Renal Nephroblastoma Research Articles

A case of rapid canine nephroblastoma presumed metastasis documented with advanced imaging

AbstractA 1.5‐year‐old, female, entire German shepherd dog was presented for evaluation of an abdominal mass. The dog had a 4‐month history of polydipsia, polyuria, diarrhoea, and weight loss despite polyphagia. Abdominal ultrasound confirmed a large abdominal mass suspected to be uterine in origin. An exploratory celiotomy revealed a 4.3 kg, 26 × 17 × 17 cm multinodular mass entirely encompassing the uterus, left ovary and left kidney. A combined ovariohysterectomy and left nephrectomy were undertaken. Histopathology confirmed stage ΙΙ renal nephroblastoma with favourable histology. Chemotherapy was declined and 86 days following surgery, significant abdominal metastases were identified on a computed tomography scan. The patient was euthanased 113 days after surgery due to progressive disease. This is the first report to display the rapid presumed metastasis of a canine nephroblastoma, without gross metastasis at initial surgical resection, through advanced imaging. This is also the first reported case of polyphagia despite weight loss as a presenting clinical sign.

Pediatric genitourinary tumors: Distribution, demographics, and outcomes.

ABSTRACTImportanceThe diversity of pediatric genitourinary malignancies requires a timely resource detailing tumor characteristics and survival.ObjectiveTo determine the incidence, demographics, and outcomes of all pediatric genitourinary tumors within the United States.MethodsA population‐based search for patients diagnosed with genitourinary cancers under age 15 was performed using the National Cancer Institute's Surveillance, Epidemiology, and End Results 18 registry. Information on primary tumor location, histologic type, patient age, sex, year of diagnosis, race, treatment, cause of death, and survival months was extracted. Descriptive epidemiological and survival statistics were calculated for all variables.ResultsA total of 4576 cases from 1973 through 2015 were identified. The most common primary tumor sites were the kidney (80.3%), testis (12.3%), bladder (2.8%), and vagina (1.5%). Nephroblastoma (87.9%) and sarcoma (3.4%) were the most common renal malignancies. Rhabdomyosarcoma was common in the vagina, bladder, and testis at rates of 66.2%, 61.2%, and 24.6%, respectively. Germ cell tumors (71.0%) were the most common primary tumor of the testis. Ten‐year overall survival (OS) for renal nephroblastoma and sarcoma was 88% and 82%, respectively. Ten‐year OS for RMS of the testis was 91%, the bladder was 79%, the vagina was 79%, and the prostate was 56%. Germ cell tumor 10‐year OS were 96% in the testis and 100% in the vagina.InterpretationA better understanding of the overall distribution and outcomes associated with pediatric genitourinary cancers allows physicians to best understand the patient's disease in the context of current frequency in a genitourinary setting and reported outcomes.

Holistic management of renal nephroblastoma in a juvenile dog with severe cachexia: A case report

This report presents a case of a 2-month-old female German Shepherd dog with renal nephroblastoma and strong cachexia. The tumour was surgically removed and graded as a stage I with favourable histopathology, but due to its large size, adjuvant chemotherapy, including doxorubicin and vincristine, was recommended. Before, during and after chemotherapy, the body weight was gradually rebuilt with an appropriately balanced diet containing proper amounts of protein, fat and numerous supplements necessary for growth and development. Eighteen months after diagnosis, the dog was in good condition and had no metastases. In conclusion, this case study shows that close cooperation between oncologists and nutritionists is crucial for successful oncological therapy, especially in a cachectic patient.

Imaging of renal tumors in children

Renal tumors in children are treated according to the guidelines of the Renal Tumor Study Group of the International Society of Pediatric Oncology (SIOP-RTSG). Nephroblastoma is the most frequent renal tumor in children. After sonography, magnetic resonance imaging (MRI) is the preferred imaging modality. The task of imaging includes differential diagnosis with the help of morphological and epidemiological criteria. Thorax computed tomography (CT) is introduced for initial staging. Current studies of diffusion-weighted imaging (DWI)-MRI with analysis of the apparent diffusion coefficient (ADC) histogram indicate the potential to differentiate blastemal or anaplastic high-risk histology nephroblatoma subtypes. Imaging criteria for nephron-sparing surgery are defined and allow an individual therapy option in unilateral and especially in bilateral renal nephroblastoma. In addition to nephroblastoma, the differential diagnosis includes congenital mesoblastic nephroma, malignant rhabdoid tumor of the kidney, clear cell sarcoma and renal cell carcinoma. The diagnosis of nephrogenic rests and nephroblastomatosis is challenging. Diagnostic standardization improves diagnosis and therapy of renal childhood tumors, and new prognostic markers may be introduced in the near future.

Renal nephroblastoma in a 10-month-old boxer

A 10-month-old male entire Boxer dog was presented for weight loss and abdominal distension with a large mass palpable within the abdomen. A unilateral nephrectomy was performed for a large...

Renal nephroblastoma in an adult dog

Nephroblastoma is a neoplasia originating from the poor differentiation of the metanephrogenic blastema, therefore neoplasia of embryonic origin. It is commonly reported in dogs less than two years old and is rare in adults. Clinical signs include hematuria, polyuria and palpable abdominal mass. Surgery is elective therapy. Chemotherapy depend on the stage of the disease. The purpose of this paper is to report a case of nephroblastoma in a mixed-breed, six-year-old male canine with a stage I nephroblastoma (according to human staging for Wilm's tumor). The patient performed clinically well and without changes in the staging exams (thorax radiographs). The initial complete blood count indicated only lymphopenia and biochemical profile without alterations. Abdominal ultrasound showed vascularized formation with no defined limits, cystic, with probable origin in left kidney, dislocating liver and spleen. The recommended treatment was total nephrectomy. Kidney size was about 25.0 cm in diameter, with irregular surface. Numerous cysts were present and were covered by a capsule with brownish-coloured liquid and without adhering to adjacent structures. The nephroblastoma diagnosis was obtained by histopathological exam. No adjuvant chemotherapy treatment was carried out. The patient presented a survival of 33 months.

Nephroblastoma in a black-tufted marmoset (Callithrix penicillata)

ABSTRACT: A renal nephroblastoma is described in a free-living black-tufted marmoset (Callithrix penicillata) in Central Brazil. The monkey was found dead and subjected to necropsy. Gross anatomic changes consisted of a ruptured left kidney, which was almost completely effaced by a white to yellow, partially encapsulated friable mass. The left ureter was distended due to obstruction by a red, spherical, 2mm in diameter friable mass. The urinary bladder was also distended. Histologically the renal and ureteral masses consisted of a triphasic embryonal neoplasm composed of embryonic epithelium forming glomeruli and tubules, polygonal blastemal cells, and a mesenchymal stroma. The embryonic epithelium exhibited rare nuclear immunoreactivity for WT-1, whereas blastemal cells exhibited robust cytoplasmic and rare nuclear immunoreactivity for WT-1; blastemal cells were also immunoreactive for vimentin. No immunoreactivity was detected for pan-cytokeratin (AE1/AE3), actin, and desmin. Morphological and immunohistochemical features of the present neoplasm are consistent with those described for renal nephroblastoma.

Intra-cardiac extension of a Wilms’ tumor

Wilms᾿ tumor, or renal nephroblastoma, is the commonest malignant renal tumor in children and young adults. Intravascular and intracardiac extension of this tumor is a very rare and challenging clinical entity. The case is reported of a 6-year-old boy diagnosed with nephroblastoma, in whom the tumor involved the inferior vena cava and the right atrium. The successful multimodal surgical management consisted of combined, synchronous surgical resection of both renal and intracardiac tumors. The boy had an uneventful postoperative course and recovery, followed by relapse-free living. Intra-cardiac extension of Wilm’s tumor poses a surgical challenge where the favorable outcome is provided only by the combined and well-coordinated, skilled multidisciplinary care of pediatric surgeons and pediatric cardiac surgeons.

Nephroblastoma in Two Siamese Fighting Fish Betta splendens

In 2005 and 2008, renal nephroblastomas were diagnosed in two adult male Siamese fighting fish Betta splendens. In both cases the masses effaced approximately 90 percent of renal tissue and histopathologically, were composed of a triptych of diagnostic features, to include: nests of cells suggestive of blastema, a primitive mesenchyme as well as an epithelial component forming glomeruloid structures. To our knowledge, these cases represent the first reports of nephroblastoma in Siamese fighting fish.

Pattern of paediatric renal disease in inpatients in Calabar, Nigeria

A 10-year retrospective review of inpatients in Calabar showed that of 5641 children admitted,182 (3.2%) had renal disease. The major lesions were acute glomerulonephritis (36.9%) and nephrotic syndrome (30.7%), while less common ones included urinary tract infection (8.9%), obstructive uropathy, acute renal failure (6.7%, each) and nephroblastoma (6.2%).

Intrarenal Pelvic Nephroblastoma in a Meerkat (Suricata Suricatta)

Nephroblastoma is the most common primary renal tumor in children and has also been reported in domestic and nondomestic animal species. Intrapelvic renal nephroblastoma is a rare variant of this tumor type in human patients. Postmortem examination of a captive meerkat (Suricata suricatta), which was found dead, revealed enlargement of the pelvis of the left kidney by a tumor mass. Gross, histological, and immunohistochemical findings were consistent with a diagnosis of triphasic intrapelvic renal nephroblastoma. This is the first reported spontaneous case of intrapelvic renal nephroblastoma in a nonhuman species.

Development of an siRNA-based method for repressing specific genes in renal organ culture and its use to show that the Wt1 tumour suppressor is required for nephron differentiation

Wt1 is a tumour suppressor gene, mutation of which is a cause of Wilms' tumour, a childhood renal nephroblastoma. Wt1 is expressed in a rich pattern during renal development suggesting that it acts at three stages: determination of the kidney area, the differentiation of nephrons and maturation of glomeruli. Wt1-/- mice confirm that Wt1 is essential for the inception of kidney development; cells that ought to form kidneys die by apoptosis instead. Specific human WT1 mutations cause defects of glomerular maturation (Denys-Drash and Frasier syndromes), providing circumstantial evidence for action of Wt1 during glomerular maturation. There is, however, no genetic evidence for a function during nephron differentiation because this stage is never reached in Wt1-/- mice. We have therefore developed a novel technique, based on small interfering RNA (siRNA), to repress the expression of Wt1 and other specific genes at different stages of kidney development in culture. We find that early repression of Wt1 phenocopies the Wt1-/- mouse, but later repression prevents cells differentiating into nephrons and causes them instead to proliferate abnormally, possibly mimicking aspects of Wilms' tumour. In line with established hypotheses about genetic pathways that control kidney development, we find that repressing Pax2 using siRNAs represses Wt1 expression and blocks both bud growth and nephron differentiation, but that repressing Wnt4 blocks nephron differentiation without affecting Wt1 expression. As well as illuminating previously inaccessible aspects of Wt1 biology, our results suggest that siRNA in organ culture will be a powerful method for analyzing other developmental pathways and testing the effects of stage-specific loss of tumour suppressor genes.

Wilms' Tumor of the Cervix: A Case Report and Review of the Literature

Introduction. Extrarenal Wilms' tumors are rare, with only 55 well-documented cases in the literature and only 4 confined to the uterus. A fifth case along with review of the current literature is presented.Case. A 13-year-old girl presented with a 2-month history of irregular vaginal spotting. A 6.5 × 4.5 × 2.5-cm polypoid mass meeting criteria for an extrarenal Wilms tumor was removed from the superior aspect of the cervix, but recurred 8 months later on the posterior lip. The patient underwent a Schauta procedure, lymph node dissection, and bilateral oophoropexy. The surgery was followed by typical renal Wilms' tumor chemotherapy. The patient is without evidence of disease 5 years after surgery.Discussion. This case uniquely suggests that limited excision of the original tumor has inherent risks for relapse. Oophoropexy appears to be reasonable when fertility preservation is desired and reconfirms that treatment mimicking renal nephroblastomas offers good long-term survival.

Promoting effects of ethinyl estradiol on development of renal proliferative lesions induced by N-nitrosobis (2-oxopropyl)amine in female Syrian golden hamsters.

The modulating effects of female sex hormones, ethinylestradiol and levonorgestrel, on the development of renal proliferative lesions after initiation with N-nitrosobis(2-oxopropyl)amine (BOP) were investigated. Three groups of female Syrian golden hamsters, each comprising 30 animals, were given four weekly s.c. injections of BOP (10 mg/kg body weight) and then fed diet containing 1 ppm ethinylestradiol (group 1: BOP/EE), diet containing 10 ppm levonorgestrel (group 2) or basal diet (group 3) for the next 27 weeks. As hormone controls, two groups of 20 female hamsters each received diet containing 1 ppm ethynylestradiol (group 4) and 10 ppm levonorgestrel (group 5) from week 3 for 27 weeks without the prior initiation treatment. The severity of diffuse anisokarya, characterized by varied nuclear size and the incidence of dysplasias of the proximal tubular epithelia induced by BOP, were significantly increased in the BOP/EE group, indicating the promoting effects of the hormone. In the renal dysplastic lesions (small-cluster, cystic, clear-cell and acidophilic cell types), adenomas and nephroblastomas, increases in the numbers of argyrophilic proteins associated with nucleolar organizer regions (NOR) in the nucleus, suggesting a high proliferative activity, were seen in dysplasia of acidophilic cell types and adenomas. In addition, the number of bizarre NOR per nucleus was significantly higher in adenomas than in dysplasias and highest in nephroblastomas. This morphological change in NOR should therefore be a useful parameter for the diagnosis of malignancy of renal tumors.

Dose-related induction of lung, thyroid and kidney tumors by N-bis(2-hydroxypropyl)nitrosamine given orally to F344 rats.

The organotropic effect of orally administered N-bis(2-hydroxypropyl)nitrosamine (DHPN) in male F344 rats was studied with respect to total dosage and length of observation period. DHPN was given at 5 dosages, a single intragastric intubation of 800 mg/kg body weight, or administration as a 0.2% solution in the drinking water for 4, 7, 14 or 21 days. Animals were killed at weeks 32, 40 and 48. Tumors were induced in the lung, thyroid, kidney and urinary bladder. High tumor incidence was demonstrated in the lung and thyroid. Dose-related increases in overall tumor incidences in these 4 organs were clearly shown but time-related increases at the 3 sacrifice time points were not clear. Histologically, the tumors were adenoma and adenocarcinoma of the lung and thyroid, renal cell tumor, nephroblastoma and transitional cell tumor of the kidney, and transitional cell papilloma of the urinary bladder.