reviewed. All arose near the base of the heart, and all contained a fibrosarcomatous element. Women in the fourth and fifth decade are most frequently affected and survive an average of 27 months from first symptom. These tumors are sluggishly aggressive, but eventually fatal because of their critical location. They probably arise from fibrous tissue of the cardiac valves, atrioventricular groove, or vascular intima and undergo malignant metaplasia into bone, cartilage, and other mesenchymal elements.

Canine Extraskeletal Osteosarcoma and Chondrosarcoma: a Clinicopathologic Study of 14 Cases

“…'~J~~~~ The extremities are the most common site (77% to 85%) for these neoplasms in human beings, but they have been reported in other tissues including the urinary bladder, intestines, liver, thymus, major blood vessels, testis, and retropentoneum. 10,15,20,22,24,32,33,37,40 These tumors are very malignant in human beings; 69% recur locally and 80% met a s t a~i z e .~~,~~,~~ A five-year survival rate is reported in 20% to 25% of p a t i e n t~. l~J~.~~ Extraskeletal osteosarcomas, also rare in human beings, have been described at various ~i t e s .…”

A fatal case of primary cardiac chondrosarcoma presenting with amaurosis fugax

“…Sarcomas comprise the largest group of malignant primary cardiac neoplasms, with angiosarcomas being the most common subtype. In contrast, PCCs are exceedingly rare, especially in the left heart, with only nine cases reported in the English literature since the initial report by McConnell in 1970 4. Although right heart PCCs are more common, they are also more likely to represent metastatic lesions than primary cardiac tumours 5 6…”

Leiomyosarcoma of the pulmonary trunk associated with pericardial effusion

“…Their patient had a fibromyxosarcoma of the pulmonary artery associated with syncope, secondary polycythaemia, and thrombocytopenia. In a few other patients syncope was a presenting symptom (Lowell and Tuhy, 1949;Hagstrom, 1961;McConnell, 1970). The case described by Wackers et al (1969) concerned a leiomyosarcoma of the pulmonary trunk with a haemorrhagic tendency attributed to an intravascular coagulopathy.…”

“…In the same tumour different histological types may be present (Moegen, 1951;Hagstrom, 1961;McConnell, 1970). McConnell (1970) postulates that chondroand osteosarcoma arise as a fibrosarcoma and then undergo metaplasia into specific mesenchymal tissue. Secondary deposits in the lungs and direct invasion of the mediastinum are often seen, but metastases outside the thorax are rarely encountered (Elphinstone and Spector, 1959).…”

“…In 1969 Wackers, van der Schoot, and Hampe collected 21 cases from the literature, including their own case, and presented a review of the reported case histories. McConnell (1970) called attention to primary neoplasms of the heart and great vessels containing bony and cartilaginous elements. He found eight patients described in the literature, three of whom had tumours located in the pulmonary artery, and reported another patient with an osteosarcoma of the pulmonary artery arising from the area of the pulmonary valves.…”