1. Intimal sarcoma of the pulmonary artery

1. Intimal sarcoma of the pulmonary artery, post-operative recurrence, involving the main trunk, bilateral main branches and the left atrium. (OMH 98-0339)

2. [Heart failure]

1. The cadaver is in state after resection of the intimal sarcoma of the pulmonary artery, performed on February 9 1998. Median incision scar of the anterior thoracic wall is noted at autopsy.

Operation specimen (jpg.20KB) (OMH 98-0339): Primary pulmonary artery sarcoma, open thoracic surgery - intimal sarcoma (HE stain [jpg.44KB]), with involvement of the pulmonary valve. Electron microscopic examination reveals elongated cells (jpg.52KB) and occasionally multipolar giant cells (jpg.75KB) with irregulary indented nuclei, whose cytoplasm contains electron-dense inclusion bodies that measure 500 nm in largest diameter (jpg.46KB), suggesting of lysozomal origin but being somewhat similar to neurosecretory granules. No basal lamina is formed near the tumor cells.

2. The left pleura is firmly adherent to the chest wall, and 330 ml of yellow pleural effusion is present in the left pleural cavity and 400 ml in the right. Pericardial space is firmly adhered. An elastic firm and smooth surfaced tumor is noted around the main trunk of the pulmonary artery (PA) and its main branches that measures about 9 x 6 x 5 cm in external dimension inclusively, accompanying a dumb-bell shaped, apparently separate mass on the posterior aspect of the left atrium that measures 8 x 6 x 3 cm in dimension. Anterior incision through right ventricular outflow tract and pulmonary trunk (jpg.43KB) reveals a tumor thrombus occupying the lumen of the main trunk and bilateral branches of the PA (elastic van Gieson and HE stains [jpg.34KB]), measuring about 7 x 4 x 3 cm in dimension, which in a retrograde fashion protrudes from the outlet at the level of the pulmonary valve into the lumen of the dilated right ventricle that measures 3.5 x 3.5 cm in dimension with smooth surface, and distally involves the upper lobe branch of the right PA 4.0 cm long from the hilus, and the upper and lower lobe branches of the left PA massively. The lumen of the right lower lobe branch of the right PA is free of invasion. The stent, which is consisting of four tandemly and one parallelly arranged metal pieces fairly evenly implanted in the lumen from the pulmonary valve to the hilum of the right PA, is being completely embedded in the tumor thrombus. The lumen of the stent, too, is almost completely obstructed by organizing tumor thrombus. On cut surface at the anterior aspect of the main PA, where the tumor is invading its wall and forming a nodular sessile mass that measures about 6 x 3.5 x 4 cm in dimension, the tumor is yellowish white, solid and uniform. The remaining thread from the previous operation is also identified there. The esophagus and ascending thoracic aorta and other mediastinal structures are free of involvement. Microscopic examination reveals diffuse proliferation of atypical spindle cells arranged in storiform pattern (ordinary type) (jpg.45KB) or diffuse proliferation of pleomorphic giant cells, including bizarre multinucleated giant cells (giant cell type) (jpg.47KB). Immunostains reveal distinct positivity of tumor cells for vimentin (jpg.44KB), questionable positivity for lysozyme, S-100 and alpha-1-anti-trypsin, and negative staining for desmin and SMA.

1. The left lung reveals contraction and induration, especially the medial aspect of the upper lobe that shows marked fibrosis with anthracosis. The left lower lobe shows that sclerotic foci with irregular margins, which measure up to 3 cm in largest diameter, are scattered in the otherwise fairly intact parenchyma. The right lung reveals fairly well aerated parenchyma, but a fibrous scar that is suggestive of old tuberculosis is noted at the apex. Microscopic examination reveals hemorrhagic infarcts of the left lower lobe, and fibrosis with anthracosis of the left upper lobe. The right lung shows fairly intact alveolar structure.

2. The heart weighs 280 gram inclusively with adhered pericardium. The cross sections reveal dilated hypertrophy of the right ventricle, its myocardium measuring 1.0 cm thick. The myocardium of the left ventricle is diffusely softened, which measures 1.2 cm thick. The coronary arteries reveal no remarkable atheroma nor narrowing. Microscopic examination reveals diffuse atrophy of myocardial fibers with interstitial edema and congestion.

3. The capsule of the liver, which weighs 1,040 gram, is smooth, the edge being sharp. On sections, the parenchyma shows remarkable passive congestion. Microscopic examination reveals severe confluent centrilobular congestion associated with atrophy and degeneration of hepatocytes that contain bile thrombi focally.

1. The aorta and its branches are remarkably elastic for a man of that age and only with sparse fatty plaques.

2. Urinary retention of the urinary bladder is noted that contains 400 ml of yellow clear urine. The wall is extended and thinned. The prostate shows benign hypertrophy, which appears a cause of urinary retention.

3. The kidneys are externally healthy and weigh 150 gram each, the capsule stripping with ease. On section, the parenchyma is unremarkable. Histologically, glomeruli appear intact, interlobar, arcuate and interlobular arteries showing mild intimal thickening without remarkable narrowing. There are focal lymphocytic infiltrates in the interstitium.

4. The spleen is elastic firm and weighs 105 gram. Microscopic examination is performed.

5. The esophagus, stomach, small and large intestines are unremarkable. The content is not bloody.

6. The pancreas is healthy to the naked eye and microscopically.

7. The thyroid is unremarkable to the naked eye and weighs 16.5 gram. Histologically, interstitial fibrosis is noted.

8. The adrenals weighs 4.2 gram, left, and 5 gram, right, respectively. Histologically, cortical atrophy with lipoid depletion.

9. The testis is microscopically fairly intact with residual spermatogenesis.

10. Granulomas of foreign body type are scattered in the bone marrow of the sternum. The bone marrow of the lumbar vertebrae and sternum shows normal cellularity of hematopoietic series.

11. The deceased is a well-nourished elderly male who weighs 60 kg and measures 175 cm in height. There is no generalized jaundice.