Extrarenal Lesions Research Articles

Evaluation of PET/CT imaging with [89Zr]Zr-DFO-girentuximab: a phase 1 clinical study in Japanese patients with renal cell carcinoma (Zirdac-JP).

PET/CT imaging with Zirconium-89 labeled [89Zr]Zr-DFO-girentuximab, which targets tumor antigen CAIX, may aid in the differentiation and characterization of clear cell renal cell carcinomas (RCC) and other renal and extrarenal lesions, and has been studied in European and American cohorts. We report results from a phase I study that evaluated the safety profile, biodistribution, and dosimetry of [89Zr]Zr-DFO-girentuximab in Japanese patients with suspected RCC. Eligible adult patients received 37MBq (± 10%; 10mg mass dose) of intravenous [89Zr]Zr-DFO-girentuximab. Safety and tolerability profile was assessed based on adverse events, concomitant medications, physical examination, vital signs, hematology, serum chemistry, urinalysis, human anti-chimeric antibody measurement, and 12-lead electrocardiograms at predefined intervals. Biodistribution and normal organ and tumor dosimetry were evaluated with PET/CT images acquired at 0.5, 4, 24, 72h and Day 5±2d after administration. [89Zr]Zr-DFO-girentuximab was administered in six patients as per protocol. No treatment-emergent adverse events were reported. Dosimetry analysis showed that radioactivity was widely distributed in the body, and that the absorbed dose in healthy organs was highest in the liver (mean±standard deviation) (1.365±0.245mGy/MBq), kidney (1.126±0.190mGy/MBq), heart wall (1.096±0.232mGy/MBq), and spleen (1.072±0.466mGy/MBq). The mean effective dose, adjusted by the radioactive dose administered, was 0.470mSv/MBq. The radiation dose was highly accumulated in the targeted tumor, while any abnormal accumulation in other organs was not reported. This study demonstrates that [89Zr]Zr-DFO-girentuximab administered to Japanese patients with suspected RCC has a favorable safety profile and is well tolerated and has a similar dosimetry profile to previously studied populations.

Primary Kidney Lymphoma in a Dog

Background: Lymphoma is a malignant lymphoid tumor originating in the lymph nodes or other solid organs and comprises 90% of all hematopoietic tumors in dogs. However, primary renal lymphoma is rare and is associated with nonspecific clinical signs. Tumor invasion in both kidneys can cause severe clinical signs due to renal failure, complicating the patient's treatment and prognosis. The aim of this case was to report the case of a dog affected by bilateral primary renal lymphoma. In addition, to characterize the clinical and histopathological presentation due to the intense morphological changes. Case: A 5-year-old male Poodle canine was admitted showing apathy and emesis for 5 days. On physical examination, the dog showed 10% of dehydration, reddish oral mucous membranes, poor body condition (score 1/5), uremic breath, and pain in the kidney area. Complementary tests revealed severe low white blood cells count, high BUN levels, high levels of potassium, calcium, and phosphorus (serum biochemistry). Abdominal ultrasound showed bilateral kidney enlargement. Fine needle aspiration of the mass (guided by ultrasound) revealed round cell tumor. Radiographs showed no alterations. The dog died due to his poor condition and necropsy was performed. On post-mortem examination, the kidneys were both enlarged, pale, and with an irregular subcapsular surface. The histopathological diagnostic was primary renal lymphoma. Immunohistochemical staining revealed that neoplastic cells were strongly positive for anti CD20 and PAX5, while negative for CD3, supporting the diagnosis of B-cell lymphoma. Discussion: The diagnosis was based on clinical, complementary tests, fine needle aspiration, histopathological and immunohistochemical findings. In dogs, primary kidney tumors are uncommon and usually malignant. The presence of vomiting, uremic breath, dehydration, weight loss, and erosive and ulcerative lesions on the tongue (uremic glossitis) are clinical signs of chronic renal failure, and this condition was later confirmed by laboratory tests and histopathological findings. Dogs diagnosed with extra-nodal renal lymphoma, present clinical signs such as polydipsia, polyuria, vomiting, and uremic breath in some cases. These changes are compatible with changes observed in cases of renal failure. In this case, the severe azotemia, hyperphosphatemia, hypocalcemia, and hyperkalemia were due to the neoplastic infiltration in both kidneys. Additionally, the abdominal ultrasound revealed the tumor in both kidneys. Almost 38% of dogs with renal lymphoma presented in urine evaluation normal urine density and a large amount of protein in the urine, similar to those observed in this dog. When the lesions are on both kidneys, kidney failure develops and uremic extra-renal lesions appear, as observed in this case. The prevalence of primary kidney tumors in domestic animals corresponds to less than 1% of the total of the tumors reported, and they are usually in one kidney. In dogs, almost 60 - 70% of lymphomas are B cells, 30 - 40% are T cells, and less than 1% are null cells. B-cell lymphomas usually show less aggressive behavior when compared to T-cell lymphomas. Kidney lymphoma can be included as an important cause of kidney failure, and has slow and progressive development, making early diagnosis and treatment difficult. Keywords: dog disease, lymphoproliferative disorder, renal neoplasm, uremia. Título: Linfoma renal primário em cãoDescritores: doença de cão, distúrbio linfoproliferativo, neoplasma renal, uremia.

ANCA-associated nephritis without crescent formation has atypical clinicopathological features: a multicenter retrospective study

Although crescentic glomerulonephritis is a hallmark of ANCA-associated nephritis, the clinicopathological features of ANCA-associated nephritis without crescent formation remain to be elucidated. We enrolled 146 Japanese ANCA-associated vasculitis (AAV) patients subjected to renal biopsy in 16 hospitals from 2001 to 2018, and compared those with and without crescent formation (C + and C- groups). The primary endpoint was end-stage renal disease (ESRD) and/or death. C- group comprised 25 (17.1%) subjects. They had better renal function at the time of renal biopsy [estimated glomerular filtration rate (eGFR); median 41.7 vs 27.5ml/min/1.73m2, p < 0.01] with minor urinary abnormalities but had a higher serum C-reactive protein level (8.8 vs 5.4mg/dl, p = 0.01) and frequency of extra-renal lesions of AAV (76.0% vs 48.8%, p = 0.02) than C + group. Pathologically, C- group had a higher frequency of arteritis (40.0% vs 16.5%, p < 0.01). Kaplan-Meier method with log-rank tests showed no significant difference in renal and life prognosis combined, regardless of crescent formation. Multivariate Cox regression analysis revealed baseline eGFR, sclerotic class, and extra-renal lesions to be risk factors of ESRD and death combined. Competing risk analysis showed baseline eGFR and sclerotic class to be associated with ESRD, whereas baseline eGFR and extra-renal lesions were associated with death. ANCA-associated nephritis without crescent formation had different clinicopathological features from those with crescent formation, suggesting an atypical subtype of ANCA-associated nephritis. Despite the better renal function at the time of renal biopsy, these results suggest that this subtype requires especially careful attention, especially in the presence of extra-renal involvement.

SO058IGG4-RELATED KIDNEY DISEASE : A FRENCH NATIONWIDE RETROSPECTIVE COHORT STUDY

Abstract Background and Aims IgG4 disease is a systemic fibroinflammatory disorder that may affect virtually any organ. IgG4-related kidney disease (IgG4-RKD) is a major manifestation, occurring in almost one third of patient. The present study addresses the diagnosis, management and outcome of French patients with IgG4-RKD. Method We conducted a retrospective observational study including patients with renal impairment due to IgG4-RKD from 32 centers. Clinical, biological, and imaging data, as well as management modalities and outcome, were retrieved from medical records. Results 74 patients, 64 males and 10 females, were diagnosed with IgG4-RKD between 1997 and 2019 and were included in the present study. The mean age at diagnosis was 65.2 ± 15-year-old. Renal involvement was present at diagnosis in 63% of cases. Nine (12%) patients had isolated renal involvement, whereas extra-renal involvement included retroperitoneal fibrosis (20%), auto-immune pancreatitis (49%), cholangitis (27%), and lung disease (22%). Renal imaging lesions were observed in 48 (64%) patients, and included renal hypertrophy, patchy lesions and pseudo-tumor solitary renal lesion. PET-CT scan was performed in 47 (65%) patients and showed hypermetabolic renal lesion(s) in 36% of cases. The presence of extra-renal hypermetabolic lesions was observed in 35 cases (74%). 43 %, 25% and 14% of patients presented with AKI, AKI-on-CKD, and isolated CKD, respectively. The mean serum creatinine level at diagnosis was 244 ± 140 µmol/L, corresponding to an eGFR of 69 ± 22ml/min/1.73m. Urinary sediment was most often bland. Mean urine protein-to-creatinine ratio was 1.27 g/g. of note, 26% of patients had more than 1 g/g. 82% of patients underwent a kidney biopsy showing tubulointerstitial involvement in all cases, 12 (16%) had additional glomerular involvement, mainly membranous nephropathy. The major additional laboratory abnormalities included polyclonal hypergammaglobulinemia, and increased serum IgG4 increase in 83% and 86%, respectively. Complement levels were decreased in 27 (37%) patients. Two patients were lost for follow-up and 69 patients were treated with corticosteroid therapy in 66 (89%) patients and 10 received Rituximab as first line therapy. The mean duration of follow-up was 28.8 ± 30 months. During follow-up, 22 (30%) patients relapsed, while 45 (62%) patients had persistent CKD at last follow-up, with a mean eGFR of 47±27 ml/min/1.73m. Seven (9%) patients progressed to ESRD and 7 died. A significant response was achieved in 46 (62%) patients. Patients who received steroids higher than 0.5mg/kg/day had lower serum IgG4 levels (1.8 g/l versus 5.7 g/l, p=0.007) at last follow up but there was no significant difference observed in terms of renal function between the two groups (45.5 vs 48.8 ml/min/1.73m, p= 0.72). By univariate analysis, predictors of relapse were low C3 or C4 serum level (p=0.01), ANCA positivity (p= 0.02), renal radiological lesions (p=0.05). At last follow-up the eGFR was worse in patients with a serum creatinine diagnosis greater than 300 µmol/L (30.25 vs 57 ml/min/1.73m p=0.0001) and more progressed to ESRD (p=0.03). Conclusion IgG4-RKD has been recently described and may affect middle-aged males. The disease presents as tubulointerstitial nephritis with glomerular involvement in 16% of cases. Disease response to corticosteroid therapy is favorable but is characterized by a high rate of relapses and a significant risk of persistent CKD in the majority of patient. Risk of ESRD and death in almost 20% of patients.

Renal involvement in IgG4-related disease

IgG4-RD may affect several organs including kidneys. The kidney is involved in approximately 20% of patient with IgG4-RD. The most common intrinsic kidney disease is tubulointerstitial nephritis (IgG4-TIN). Retroperitoneal fibrosis (IgG4-RPF) may induce obstructive acute renal failure. More rarely, IgG4-RKD can manifest as a glomerular disease, in particular as a membranous nephropathy (MN). It mostly affects middle-aged to elderly men and causes acute or chronic renal dysfunction, multiple hypodense lesions on CT-Scan and various extra-renal lesions. Increased serum IgG4 and hypocomplementemia are the most important serological findings for the diagnosis of IgG4-RD and thus should be systematically assessed when IgG4-RKD is suspected. Specific diagnosis criteria for IgG4-TIN including interstitial infiltration of IgG4-positive plasma cells, storiform fibrosis and tubular basement membrane immune complex deposits have been proposed. Corticosteroids are effective and remain the first-line therapy but relapses or severe forms could respond to immunosuppressive therapy.

Lily Poisoning in Domestic Cats

Background: Cases of plant intoxication in small animals are observed frequently in the domestic environment, mainly because most dogs and cats live in households and occasionally have access to streets and rural areas. Among such toxic agents, ornamental plants of the genus Lilium and Hemerocallis, which are potentially nephrotoxic to the feline species, are highlighted. Affected cats start presenting clinical signs 1-6 h after plant ingestion. Renal failure takes place in 12-72 h, and death may occur in an interval ranging from three to seven days. The objective of this article is to describe the epidemiological, clinical and pathological findings of lily (Lilium sp.) poisoning in two cats.Case: The aspects of lily poisoning in two cats are described (cat #1 and cat #2). Cat #1 was a 3-year-old, mixed breed female cat, which presented a clinical history of anorexia, apathy, drooling, vomiting and polydipsia. Serum biochemical analysis revealed creatinine elevation (21.2 mg/dL), as well as hyperphosphatemia (19 mg/dL). Seventy-two h after the onset of clinical signs, renal failure progressed to anuria, followed by death. The second animal of this report (cat #2) was a 2-year-old, mixed-breed male cat. The animal was found dead by the owner without displaying any previous clinical signs. Cats #1 and #2 ingested leaves of lily, which were present in their households as ornamental plants. At necropsy, the kidneys of both cats presented mild enlargement. Moderate perirenal edema was also noted. Cat #1 showed morphologic extrarenal uremic lesions, characterized by ulcers in the oral mucosa and in the margin of the tongue ventral surface. Microscopic lesions observed in both cases were similar and compatible with acute toxic nephropathy. Histologically, severe epithelial cell degeneration and necrosis of proximal and distal convoluted tubules were noted. Other renal microscopic findings included hyaline and granular casts, tubule regeneration and occasional birefringent oxalate crystals. Cat #1 also presented moderate white matter vacuolation in the telencephalon and cerebellum.Discussion: The epidemiologic, clinical and pathological findings reported in the present study are similar to previous descriptions of lily poisoning in cats. Lily poisoning has been described in both males and females, without breed and age predisposition, similarly to what has been found in the present study. Kidney metabolite excretion, including the elimination of molecules such as creatinine, urea, and phosphorus is usually compromised in these cases, which was noted in cat #1. The same animal showed extrarenal manifestations of renal failure, leading to a clinical presentation of uremic syndrome, which is not frequent in these intoxications. Animals intoxicated by lily usually die from renal failure and anuria. In most cases, lesions are restricted to the kidneys. In the reported cases, the microscopical lesions consisted of tubule epithelial cells degenerative changes and necrosis. Acute lily intoxication in cats must be differentiated from other conditions, such as intoxications due to aminoglycoside antibiotics, heavy metals, nonsteroidal anti-inflammatory drugs, antifungal agents, chemotherapeutic drugs, and ethylene glycol. The knowledge regarding the toxic potential of ornamental plants is fundamental in order to prevent such events of intoxication, as well as to reach the final diagnosis. Epidemiological, clinical and pathological findings were essential to conclude the final diagnosis.

Quiz: Acute Kidney Injury in a Patient on a TNF-α Blocking Agent

Quiz: Acute Kidney Injury in a Patient on a TNF-α Blocking Agent

Post Renal Transplant Surgical Complications; MRI Standard Applications and Diagnostic Outcomes

Objective: Fast MR imaging sequences together with paramagnetic contrast agents, offer multiple advantages in the assessment of renal function. It provides cross sectional and vascular information without the risk of ionizing radiation, iodinated contrast or arterial catheterization. Post transplantation complications can be grouped as surgical or medical. Immediate surgical complications include renal artery thrombosis or stenosis, urinary leak or lymphocele. Renal allograft frequently require repeated imaging studies during the immediate post-operative period and various times thereafter, when renal function is compromised. Background: End stage renal disease is common and can result from a variety of diseases. Kidney transplantation from living-related donors offered the best prognosis. Imaging modalities that are currently used to evaluate transplanted kidneys are ultrasound (US), computed tomography (CT), scintigraphy, intravenous urography (IVU), contrast angiography, and magnetic resonance imaging (MRI). Methods: This study was conducted on 181 renal transplant recipients. Recipients were 139 males and 42 females. Their age ranged from 20 to 58 years (mean age 39 years). The patients underwent clinical assessment, Laboratory investigations, and different Radiological imaging procedures as: I- Gray scale and color Doppler ultrasonography. II- Magnetic Resonance Imaging. 3D Gd-enhanced MRA. MR Urography. Selective IA-DSA of the graft artery. III- Percutaneous catheter nephrostomy (PCN) and antegrade pyelography. IV- Radio-isotope diuretic renogram using 99m Tc-MAG3. Results: 30 renal transplants were examined by MRI in the 1st 2 weeks after renal transplantation. At the end of 1st 2 weeks, MR examinations were carried out, as basal studies (including MRI, MRA and MRU) for 98 transplants. From this group, 64 transplants were subjected to other MR examinations. After the 1st 2 weeks, 53 transplants were subjected to MR examinations for the 1st time at variable post-transplant duration. Among the studied 181 renal transplants, MR examinations detected 3 cases with graft arteries thrombosis (1.6%), 10 with graft arteries stenosis (5.5%), 6 with segmental infarctions (3.3%), 3 cases with graft intrarenal arteries pseudo-aneurysms (1.6%) and 2 cases with arterio- venous fistulae (1.1%) after graft biopsies. Conclusion: MRI is highly recommended to evaluate intra-/extra-renal graft vascular lesions, urinary obstructive syndrome, compressive collections (urinoma, lymphocele), inflammatory and tumoral lesions of the renal graft.

Present Status and Advances in Autosomal Dominant Polycystic Kidney Disease

Autosomal dominant polycystic kidney disease (ADPKD) is the most common genetic disorder of the kidney. The mainly clinical manifestation is progressive cyst formation in bilateral kidneys, impairs normal renal parenchyma, and ultimately results in end-stage renal disease (ESRD). Extra-renal lesions include cystic liver or pancreas, brain aneurysm and cardiovascular defects. Mutations in either PKD1 or PKD2 genes result in the disease, but the former develops more severe clinical phenotypes than the latter. Currently, ADPKD still challenge vast clinicians on account of lacking effective and less side-effect therapy. Intensive study of molecular mechanism of the disease can establish theoretical basis for potential therapeutic targets and guide clinicians to develop new approaches in treatment of this disease. This review will focus on current advances of ADPKD pathogenesis which may benefit the translational therapy and precision medicine for the disease treatment.

Management of anesthesia in henoch schonlein purpura: a case report /Henoch schonlein purpurasinda anestezi yonetimi: olgu sunumu

Henoch schonlein purpura (HSP) is the most common kind of acute small-vessel vasculitis especially affecting children. It is initiated by deposition of immune complexes as responses to infections such as group A streptococci, mycoplasma, Epstein -Barr, and Varicella zoster. HSP is characterized as a systemic vasculitis involving skin, gut, kidneys and joints. Skin rashes, arthritis, abdominal pain and nephritis are the main clinical features. HSP is a medical disease and requires supportive treatment. Treatment is symptomatic. Immunosupression is another treatment modality. It may be benefical on extrarenal lesions but controlled trials are needed to show the efficacy of the immunosupressive treatment. The long term morbidity depends on the renal and neurological involvement. We present a 36- year old man who was a known case of HSP underwent a right ureter stone surgery. He was stable both intraoperative and postoperative periods. We wanted to underline the management of anesthesia in HSP patients.

Dioctophyma renale em 28 cães: aspectos clinicopatológicos e ultrassonográficos

RESUMO A infecção em cães por Dioctophyma renale, relatada em diversas partes do mundo, é considerada incomum, na maioria das vezes. No entanto, em algumas regiões são descritos números crescentes da infecção e muitos dados da epidemiologia e do ciclo biológico do parasito ainda são obscuros. Dessa forma, o trabalho tem como objetivo descrever os aspectos epidemiológicos, clinicopatológicos e ultrassonográficos de casos de infecção por Dioctophyma renale em cães na região da Fronteira Oeste do Rio Grande do Sul. Foram estudados 28 casos de dioctofimose em cães necropsiados ou clinicamente avaliados, submetidos à ultrassonografia e cirurgia para retirada dos parasitos. Os cães errantes foram os mais acometidos e todos com possível acesso às margens do Rio Uruguai. As lesões renais e extrarrenais foram caracterizadas predominantemente por atrofia do parênquima renal com glomerulonefrite esclerosante e peritonite granulomatosa associada a parasitos adultos livres na cavidade abdominal e ovos, bem como migrações erráticas para o tecido subcutâneo. Por fim, os achados ultrassonográficos corresponderam, especialmente, a imagens transversais circulares de até 0,6 cm de diâmetro, com margem hiperecoica e centro hipoecoico. Esses achados foram patognomônicos para infecção por Dioctophyma renale, e o exame ultrassonográfico se mostrou indispensável para o diagnóstico definitivo durante a avaliação clínica. Os achados observados nesse estudo demonstram a importância dessa parasitose na região. Além disso, alertam para a importância do diagnóstico, que vem sendo subestimado, além de apontar a necessidade de mais dados acerca da epidemiologia da doença para que se chegue a métodos efetivos de controle.

Epidemiologia e distribuição de lesões extrarrenais de uremia em 161 cães

Resumo: Com o objetivo de determinar a epidemiologia e as características morfológicas, incluindo a localização anatômica, das lesões extrarrenais de uremia, bem como determinar as principais lesões do sistema urinário associadas à ocorrência de uremia, foram revisados os protocolos de necropsias de cães realizadas no Laboratório de Patologia Veterinária da Universidade Federal de Santa Maria de janeiro de 1996 a dezembro de 2012 (17 anos). Nesse período foram necropsiados 4.201 cães, sendo que 161 (3,8%) apresentaram lesões extrarrenais de uremia. Em 134 cães (83,2%) foram descritos sinais clínicos associados à uremia. As lesões extrarrenais mais frequentes, em ordem decrescente, foram: gastrite ulcerativa e hemorrágica (56,5%), mineralização de tecidos moles (55,9%), edema pulmonar (47,2%), estomatite e/ou glossite ulcerativa (30,4%), endocardite/trombose atrial e aórtica (28,6%), hiperplasia das paratireoides (9,3%), osteodistrofia fibrosa (8,1%), anemia (6,2%), laringite ulcerativa (5%), enterite ulcerativa/hemorrágica (3,7%), esofagite fibrinonecrótica (1,9%) e pericardite fibrinosa (1.9%). Na maioria dos casos as lesões extrarrenais de uremia foram decorrentes de azotemia prolongada por lesões renais graves, sendo as mais prevalentes a nefrite intersticial e a glomerulonefrite.

PEComas of the kidney and of the genitourinary tract.

PEComas are mesenchymal tumors composed of histologically and immunohistochemically distinctive perivascular epithelioid cells that are characterized by the coexpression of muscle and melanogenetic markers. This group of lesions includes angiomyolipoma, clear cell "sugar" tumor of the lung and extrapulmonary sites, lymphangioleiomyomatosis, clear cell myomelanocytic tumor of the falciform ligament/ligamentum teres, and rare clear cell tumors of other anatomical sites. In the genitourinary tract, PEComas have been described in the kidney, bladder, prostate, testis, and urethra. Although most PEComas behave as benign tumors, some are potentially malignant, and criteria for malignancy have been suggested for both and renal and extrarenal lesions. Recently, the expression of cathepsin K has been demonstrated in a large number of PEComas and has been proposed as a relatively specific marker to distinguish these proliferations from the majority of human cancers. In addition, a distinctive subset of PEComas harboring TFE3 gene fusions has been reported, giving rise to a possible relationship between them and MiTF/TFE family translocation renal cell carcinomas. The genetic alterations of tuberous sclerosis complex that promote activation of the mTOR pathway have been identified in PEComas. Therapy with mTORC1 inhibitors has been shown to be effective in some cases.

Long-term results of combined liver-kidney transplantation for primary hyperoxaluria type 1: the French experience.

Primary hyperoxaluria type 1 (PH1) is a hepatic metabolic defect leading to end-stage renal failure. The posttransplant recurrence of kidney disease can suggest a need for combined liver-kidney transplantation (LKT). However, the risk of LKT is theoretically far higher than the risk of kidney-alone transplantation (KAT). An unselected consecutive series of 54 patients with PH1 was analyzed according to the type of transplantation initially performed between May 1979 and June 2010 at 10 French centers. The duration of dialysis, extrarenal lesions, age, and follow-up were similar between the groups. Postoperative morbidity and mortality did not differ between the groups, and 10-year patient survival rates were similar for the LKT (n = 33) and KAT groups (n = 21; 78% versus 70%). Kidney graft survival at 10 years was better after LKT (87% versus 13%, P < .001) . Four patients (12.1%) lost their first kidney graft in the LKT group, whereas 19 (90%) did in the KAT group (P < .001). The recurrence of oxalosis occurred in 11 renal grafts (52%) in the KAT group but in none in the LKT group (P < .001). End-stage renal failure resulting from rejection was also higher in the KAT group (19% versus 9%, P < 0.0001). A second kidney transplant was performed for 15 patients (71%) in the KAT group versus 4 patients (12%) in the LKT group (P < 0.001). In conclusion, LKT for PH1 provides better kidney graft survival, less rejection, and similar long-term patient survival and is not associated with an increased short-term mortality risk. LKT must be the first-line treatment for PH1 patients with end-stage renal disease.

IgG4-related kidney disease

IgG4-related kidney disease (IgG4-RKD) is a comprehensive term for renal lesions associated with IgG4-related disease, which is a recently recognized clinical entity characterized by a dense lymphoplasmacytic infiltrate rich in IgG4-positive plasma cells with fibrosis affecting several organs. Tubulointerstitial nephritis with increased IgG4-positive plasma cells and fibrosis is the most dominant feature of IgG4-RKD and may cause acute or chronic renal dysfunction, although some glomerular lesions such as membranous nephropathy are sometimes evident. Radiologically, several characteristic abnormalities are often demonstrated, sometimes mimicking malignancies. IgG4-RKD predominantly affects middle-aged to elderly men, and most patients have accompanying IgG4-related extrarenal lesions such as sialadenitis, lymphadenopathy, or type 1 autoimmune pancreatitis. Serology usually demonstrates high levels of serum total IgG and IgG4, and high levels of serum IgE and hypocomplementemia are also frequent features. Corticosteroid therapy is usually quite effective, leading to amelioration of the renal dysfunction and radiological and serological abnormalities. However, as any delay in treatment may result in irreversible renal failure, early diagnosis and appropriate therapy are very important. Despite these distinctive clinicopathological features of IgG4-RKD, its pathogenesis remains poorly understood. Awareness of this condition and accumulation of more cases worldwide are necessary.

Can Initial (18)F-FDG PET-CT Imaging Give Information on Metastasis in Patients with Primary Renal Cell Carcinoma?

PurposeThe aim of this study was to investigate the relationship between the maximum standardized uptake values (SUVmax) of primary renal cancers with and without metastatic lesions, if any. We also studied the relationship between the size of primary renal cancers and their SUVmax, and tried to find a clinical value of 18F-FDG PET-CT for the initial evaluation of renal cell carcinoma (RCC).MethodsThe cases of 23 patients, 16 men and 7 women, who underwent PET-CT examination before operation were retrospectively reviewed. We measured the SUVmax of the primary renal cancers and those of any existing metastatic lesions, and the size of the primary renal cancers. We compared the SUVmax of primary RCCs with metastases and those without metastases, SUVmax of primary RCC and those of metastases, and studied the correlation between the size and SUVmax of primary RCCs.ResultsThe SUVmax of primary RCC of the 16 patients without metastasis ranged from 1.1 to 5.6 with a median value of 2.6. Those of the patients with metastasis ranged from 2.9 to 7.6 with a median of 5.0. The size of the all 23 primary renal cancers ranged from 1.7 cm to 13.5 cm, with a median of 4.5 cm, and their SUVmax ranged from 1.1 to 7.6, with a median of 2.9. There was a statistically significant difference between the SUVmax of the primary RCC with metastasis (5.3 ± 1.7) and those without metastasis (2.9 ± 1.0). There was a moderate positive correlation between the sizes and SUVmax of all 23 primary RCCs. However, there was no statistically significant correlation between the sizes and SUVmax of primary RCCs with metastatic lesions and the same for RCCs without metastasis. The cutoff value of SUVmax for predicting extra-renal lesion was 4.4 and that for size was 5.8 cm according to the receiver operating characteristic curves.ConclusionsThose who have primary RCC with high SUVmax are suggested to have a likelihood of metastasis. Also, there was a moderate trend of increasing value of SUVmax of primary RCC as their size increases. Physicians should beware of missing extra-renal lesions elsewhere.

Infantile Intravesical Malignant Rhabdoid Tumour

Malignant rhabdoid tumour is a rare, but highly aggressive, malignancy of early childhood. Malignant rhabdoid tumour can be classified according to renal and extrarenal locations. For extrarenal lesions, the spinal cord and central nervous system are most commonly involved, although such tumours may arise in almost any site. However, malignant rhabdoid tumour of the bladder has only been reported in a few patients, mainly focusing on its pathological aspects. We report on a patient with malignant rhabdoid tumour of the urinary bladder. The clinical, radiological, and histological features of this disease entity are discussed.

Clinical evaluation of 2-(18F) fluoro-2 deoxy-D-glucose PET/ CT in hereditary leiomyomatosis and renal cell carcinoma.

383 Background: 2-(18F) fluoro-2 deoxy-D-glucose (FDG) PET/CT is a useful tool in the staging of malignancies. In patients with kidney cancer, the role of FDG is limited in those with clear-cell histology and remains to be evaluated with other subtypes. Kidney cancer associated with hereditary leiomyomatosis and renal cell carcinoma (HLRCC) is characterized by a defect in the Krebs cycle rendering these tumors highly dependent on aerobic glycolysis (the ‘Warburg effect’) with high glucose uptake to fulfill their energy requirements; we hypothesized that FDG PET/CT may have excellent sensitivity for staging in this condition. Methods: We retrospectively reviewed patients with HLRCC kidney cancer that underwent FDG PET/CT in conjunction with anatomic imaging at our institution. The ability of FDG PET/CT to detect malignant lesions (defined using pathologic or radiologic criteria) was evaluated. Results: A total of 30 patients underwent 42 PET /CTs. Conventional imaging identified a total of 107 lesions. Both patient and lesion-based analyses were performed. A total of 90 lesions, including ten renal lesions, were classified as malignant. 76 of 80 extra-renal lesions were correctly identified as malignant by PET/CT (sensitivity, 95%, CI 88-98%). In contrast, only 4 of 10 renal lesions were correctly identified as malignant (sensitivity, 40%, CI 17-69%). 11 of 12 histologically confirmed extra-renal lesions were PET avid (sensitivity, 92%, CI-64-98%). 10 of 12 (83%) benign lesions associated with HLRCC including uterine/cutaneous leiomyomas and adrenal nodules were PET avid. In a patient based analysis, all 18 patients with extra-renal spread of kidney cancer were correctly identified (sensitivity 100%, CI 82-100%). Conclusions: FDG PET/CT is a highly sensitive diagnostic modality for identifying metastatic kidney cancer associated with HLRCC. Prospective studies evaluating the utility of PET/CT imaging to characterize response to systemic therapy are currently underway.

Aspectos anatomopatológicos da leptospirose em cães: 53 casos (1965-2011)

Os aspectos anatomopatológicos da leptospirose foram estudados em 53 cães que tiveram diagnóstico definitivo confirmado por imuno-histoquímica do tecido renal. Na necropsia, as principais lesões observadas incluíram icterícia (79,2%) e hemorragia (75,5%), principalmente no pulmão (56,6%). Alterações macroscópicas hepáticas (56,6%) e renais (50,9%) foram frequentes e caracterizavam-se principalmente por descolorações (30,2% e 32,1% respectivamente), acentuação do padrão lobular hepático (26,4%) e estriações brancas na superfície de corte dos rins (22,6%). Lesões extrarrenais de uremia ocorreram na metade dos casos (50,9%). Hepatomegalia (11,3%), nefromegalia (9,4%) e irregularidade da superfície capsular dos rins (3,8%) foram menos comuns. Na histologia dos rins (n=53), as lesões encontradas (98,1%) foram quase que exclusivamente agudas ou subagudas (96,2%) e caracterizavam-se por graus variados de nefrose tubular (86,8%) e nefrite intersticial não supurativa (60,4%), com evidente dissociação degenerativo-inflamatória. Na histologia do fígado (n=42), as lesões encontradas (97,6%) eram constituídas principalmente por dissociação dos cordões de hepatócitos (78,6%), colestase intra-canalicular (33,3%) e necrose hepática (31%). Lesões reativas, como hipertrofia das células de Kupffer, leucocitostase sinusoidal e infiltrado inflamatório mononuclear nos espaços porta, foram vistas em muitos casos (42,8%). Na histologia do pulmão (n=28), hemorragia (85,7%) e edema (57,1%) alveolares foram muito prevalentes. Neutrófilos e macrófagos nos espaços alveolares (35,7%) e neutrófilos no interior de pequenos vasos pulmonares (17,9%) também foram achados frequentes. Os resultados aqui demonstrados devem servir de alerta aos patologistas veterinários brasileiros, pois a apresentação anatomopatológica da leptospirose canina em nossa região (Região Central do Rio Grande do Sul, Brasil) não se modificou nos últimos 50 anos, mantendo-se semelhante àquela descrita internacionalmente até a década de 1980, mas muito diferente do que é atualmente reconhecido para os Estados Unidos, o Canadá e parte da Europa Ocidental. Recomendamos que os critérios histopatológicos para o diagnóstico da leptospirose canina devem incluir a presença concomitante de nefrite tubulointersticial aguda ou subaguda, hepatite reativa não específica e lesão alveolar difusa, incluindo hemorragia alveolar difusa com capilarite, em um cão que durante a necropsia demonstre icterícia, hemorragias e lesões extrarrenais de uremia na ausência de esplenomegalia.

Urolitíase em 76 cães

Entre janeiro de 1990 e dezembro de 2010 foram necropsiados 4.872 cães no Laboratório de Patologia Veterinária da Universidade Federal de Santa Maria (LPV-UFSM). Destes, 76 (1,6%) apresentaram urólitos em algum local do sistema urinário. O perfil epidemiológico dos cães afetados demonstrou o predomínio de machos (64,5%), adultos (52,6%) e com raça definida (56,6%). Sinais clínicos indicativos de urolitíase foram reportados em 30,3% dos casos e consistiram principalmente de hematúria, anúria, disúria e incontinência urinária. Os urólitos tiveram localização única ou múltipla e os locais anatômicos mais frequentemente acometimentos, em ordem decrescente de frequência, foram: bexiga, rim e uretra. Urolitíase ureteral não foi observada. Lesões secundárias à urolitíase foram observadas em aproximadamente 40% dos cães afetados; as mais prevalentes, em ordem decrescente de frequência, foram: cistite, obstrução uretral, hidroureter, hidronefrose, ruptura vesical (com uroperitônio) e pielonefrite. Em 25% dos cães afetados ocorreu morte espontânea ou eutanásia decorrente das lesões secundárias à urolitíase. Lesões extra-renais de uremia foram observadas em 11,8% dos casos.