An autopsy case of retroperitoneal paraganglioma that had presented with pheochromocytoma multisystem crisis

A 40's woman was seen at the emergency room because of severe back pain and vomiting. Abdominal CT scan revealed a tumor about 7cm in diameter located on the retroperitoneum. For 6 hours after admission, her body temperature was 39°C and respiratory failure occurred. After 30 hours, the patient died because of a complication in circulatory failure. From the abnormally high level of catecholamines in the blood and autopsy results, we determined that pheochromocytoma multisystem crisis had been caused by the retroperitoneal paraganglioma.     

An autopsy case of chronic thromboembolic pulmonary hypertension with long-term progressive dyspnea]

A 71-year-old man who had been suffering from slowly progressive dyspnea on exertion for 16 years was admitted because of hemoptysis and severe dyspnea in 1997. His medical history included respiratory failure in 1986. At that time, chest X-ray films showed cardiomegaly and striking enlargement of hilar pulmonary arteries, while a lung perfusion scan revealed segmental defects. A diagnosis of chronic thromboembolic pulmonary hypertension (CTPH) was made on the basis of multiple filling defects detected by pulmonary angiography and marked pulmonary hypertension (104/29 mmHg) measured by right heart catheterization. The patient died unexpectedly 1 month later. Autopsy revealed a large new thrombotic lesion straddling the organizing thrombotic lesion in the right pulmonary artery. It is important to keep CTPH in mind when examining and treating patients with unexplained dyspnea. In this respect, our case was considered of value to an understanding of the natural course of CTPH.     

A case of chest wall rhabdomyosarcoma]

A 56-year-old man was admitted to our hospital with right chest pain. Chest X-ray, CT scan and MRI revealed a chest wall tumor and enlarged mediastinal lymph nodes. Percutaneous lung biopsy was performed, and the pathological diagnosis of pleomorphic rhabdomyosarcoma was obtained. The only significant abnormal laboratory finding was elevation of serum NSE (24.5 ng/ml). Although chemotherapy (VAC-ADM) and radiation therapy were performed, the patient died about 7 months after admission. To our knowledge, only 17 cases of chest wall rhabdomyosarcoma have been reported in Japan.     

Pseudomesotheliomatous angiosarcoma of the chest wall and pleura

Angiosarcoma is a rare soft tissue sarcoma that usually occurs in deep soft tissues, breast, spleen, liver, and bone. Primary thoracic parietal localization of angiosarcoma is rare and prognosis is poor. In this report, we present the CT and PET features of a patient with pseudomesotheliomatous angiosarcoma of the chest wall and pleura, which, to the best of our knowledge, have not previously been described.     

An autopsy case of sarcoidosis associated with renal failure]

A 62-year-old woman presented with uveitis and abnormal chest X-ray (bilateral hilar adenopathy). Skin biopsy in 1983 had revealed non-caseating epithelioid cell granuloma consistent with sarcoidosis. Her serum biochemical investigations and exploratory laparoscopy suggested nodular liver cirrhosis, but biopsy was not performed. Both blood urea nitrogen (BUN) and serum creatinine values were within normal limits. She received prednisolone therapy of 15 mg daily initially, and later a maintenance dose of 5 mg daily. In 1985, she complained of skin itching and her laboratory data revealed severe renal insufficiency (BUN 97 mg/dl, serum creatinine 12.2 mg/dl) and hypercalcemia (corrected serum calcium level: 11.5 mg/dl). Prednisolone treatment (40 mg daily) resulted in a dramatic improvement of renal function as well as other clinical abnormalities due to sarcoidosis, without any significant changes in liver function. She died of cerebral infarction in 1989. Autopsy showed interstitial nephritis with tubular calcinosis and hyalinized glomeruli. It is postulated that hypercalcemia due to sarcoidosis contributed to the renal failure in this patient. This case suggests that renal damage due to sarcoidosis may be reversible with appropriate corticosteroid therapy.     

An unusual case of massive subcutaneous chest wall haemorrhage with enoxaparin

Enoxaparin is used in the treatment of acute coronary syndromes and offers improved outcome in the composite endpoint of death, myocardial infarction and major bleeding when compared with unfractionated heparin (UFH). Our report describes a rare case of massive life-threatening subcutaneous chest wall haemorrhage, distant to the injection site. Clinicians should be aware of atypical presentations of haemorrhage when using combination antiplatelet and antithrombotic therapy.     

An unusual case of massive subcutaneous chest wall haemorrhage with enoxaparin

Enoxaparin is used in the treatment of acute coronary syndromes and offers improved outcome in the composite endpoint of death, myocardial infarction and major bleeding when compared with unfractionated heparin (UFH). Our report describes a rare case of massive life-threatening subcutaneous chest wall haemorrhage, distant to the injection site. Clinicians should be aware of atypical presentations of haemorrhage when using combination antiplatelet and antithrombotic therapy.     

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.     

A case of non-invasive pulmonary aspergillosis that rapidly deteriorated]

The pulmonary diseases caused by the Aspergillus species include invasive forms, for example, invasive pulmonary aspergillosis, chronic necrotizing pulmonary aspergillosis, and non-invasive pulmonary aspergillosis. Though these forms are defined pathologically by the presence of the Aspergillus species that invades the lung tissue, they are used as clinical entities. We report a case of non-invasive pulmonary aspergillosis which, from the clinical data, appeared likely to be misdiagnosed as the chronic invasive form. A 45 year-old man received chemoradiotherapy for lung cancer as well as undergoing an left upper lobectomy. Two weeks after the surgery the patient developed a cough, high fever and chest pain. Chest radiography and chest computed tomography showed a rapidly enlarging cavity with an internal mass and infiltration in the left lower lung field. A transbronchial biopsy specimen of the cavity wall showed fungal hyphae. Bronchial washing culture grew Aspergillus fumigatus. Itraconazole and amphotericin B were administered, but the patient's condition did not improve. A left lower lobectomy was performed. The histologic findings showed that the fungal hyphae were only on the surface of the cavity wall, and were surrounded by necrosis and widespread inflammatory cell infiltration. No fungal invasion of the viable lung tissue was seen. The area of infiltration revealed an organizing pneumonia without Aspergillus or other organisms. Our final diagnosis was non-invasive pulmonary aspergillosis. There has been no recurrence of the lung cancer or of the pulmonary aspergillosis in the three years since surgery. It is reported that non-invasive pulmonary aspergillosis passes through a period so active that it seems to be the invasive form for its entire clinical course. To avoid confusion in diagnosis, establishment of a comprehensive clinical classification of pulmonary aspergillosis will be needed.