Sarcoidosis-associated renal AA amyloidosis and crescentic necrotizing glomerulonephritis.

Sarcoidosis has a rare and independent association with renal AA amyloidosis and crescentic necrotizing glomerulonephritis. However, coexisting entities in sarcoidosis have not been previously described. Herein, we report a 66-year-old Caucasian woman who presented with generalized fatigue, weight loss, and acute kidney injury in the setting of likely sarcoidosis.

Histologic acute graft pyelonephritis after kidney transplantation: Incidence, clinical characteristics, risk factors, and association with graft loss.

Background: Histologic acute graft pyelonephritis (HAGPN) after kidney transplantation (KT) has been assessed less frequently than urinary tract infections (UTIs) or clinical acute graft pyelonephritis. Risk factors for HAGPN, its association with graft loss, and measures that might prevent it are not known.

Methods: We performed a retrospective review of HAGPN cases identified from KT occurring between January 2008 and December 2017 at our institution.

Minimal change disease and subacute interstitial nephritis in association with gastroenteritis following oyster consumption.

() is a gram-negative, facultatively anaerobic bacillus that is associated with gastroenteritis and a host of other extra-intestinal manifestations in humans. However, its impact on the kidneys is unclear. Most literature that has explored this association involves fish, marine life in which inhabits.

Mucinous Tubular and Spindle Cell Carcinoma of the Kidney with Nodal Metastasis Managed with Surgical Resection.

Mucinous tubular and spindle cell carcinoma (MTSCC) is a rare renal malignancy that usually follows an indolent course. The few existing reports of metastatic MTSCC show poor response to systemic therapy. Here we describe the unusual case of a 39-yr-old male with MTSCC presenting as a large renal mass with bulky retroperitoneal lymphadenopathy managed with complete resection of disease.

Fibrillary glomerulonephritis or complement 3 glomerulopathy: a rare case of diffuse necrotising crescentic glomerulonephritis with C3-dominant glomerular deposition and positive DNAJB9.

Fibrillary glomerulonephritis (FGN) and complement 3 glomerulopathy (C3G) are rare forms of glomerulonephritis with distinct aetiologies. Both FGN and C3G can present with nephritic syndrome. FGN is associated with autoimmune disease, dysproteinaemia, malignancy and hepatitis C infection.

Prosthetic valve endocarditis from infection causing granulomatous interstitial nephritis.

is a rare infection associated with cardiopulmonary bypass. We describe a case of granulomatous interstitial nephritis caused by in a patient with prosthetic aortic valve endocarditis. A 63-year-old female with a mechanical aortic valve replacement developed fatigue, 20 lbs.

Successful lung transplantation from a donor with lung and ovarian masses.

Declining a donor when there is a reasonable possibility that the abnormality on chest imaging could be benign carries the risk of losing out on potentially usable lungs in an already parched landscape of donor organ availability. Cautiously aggressive attitudes to acceptance of borderline donors can help bridge the significant discrepancy that exists between the demand and availability of donor organs. Herein, we present a case highlighting successful bilateral lung transplantation from a relatively imperfect donor.

Arterial Blood Pressure at Liver Transplant Evaluation Predicts Renal Histology in Candidates With Renal Dysfunction.

Renal dysfunction is common in liver transplantation (LT) candidates, but differentiating between reversible and irreversible renal injury can be difficult. Kidney biopsy might be helpful in differentiating reversible from irreversible renal injury, but it is associated with significant complications. We aimed to identify pre-LT predictors of potentially reversible renal injury using histological information obtained on pre-LT renal biopsy.

Cystic Fibrosis-Associated Liver Disease in Lung Transplant Recipients.

Cystic fibrosis (CF) is an autosomal recessive disease characterized by mutations in the gene that encodes for the cystic fibrosis transmembrane conductance regulator protein (CFTR). CFTR gene mutations manifest as epithelial cell dysfunction in the airways, biliary tract, pancreas, gut, sweat glands, paranasal sinuses, and genitourinary tract. The clinical manifestations of this dysfunction include respiratory tract infections, bronchiectasis, pancreatic insufficiency, malabsorption, intestinal obstruction, liver disease, and male infertility.

A novel variant in in a family with fibronectin glomerulopathy.

Glomerulopathy with fibronectin deposits (GFND) is a rare glomerular disorder. We report a 28-year-old male diagnosed with GFND by mass spectrometry on kidney biopsy tissue. Whole-exome sequencing (WES) identified that a previously undescribed gene mutation (c.

A method to reduce variability in scoring antibody-mediated rejection in renal allografts: implications for clinical trials - a retrospective study.

Poor reproducibility in scoring antibody-mediated rejection (ABMR) using the Banff criteria might limit the use of histology in clinical trials. We evaluated the reproducibility of Banff scoring of 67 biopsies by six renal pathologists at three institutions. Agreement by any two pathologists was poor: 44.

Karyomegalic interstitial nephritis in a renal allograft.

Karyomegalic interstitial nephritis (KIN) is a rare renal interstitial disease entity characterized by large tubular nuclei, accompanied by interstitial inflammation, tubular atrophy, and interstitial fibrosis. Approximately 50 cases of KIN have been described in the native kidney. In this case study, we describe the first case of KIN in a kidney allograft.

Primary synovial sarcoma of the kidney-A case report and literature review.

Primary synovial sarcoma of the kidney is a very rare spindle cell neoplasm that occasionally displays epithelial differentiation. It occurs between 15-60 years of age with a mean of 35 years and a slight male predilection. Most of synovial sarcomas appear as relatively nonspecific soft tissue masses involving the kidney.

Behçet's syndrome and focal segmental glomerulosclerosis with nephrotic syndrome - successful treatment with etanercept .

Behçet's syndrome (BS) often presents with aphthous and genital ulcers, uveitis, and erythema nodosum. Renal involvement has been reported with most cases presenting with renal amyloidosis, IgA nephropathy, or crescentic glomerulonephritis. We describe a case of a 49-year-old woman with relapsing BS symptoms coinciding with new-onset development of nephrotic syndrome.

Criteria for Risk Stratification of Vulvar and Vaginal Smooth Muscle Tumors: An Evaluation of 71 Cases Comparing Proposed Classification Systems.

Accurate risk stratification of smooth muscle tumors (SMTs) is essential for appropriate patient management. Yet, the rarity of SMTs of the vagina and vulva makes development of a prognostically meaningful classification system challenging. While 2 classification methods for vulvar SMTs and 1 for vaginal SMTs have been proposed, it is our experience that many pathologists tend to apply criteria for uterine SMTs when evaluating vulvovaginal tumors.

Recurrent myxoinflammatory fibroblastic sarcoma: a case report.

Myxoinflammatory fibroblastic sarcoma is a rare sarcoma which typically presents in the extremities and is treated by definitive surgery. In recurrent disease, the reported utilization of radiotherapy is increasing, and more modern techniques such as intensity-modulated radiotherapy may be improving long-term outcomes.

Langerhan's Cell Sarcoma: Two Case Reports.

Langerhan's cell sarcoma (LCS) is a rare neoplasm with a poor prognosis. To our knowledge, only sixty-six cases have been published. We discuss two patients who presented very differently with LCS, as well as a recently published review of all sixty-six cases.

Tumor induced osteomalacia secondary to anaplastic thyroid carcinoma: A case report and review of the literature.

Context: Tumor induced osteomalacia related to anaplastic thyroid cancer has never been reported.

Objective: We describe a case of tumor induced osteomalacia (TIO) in a patient with a fibroblast growth factor 23 (FGF-23) secreting anaplastic thyroid carcinoma. The current imaging modalities are reviewed.

Clinical Activity of Pazopanib in Metastatic Extraosseous Ewing Sarcoma.

We report a response to pazopanib in a 69-year-old man with heavily pre-treated metastatic extraosseous Ewing sarcoma in addition to molecular profiling of his tumor. To our knowledge, this case is the earliest to demonstrate activity of an oral multi-targeted kinase inhibitor in Ewing sarcoma. This case provides rationale for adding a Ewing sarcoma arm to SARC024, a phase II study of regorafenib, another multi-targeted kinase inhibitor, in patients with liposarcoma, osteosarcoma and Ewing and Ewing-like sarcomas (NCT02048371).

Trisacryl Gelatin Microembolism and Metastases in the Lung after Renal Artery Embolization and Nephrectomy for Renal Cell Carcinoma.

This is the first report, to our knowledge, of widespread, histologically confirmed trisacryl gelatin pulmonary microembolism after renal artery embolization (RAE). In addition, this is the first report of lung involvement by both metastatic renal cell carcinoma (RCC) and an embolic agent used for RAE. The patient was a 63-year-old woman who recently presented with both dyspnea on exertion and productive cough.

Renal outcomes of liver transplant recipients who had pretransplant kidney biopsy.

Background: Kidney biopsy has been recommended to guide kidney allocation in selected liver transplant (LT) candidates with renal dysfunction. However, post-LT-alone renal outcomes in recipients who showed evidence of reversible renal injury and limited chronicity on pre-LT kidney biopsy are unclear.

Methods: Renal outcomes of 41 LT recipients who had pre-LT kidney biopsy for unexplained renal dysfunction, proteinuria, and hematuria were retrospectively reviewed.