Systemic Amyloid A Amyloidosis Secondary to Xanthogranulomatous Pyelonephritis.

The combination of systemic amyloid A (AA) amyloidosis and xanthogranulomatous pyelonephritis (XGP) resulting from a chronic urinary tract infection is extremely rare. We herein report a case of systemic AA amyloidosis secondary to XGP for which clinical remission developed after nephrectomy. To our knowledge, this is the first case report describing the clinical improvement of systemic AA amyloidosis secondary to XGP after nephrectomy in Japan. Clinicians should be aware of this uncommon combination and search for amyloid depositions in cases of XGP.

Relapsed urachal carcinoma responding to first-line chemotherapy with capecitabine-oxaliplatin plus bevacizumab.

Introduction: Advanced urachal carcinoma has a poor prognosis; however, a standard systemic treatment has not been established. We treated a patient with relapsed urachal carcinoma with capecitabine-oxaliplatin plus bevacizumab, a standard regimen for colon cancer, and obtained favorable responses. Case presentation: A 47-year-old woman presented with hematuria. Under the diagnosis of non-metastatic urachal carcinoma, an extended partial cystectomy was performed. Histopathological examination revealed adenocarcinoma with negative surgical margins and lymph nodes. Thirty-two months postoperatively, lung metastases and local recurrence were confirmed, along with elevated carcinoembryonic antigen levels, and nine chemotherapy cycles were administered. Subsequently, the recurrent lesion regressed, and tumor marker levels normalized. Fourteen months after treatment discontinuation, the disease remained stable without progression. Conclusion: This is the first report of advanced urachal carcinoma treated with capecitabine-oxaliplatin plus bevacizumab, demonstrating the potential of this treatment as first-line chemotherapy for this disease.

Kinetics of serum total and free prostate-specific antigen (PSA) after extended multisite prostate biopsy: Comparison among biopsy, transurethral resection of the prostate (TURP), and biopsy plus TURP.

BACKGROUND: The kinetics and reproducibility of serum prostate-specific antigen (PSA) following extended multisite biopsies are unknown. The aim of this study was to examine the kinetics of hematogenous leakage of PSA molecules by comparing the postintervention PSA manner among extended biopsies, transurethral resection of the prostate (TURP) and biopsy plus TURP. METHODS: Total and free PSA values were examined before and sequentially after intervention (at 1 hour, 24 hours, 2 days, 14 days, and 28 days), in patients who underwent 14-core prostate biopsy (Biopsy, n = 53), TURP (TURP, n = 21), or prostate biopsy plus TURP (Biopsy+TURP, n = 18). RESULTS: Ten patients in the Biopsy group were histologically diagnosed as having prostate cancer, and all other patients were diagnosed with non-malignant disorders. One hour after intervention, the increase in total PSA in the Biopsy group (mean 19.58 +/- 24.78-fold) and Biopsy+TURP group (mean 14.00 +/- 10.52-fold) was higher than that of the TURP group (mean 6.189 +/- 7.567-fold) (P = 0.0207 and 0.0119, respectively). The increase in total PSA in the Biopsy+TURP group was not different from that of the Biopsy group. The increase in free PSA in the Biopsy group (mean 36.52 +/- 21.18-fold or more) was greater than that of the TURP group (mean 15.57 +/- 18.17-fold) (P = 0.0002 or less). Both total and free PSA values in the Biopsy group recovered to the initial levels 28 days after intervention (P = 0.380 and P = 0.0873, respectively). The course of both total and free PSA values in the Biopsy group was not different between prostate cancer and non-malignant disorders. CONCLUSIONS: Extended multisite biopsies caused marked elevation of both total and free PSA compared with ordinary sextant protocol or TURP, and they reduced to the preoperative levels in about 4 weeks. The postintervention increase of PSA and its manner of recovery were comparable between the Biopsy and Biopsy+TURP groups, suggesting that the hematogenous leakage of PSA by biopsies occurs in an early phase just after biopsy and rapidly reduces also in the early phase.

Idiopathic retroperitoneal fibrosis associated with IgG4-positive-plasmacyte infiltrations and idiopathic chronic pancreatitis.

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 case of gas gangrene caused by colon diverticulitis with perforation into the retroperitoneal space].

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.

[Bilateral pheochromocytomas and asynchronous bilateral renal cell carcinomas associated with von Hippel-Lindau disease: a case report].

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.