Polypoid sarcoma of the pulmonary trunk: Analysis of the literature and report of a case with leptomeric organelles and ultrastructural features of rhabdomyosarcoma

Abstract

A case of polypoid sarcoma of the pulmonary trunk is described in an 80-year-old woman who had a 21-year history of episodic chest pain and hemoptysis. Ultrastructural examination revealed Z bands, characteristic of rhabdomyosarcoma, and leptomeric organelles. This is the 60th reported case of sarcoma of the pulmonary trunk. Symptoms in reported cases were variable, usually caused by tumor emboli to the lungs or by right ventricular outflow obstruction. Angiography was diagnostic. The tumor was characteristically polypoid, often multicentric, and by definition was fixed to the pulmonary trunk or valves. Non-differentiated sarcoma (often uniquely pleomorphic) was found in 37% of patients, leiomyosarcoma (once confirmed by electron microscopy) in 17%, myxosarcoma in 13%, elements of rhabdomyosarcoma in 8%, fibrosarcoma in 8%, elements of chondrosarcoma in 11%, and 5% were malignant mesenchymomas. The authors suggest that these tumors originate from the undifferentiated tissues of the bulbus cordis and propose the name myenchymoma for the tumor.

Clinicopathological and immunohistochemical features of pulmonary artery sarcoma: A report of three cases and review of the literature

“…Rhabdomyosarcoma is rare in the cardiovascular system, and occurs more often in young people (32). The tumor cells in a rhabdomyosarcoma possess eosinophilic and granular cytoplasm, and are rich in longitudinal filaments, but not stripes.…”

“…The cells are round, spindle, tadpole-, ribbon-or tennis racquet-shaped, with markedly stained nuclei. Interstitial mucus is present within the sparsely packed cells, and the tumor cells express muscle specific actin and desmin (32). In the present study, two cases expressed desmin, but the cells were mostly spindle and epithelioid, not typical of rhabdomyosarcoma.…”

Intrathoracic Myxosarcoma in a Dog

“…The extensive rough endoplasmic reticulum is a feature of fibroblasts and suggests a secretory function, which supports the hypothesis that the fibroblasts are the source of both the mucopolysaccharides and collagen fibres in the matrix (Feldman, 1979). The absence of myofilaments, which show periodic bands or dense bodies (Bleisch and Kraus, 1980), rules out a rhabdomyosarcoma, while the lack of lipid droplets within the cells excludes a myxoid liposarcoma (Feldman, 1979). The ultrastructural features of primitive, fibroblastoid mesenchymal cells in the present case further support the diagnosis of myxosarcoma (Feldman, 1979).…”

Undifferentiated Sarcoma of the Pulmonary Artery Mimicking Pulmonary Thromboembolic Disease

“…It usually grows into the lumen of the vessel as a polypoid mass. 1 One-third of sarcomas of the pulmonary artery are undifferentiated pulmonary artery sarcoma. The other major groups are fibrosarcoma (20%) and leiomyosarcoma (20%).…”

“…2 They are always highly malignant sarcomas, women are involved twice as often as men, and the incidence is 0.001-0.03%. 1 The diagnosis is complicated since the clinical and radiological features are the same as those of chronic pulmonary thromboembolic disease and both occur mostly in women between the ages 40 and 60 years. 3 The only method to differentiate between them is tissue diagnosis, which is possible during angioscopy or at the operation.…”