A rare case of combined placental site trophoblastic tumour with mature cystic teratoma and mixed germ cell tumour in the testis.

A 20-year-old male presented with persistent right testicular pain. Following ultrasound detection of testicular nodules and biopsy for intraoperative consultation which yielded germ cell tumour, he underwent radical orchidectomy. A predominantly whitish cyst and a lobulated, variegated nodule were identified. Histology showed a mature cystic teratoma with a focus of infiltrative epithelioid cells containing eosinophilic cytoplasm and pleomorphic nuclei, invading ectatic vessel wall associated with fibrinoid change. These cells were positive for cytokeratin, human placental lactogen and inhibin, while negative for Melan-A, p63 and alpha-fetoprotein, consistent with placental site trophoblastic tumor (PSTT). The variegated nodule was a mixed germ cell tumour composed of embryonal carcinoma and immature teratoma. Aside from choriocarcinoma, primary trophoblastic tumors such as PSTT, which are derived from intermediate trophoblasts, are extremely rare in the testis. Aside from a case of pure testicular PSTT, 2 other cases have been described in association with germ cell tumour, of which one is a mature teratoma with PSTT that demonstrated gain of chromosome 12p. The other presented with PSTT in retroperitoneal recurrence of a testicular mixed germ cell tumour. We discussed the features of this tumour in the testis and important differentials in its diagnosis.

Epstein-Barr virus-associated smooth muscle tumors after kidney transplantation: treatment and outcomes in a single center.

BACKGROUND: Epstein-Barr virus-associated smooth muscle tumors (EBV SMT) in adult kidney transplant recipients (KTR) are rare. The aims of this study are to document the clinical features, types of treatment given, and outcomes of KTR with EBV SMT in our institution. METHODS: Sixteen patients were identified from our institution's databases. Patients' survival, tumor outcome, and graft survival were compared between patients who remained on cyclosporine-based immunosuppressant and those who converted to sirolimus-based therapy. RESULTS: The median time of diagnosis was 9.4 yr after kidney transplantation, and majority of the patients had multifocal disease at the time of diagnosis. Overall, the patient survival rate was 75% over a mean follow-up period of five yr. Two patients with non-functioning allograft at the time of diagnosis of EBV SMT were excluded from the treatment outcome analysis. Comparing the sirolimus (n = 7) vs. cyclosporine groups (n = 7), patient survival rate was 100% vs. 42.9% (p = 0.08), graft survival 71.4% vs. 28.7% (p = 0.53), and disease-free status 42.9% vs. 14.3% (p = 0.73), respectively. CONCLUSION: Surgical resection in combination with decreasing immunosuppression or conversion to sirolimus appears to be effective in the treatment of EBV SMT in KTR.

Pseudotuberculous Granulomatous Pyelitis.

We report a case of pseudotuberculous granulomatous pyelitis in an elderly female patient with hydronephrotic right kidney secondary to obstructing urinary stone. Pseudotuberculous granulomatous pyelitis is a rarely reported entity, characterized by severe granulomatous inflammation limited predominantly to the renal pelvis. It is associated with urinary (pelvicalyceal) obstruction, urolithiasis well as non- Mycobacterial urinary tract infection.

Negative regulatory responses to metabolically triggered inflammation impair renal epithelial immunity in diabetes mellitus.

Diabetes mellitus is characterized by chronic inflammation and increased risk of infections, particularly of tissues exposed to the external environment. However, the causal molecular mechanisms that affect immune cells and their functions in diabetes are unclear. Here we show, by transcript and protein analyses, signatures of glucose-induced tissue damage, chronic inflammation, oxidative stress, and dysregulated expression of multiple inflammation- and immunity-related molecules in diabetic kidneys compared with non-diabetic controls. Abnormal signaling involving cytokines, G-protein coupled receptors, protein kinase C isoforms, mitogen-activated protein kinases, nuclear factor-κB (NFκB), and Toll-like receptors (TLR) were evident. These were accompanied by overexpression of negative regulators of NFκB, TLR, and other proinflammatory pathways, e.g., A20, SOCS1, IRAK-M, IκBα, Triad3A, Tollip, SIGIRR, and ST2L. Anti-inflammatory and immunomodulatory molecules, e.g., IL-10, IL-4, and TSLP that favor TH2 responses were strongly induced. These molecular indicators of immune dysfunction led us to detect the cryptic presence of bacteria and human cytomegalovirus in more than one third of kidneys of diabetic subjects but none in non-diabetic kidneys. Similar signaling abnormalities could be induced in primary human renal tubular epithelial (but not mesangial) cell cultures exposed to high glucose, proinflammatory cytokines and methylglyoxal, and were reversed by combined pharmacological treatment with an antioxidant and a PKC inhibitor. Our results suggest that diabetes impairs epithelial immunity as a consequence of chronic and inappropriate activation of counter-regulatory immune responses, which are otherwise physiological protective mechanisms against inflammation. The immune abnormalities and cryptic renal infections described here may contribute to progression of diabetic nephropathy.

Papillary renal cell carcinoma with metastatic laparoscopic port site and vaginal involvement: a case report.

INTRODUCTION: Laparoscopic port-site metastasis is a rare but well recognized outcome following surgery in urological cancers, with its etiology not clearly understood. Additionally, vaginal metastasis in clear cell renal cell carcinoma is rare, and has not been previously reported in the setting of papillary renal cell carcinoma. CASE PRESENTATION: We present the case of a 71-year-old Chinese woman with metastatic type II papillary renal cell carcinoma with histologically verified vaginal involvement and a concurrent laparoscopic port-site metastasis. This was also associated with a unique constellation of widely disseminated metastatic sites, which include a local relapse, the peritoneum and the urethra. CONCLUSION: Laparoscopic port-site metastases are associated with the presence of advanced cancer with multiple sites of metastasis. We hypothesize from the findings of our report and background data that this phenomenon is more likely to be related to tumor factors rather than operative factors. We also present what is, to the best of our knowledge, the first reported case in the literature of vaginal and urethral metastasis and the second reported case of laparoscopic port-site recurrence.

Glomerular basement membrane thickness in an Asian population using a novel image analysis software.

AIMS: We aimed to develop an image analysis software that enabled measurement of glomerular basement membrane (GBM) thickness. METHODS: With this software, we evaluated the range of GBM widths found in a cohort of Asian patients diagnosed with a spectrum of renal diseases including minimal change/IgM nephropathy, focal and segmental glomerulosclerosis, IgA nephropathy, systemic lupus erythematosus nephritis, diabetic nephropathy, pauci-immune crescentic glomerulonephritis, thin basement membrane disease, and tubulointerstitial nephritis. Measurements were taken from a minimum of five glomerular capillary loops of each glomerulus. For each loop, at least 10 different points of the GBM were measured. RESULTS: The average GBM width measured for minimal change disease was 347.4 +/- 9.0 nm, with the highest value being 403.9 nm and lowest being 214.7 nm. No association was found between GBM width and gender. We found a significant increase in GBM width in pathological states like lupus nephropathy (p < 0.0001), diabetic nephritis (p < 0.001) and tubulointerstitial nephritis (p < 0.01) as compared with minimal change disease. Only one case of thin membrane nephropathy (198.7 nm) was available for analysis and we found a significant thinning of the GBM. CONCLUSIONS: These observations provide insights into the range of GBM thickness in several disease states and support the use of this novel software in the daily diagnostic laboratory setting.

A case of adrenal cavernous hemangioma presenting with progressive enlargement and apparent hormonal hypersecretion.

OBJECTIVE: To report the case of a man with an adrenal cavernous hemangioma presenting as a progressively enlarging adrenal mass with apparent hormonal hypersecretion. METHODS: We report the clinical, laboratory, imaging findings, and clinical course of this patient, and we highlight the important atypical features of this case. The literature is reviewed for the typical presentations of adrenal cavernous hemangiomas. RESULTS: A 59-year-old man presented with an adrenal incidentaloma that had an imaging phenotype suggestive of a pheochromocytoma or an adrenal carcinoma. The hormonal profile also suggested a state of aldosterone and catecholamine hypersecretion. Surgery, however, proved the diagnosis to be an adrenal cavernous hemangioma. CONCLUSION: Although adrenal cavernous hemangioma is a rare entity, it should be considered in the differential diagnosis of an adrenal incidentaloma. Its radiologic features are not specific, and the presence of hormonal hypersecretion does not exclude the diagnosis.

Synchronous pancreatic serous cystic tumor, intraductal papillary mucinous tumor and gastric carcinoma: report of a case.

Synchronous cystic tumors of the pancreas are rarely reported in the literature. We report an unusual case of synchronous pancreatic serous cystic tumor (SCT) and intraductal pancreatic mucinous tumor (IPMT) with concomitant gastric carcinoma. This study highlights the importance of careful intra-operative and pathologic examination for concomitant pancreatic neoplasms.