A case report of right atrial epithelioid hemangioendothelioma with multiple pulmonary metastases

Cardiac epithelioid hemangioendothelioma (EHE) is a very rare tumour of endothelial origin with the lung and liver as the most easily metastatic organs. We describe herein a patient with hemoptysis, severe anaemia, and diffuse pulmonary nodules with halo signs that represented metastasis of cardiac EHE; these radiologic manifestations are relatively uncommon. During the initial workup for the patient’s pulmonary nodules, echocardiography missed the cardiac mass. However, positron emission tomography‐computed tomography revealed increased fluorodeoxyglucose intake in the right atrial wall, and cardiac magnetic resonance imaging (MRI) revealed an irregular nodule with normal T1‐weighted signal intensity and hyperintense T2‐weighted signal intensity. Enhanced abdominal computed tomography (CT) revealed micronodular liver metastases. Video‐assisted thoracic surgery was performed to make a definitive diagnosis. Immunohistochemistry staining proved the diagnosis of EHE with positive results for cluster of differentiation (CD) 34, CD31, erythroblast transformation‐specific‐related gene and Ki‐67. The patient started chemotherapy with docetaxel (75 mg/m²) and gemcitabine (900 mg/m²), but this failed to control his disease and he died from an opportunistic infection related to his immunocompromised status 5 months later. For the work out process of bilateral diffuse pulmonary nodules suspicious for cardiac origin, especially with atrial deviation, echocardiography alone is not sufficient to exclude atrial origin. Cardiac CT or MRI might be a better choice.     

An autopsy case of desmoplastic malignant mesothelioma]

On November 15, 2000, a 60-year-old man was admitted to our hospital with progressive dyspnea and right chest pain. He had a 40-year history of occupational asbestos exposure, which began when he was 20 years old. On admission, his chest radiographs showed pleural effusion on the right side, and asbestos bodies were detected in his sputum. Neither a cytological examination of the pleural effusion nor a histological examination of the pleura by percutaneous pleural biopsy revealed malignant cells. In addition, we could not find any other cause for the pleural effusion (such as tuberculosis, collagen disease, or heart failure). In May 2001, the patient also developed pleural thickening and pain in the right hypochondrium, and he was readmitted to our hospital on May 21, 2001. On readmission, an enhanced abdominal CT showed multiple liver tumors, and percutaneous pleural and liver biopsies were performed. The histological findings in the pleura and liver specimens revealed hypocellular collagen tissues without malignant cells. Thus, we could not determine the main cause either of the pleural effusion or of the patient's disease. However, his condition rapidly deteriorated, and he died on August 12, 2001. At the autopsy, bilateral pleural thickening, predominantly on the right side, and invasion of the lungs were observed. The histological findings in the pleural and hepatic tissues revealed hypocellular collagen fibers with a striate pattern and areas of neoplastic spindle cells. He was diagnosed as having malignant desmoplastic mesothelioma with liver metastasis. Cases of malignant desmoplastic mesothelioma have rarely been reported in Japan.     

An autopsy case of acute pulmonary toxicity associated with gemcitabine

Acute respiratory distress syndrome (ARDS) developed following intravenous gemcitabine monotherapy in a 75-year-old man with non-small cell lung cancer. The total dose of gemcitabine was 1,500 mg, and the latent period from starting gemcitabine to pulmonary toxicity was three days. The chest radiographs and high resolution computed tomographic scan revealed bilateral ground-glass opacity. He died on the fourteenth post-chemotherapeutic day due to respiratory failure. Postmortem examination of the lung revealed mixed exudative and fibrotic stages of diffuse alveolar damage. Pulmonary toxicity from gemcitabine can be acute and fatal.     

An autopsy case of pulmonary metastasis of cholangiocellular carcinoma associated with marked fibrotic change of the lungs]

An autopsy case of pulmonary metastasis of cholangiocellular carcinoma is presented. A 44-year-old woman was admitted to our hospital because of dyspnea, general fatigue and a sense of abdominal fullness on February 5, 1990. In November 1986, at an other hospital, she had been diagnosed as having diffuse metastatic lung tumor and multiple bone metastases, by transbronchial lung biopsy and other examinations. During the clinical course, she was not received chest irradiation and chemotherapy which induced fibrotic change of lungs. Chest X-ray film on December 21, 1986 showed diffuse nodular shadows in both lung fields. Chest X-ray film on February 4, 1990 showed diffuse reticular shadows with marked shrinkage of lung fields. She died two months after admission. The primary site of the carcinoma was not determined clinically, but was revealed by autopsy to be cholangiocellular carcinoma of the liver, with generalized metastasis. Microscopic findings of the autopsied lung showed markedly increased connective tissue around bronchi and blood vessels, in areas where microtubular adenocarcinoma was scattered. This is a very rare case of pulmonary metastasis of cholangiocellular carcinoma, associated with marked fibrotic change of the lungs during about 3.5 years. To our knowledge, this is the first reported case.     

Pulmonary epithelioid hemangioendothelioma coexistent with pulmonary metastasis of thyroid cancer

We report a 45-year-old man with epithelioid hemangioendothelioma (EH) and simultaneous pulmonary metastasis of thyroid cancer in his lung. Thyroid cancer, and multiple small nodules in both lungs were noted. He underwent total thyroidectomy followed by radiotherapy with 131I. However, 131I scintigraphy showed poor uptake of radionuclide in the nodules, and the size of the nodules remained unchanged. The diagnostic thoracoscopic biopsy showed two types of nodules, some were positive for thyroglobulin and cytokeratin, and others were reactive for factor VIII. The former nodules were diagnosed as pulmonary metastases of thyroid cancer, and the latter EH.     

An autopsy case of Marfan syndrome with bronchiectasis and multiple bullae]

We report an autopsy case (27-year-old male) with Marfan syndrome, who died of chronic respiratory failure due to bronchiectasis and multiple bullae in both lungs. He had suffered from expectoration of massive amounts of sputum since the age of 15 years. At this time, chest roentgenogram had revealed bronchiectatic changes in the bilateral lower lung fields. Seven years later at the age of 22 years, the formation multiple bullae in both lungs were added to the bronchiectatic changes on chest roentgenogram. Administration of erythromycin (400 mg/day) was started in February, 1987, and the massive sputum volume markedly decreased according to appearance of bullous formation. He was admitted to our department because of deterioration with chronic respiratory failure and right heart failure at 26 years in December, 1989. Although various therapy was performed, he died of chronic respiratory failure in February, 1990. Autopsy findings were as follows: (1) cyclindrical bronchiectatic changes in bilateral lower lobes and (2) extensive multiple bullae in the subpleural areas with bronchiectatic changes in the middle and bilateral lower lobes, with no bronchiectatic changes in the bilateral upper lobes. Several pulmonary disorders accompanying Marfan syndrome have been reported, especially in children. However, the present case demonstrated that fetal pulmonary involvement by Marfan syndrome may not present until adulthood, and affect both airways and lung parenchyma.     

An autopsy case of Mycobacterium abscessus pulmonary infection complicated with rheumatoid arthritis

We present the case of a 70-year-old man diagnosed with Mycobacterium abscessus pulmonary infection complicated with rheumatoid arthritis who was treated with corticosteroids. He died despite treatment according to the recommended regimen of imipenem, clarithromycin, and amikacin. Autopsy revealed granulomatous lesions throughout the bilateral lungs. We conclude that M. abscessus infection may have a fatal outcome because of the drug resistance of the pathogen. This case suggests that rheumatoid arthritis might be a risk factor for M. abscessus pulmonary infection, but further studies are necessary for clarification.