A Case of Hemangiosarcoma of the Liver which led to a Diagnosis with Hemoptysis

Angiosarcoma is a vascular endothelium-derived malignant tumor that arises in blood vessel walls, accounting for only 2.3% of soft tissue sarcomas in adults. Primary hepatic angiosarcoma(PHA)is rare, comprising< 5% of all angiosarcomas. We report a case of PHA in a 61-year-old man evaluated by another clinic around our hospital for a chief complaint of hemoptysis in May 2012. Chest computed tomography(CT) showed abnormal shadows in bilateral lung fields, so he was referred to Department of Respiratory Medicine at our hospital in late July. However, no definitive diagnosis was not made, even after bronchoscopy. In mid-August, he presented to the outpatient clinic of the respiratory department with a chief complaint of right-sided abdominal pain. Abdominal CT showed a liver lesion, and he was urgently admitted to our department. Initial physical examination was unremarkable except for palpable liver in the right hypochondrium. Tumor markers for liver and biliary cancers were all within normal limits, and negative results were obtained for hepatitis B and C virus. CT, ultrasonography, and(MRI)identified a large lesion replacing the right hepatic lobe and medial segment of the left hepatic lobe. Hemangioma or hepatic angiosarcoma was suspected, accompanied by intraperitoneal rupture. Transcatheter arterial embolization was attempted, but had to be discontinued, and the patient died from hemorrhagic shock due to tumor rupture after onset of abdominal pain. The PHA which assumes hemoptysis primary symptom is extremely rare, and by reports for the past ten years searched using PubMed, this is the second report in the world

A Case of Inflammatory Lung Disease and Retroperitoneal Fibrosis Attributed to Systemic IgG4-related Disease

Recently, immunoglobulin (Ig) G4-related diseases such as autoimmune pancreatitis (AIP), sclerosingsialadenitis, retroperitoneal fibrosis, and sclerosing cholangitis have been reported. IgG4-related diseases arecharacterized by high serum IgG4 concentrations, sclerosing inflammation with numerous IgG4-positiveplasma cells, and steroid sensitivity, irrespective of their organ of origin. We describe a case of inflammatorylung disease and retroperitoneal fibrosis, suggested to involve IgG4. The patient was a 76-year-old man. Acomputed tomographic scan of the chest showed nodular air-space consolidation in the left upper lobe. Theserum IgG4 concentration was abnormally elevated, but there was no evidence of AIP. Bilateral hydronephrosisassociated with thickened soft tissue around the abdominal aorta had been diagnosed previously. Hehad undergone surgery, and retroperitoneal fibrosis was diagnosed histologically (hematoxylin and eosinstain). Histological examination of bronchoscopic specimens taken from the left S3 region showed mononuclear-cell infiltration of the fibrotic bronchial wall, including many IgG4-positive plasma cells. Specimens ofthe region affected by retroperitoneal fibrosis were retrospectively reanalyzed, and the cells were positivefor IgG4 on immunostaining, similar to the lung tissue. The patient responded to treatment with corticosteroids.In conclusion, the present case shared many clinical and clinicopathological similarities with systemicIgG4-related autoimmune disease. To our knowledge, however, this is the first reported case of inflammatorylung disease with retroperitoneal fibrosis in a patient with systemic IgG4-related autoimmune disease