Idiopathic retroperitoneal fibrosis (IRPF) is an inflammatory fibrosclerosing condition, leading to renal failure by obstruction of the ureters. Idiopathic chronic pancreatitis associated with marked inflammatory infiltrates has recently been referred to as autoimmune pancreatitis (AIP), and infiltrating plasmacytes carrying immunoglobulin-gamma type 4 (IgG4) are relevant to its pathogenesis. The case is described herein of IRPF associated with subclinical pancreatitis that was most probably AIP in a 70-year-old man. Biopsy specimens of the retroperitoneal pseudotumor revealed a marked lymphoplasmacytic infiltration with dense fibrosis. Infiltrating plasma cells were immunoreactive for anti-IgG4 antibodies. Subsequent systemic examinations showed an extremely elevated serum IgG4 level and pancreatitis concordant with AIP. Following oral steroid administration, the serum IgG4 level normalized, although the appearance of the pseudotumor did not alter. Some AIP cases have been associated with idiopathic fibrosclerosing disorders including IRPF, but histological evidence of IgG4-related IRPF has rarely been provided.

A 38-year-old man who had been followed with diagnosis of dilated cardiomyopathy and retinal angioma was referred to our hospital because of incidentally detected bilateral adrenal masses. Although he was normotensive, levels of catecholamine in urine were elevated and I131-MIBG scintigraphy showed accumulation in bilateral adrenal glands. Screening of central nervous system by MRI revealed cerebellar hemangioblastoma. Right adrenalectomy and left partial adrenalectomy were performed, both of which tumors pathologically diagnosed as pheochromocytoma, followed by resection of the cerebellar hemangioblastoma. Five months later, abdominal CT revealed a left renal tumor and underwent left partial nephrectomy, being diagnosed as renal cell carcinoma. A right renal tumor was detected on follow up CT at 1 year after the partial nephrectomy. Since cardiac function was deteriorated, we have followed with careful observation. This is the second documented case of bilateral renal cell carcinomas and bilateral pheochromocytomas with VHL in Japan.

We report a case of retroperitoneal gas gangrene, which was caused by cecal diverticulitis with perforation. A-57-year-old male was admitted to the Sado General Hospital with the chief complaint of right lateral abdominal pain. Roentogenogram and Computelized Tomography (CT) showed gas accumulation in the retroperitoneal space behind the ascending colon. Based on the clinical, labolatory, and instrumental examination findings gas gangrene was diagnosed. Since urolithiasis or urinary tract infection was suspected to be the cause of the lesion at that time, the patient was transferred to our department immediately. CT scan done on day 3 at our inpatient department provided data suspicious for the cecal perforation into retroperitoneal space due to appendicitis or diverticulitis. We performed an acute drainage of the abscess and intensive care including continuous hemodiafiltration (CHDF), oxygen under high pressure (OHP), and chemotherapy with antibiotics was carried out. However, in spite of the above mentioned measures, the patient's condition deteriorated and he died due to progression of gangrene and multiple organ failure in 23 days. The autopsy revealed that the cause of perforation was cecal diverticulitis. Retroperitoneal gas gangrene is an uncommon entity and has been rarely reported. It is supposed that laparotomy with diagnostic and therapeutic purpose should have been performed in this case.

Our data suggested that conventional treatments were not effective for almost patients with regional lymph node metastasis. However in the patients with low grade, under pT3 and M0, pN1-2, the possibility of long time survival was shown.

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