Congenital Bilateral Wilms Tumor: A Case Report.

Compare to congenital mesoblastic nephroma in fetus, congenital wilms tumor is extremely rare. Herein we report a case of congenital bilateral solid masses on antenatal ultrasound. The mass was evaluated by ultrasonography and contrast computed tomography scan in postnatal period, and the patient was undergoing tumor enucleation separately in short period after neoadjuvant chemotherapy. The diagnosis was confirmed by histology analysis for each side, and the treatment was taken according to the International Society of Pediatric Oncology.

The clinical presentation, imaging features and differential diagnoses of congenital Wilms tumour.

Solid fetal renal masses are a rare finding on antenatal ultrasound, with hydronephrosis and cystic disease of the kidney usually being the most common causes for fetal renal enlargement. Herein we report a case of a solid fetal renal mass which was detected on third trimester antenatal ultrasound scanning. This renal mass was evaluated by MRI in the postnatal period and diagnosis confirmed by histological analysis, after surgical excision. Also discussed are the differential diagnoses and imaging features of other solid fetal renal masses, including congenital mesoblastic nephroma, nephroblastomatosis, renal sarcoma and angiomyolipoma.

Congenital renal tumor: metanephric adenoma, nephrogenic rest, or malignancy?

We report a renal tumor detected by prenatal ultrasound and resected at 2 months of age. This 9-cm, solid mass was composed of tubular and papillary structures lined by small, uniform epithelial cells. There was local invasion into renal parenchyma and a tumor deposit in a hilar lymph node. The tumor was immunopositive for WT1, pankeratin, and CD10; focally positive for CK7; and negative for EMA and TFE3. Based on morphology and immunophenotype, the favored diagnosis was metanephric adenoma over Wilms tumor, renal cell carcinoma, and nephrogenic rest. However, metanephric adenoma only occasionally occurs in children and has never been reported prenatally. Alternatively, this tumor might be a congenital Wilms tumor that differentiated completely. Although the nature of the tumor remains unconfirmed, resection appears to have been curative; the patient remains disease-free 18 months following surgery alone.

Fine needle aspiration cytology of fetal rhabdomyomatous and teratoid Wilms tumor.

OBJECTIVE: To review nephrectomy specimens for pediatric renal tumors seen over a period of 9 years (1995-2003). STUDY DESIGN: Specimens categorized as fetal rhabdomyomatous Wilms tumors (WTs) or teratoid WTs were selected. Corresponding fine needle aspiration cytology slides were subjected to cytomorphologic analysis. RESULTS: Of 93 specimens of WT, 3 cases of fetal rhabdomyomatous WT and 2 cases of teratoid WT were identified. The aspirates were stromal predominant, and all of them showed rhabdomyoblasts embedded within. These stained variably green or orange on Papanicolaou stain and blue-gray on May-Grünwald-Giemsa stain. In all 5 aspirates, foci of blastema with or without tubules were identified, permitting a diagnosis of WT. A squamous morule was seen in an aspirate from teratoid WT. CONCLUSION: The outcome of fetal rhabdomyomatous and teratoid WTs is good, but the tumors do not shrink with chemotherapy. Identification of rhabdomyoblastic elements on aspirates can help in assessment of subsequent response to treatment.

Characteristics and survival of 750 children diagnosed with a renal tumor in the first seven months of life: A collaborative study by the SIOP/GPOH/SFOP, NWTSG, and UKCCSG Wilms tumor study groups.

BACKGROUND: To review the clinical characteristics and survival of infants diagnosed with a primary renal tumor in the first 7 months of life. PROCEDURE: A retrospective data review of patients registered in five large international protocols (SFOP/GPOH/SIOP9/93-01, UKW3 and NWTSG 4 and 5) spanning 1985-2002. RESULTS: 750 (7.2%) of 10,430 registered patients were diagnosed with a renal tumor before age 213 days. Tumor types were Wilms tumor (WT) 58%; congenital mesoblastic nephroma (CMN) 18%; malignant rhabdoid tumor (MRTK) 8%; clear cell sarcoma (CCSK) 2%; non-Wilms tumor (unspecified) 6%; histology unknown, 9%. CMN predominated among tumors diagnosed in the first month of life (54%) but its relative contribution diminished to <10% of all cases diagnosed after the age of 3 months (P < 0.001). Among 639 cases with specified histology and stage, 9/11 stage IV tumors were MRTK, 37/39 bilateral tumors were WT. In 626 children where surgical approach was specified, 522 had immediate nephrectomy. For all cases, 5 years event-free survival (EFS) was 80% and overall survival (OS) 86%. Five years EFS and OS respectively by tumor type were WT (86%, 93%), CMN (94%, 96%), CCSK (49%, 51%), MRTK (16%, 16%). CONCLUSION: Renal tumors diagnosed in the first 7 months of life generally have an excellent prognosis though histology is an important prognostic factor. In the first 2 months of life the prevalence of CMN is high. The relative occurrence of WT increases rapidly with age thereafter. Bilateral tumors are usually WT. Tumors with metastases at diagnosis are usually MRTK.

[Congenital solid tumors. A thirteen-year review]. / Tumores sólidos congénitos. Revisión de 13 años.

UNLABELLED: Tumors diagnosed during the first month of life are infrequent: 0.5 to 2% of all childhood neoplasms. This is an interesting group of tumors because their type, relative incidence, natural history and response to treatment differ from those seen in older children. AIM: To contribute the experience of our institution in congenital tumors the last 13 years. MATERIAL AND METHODS: The records of all neonates (< 31 days old) diagnosed with solid tumors since January 1990 to December 2002 have been retrospectively reviewed. RESULTS: Twenty-seven neonates have been diagnosed with tumors in the last 13 years. Thirteen patients (48%) were prenatally diagnosed. Nine babies were diagnosed at the initial neonatal exam (40% of those diagnosedd after birth). Neuroblastoma was the commonest tumor (10 cases, 37%), of which 4 were stage I, 4 stage IV-S and 2 stage III. There were 8 teratomas (3 sacrocoxigeal, 1 retroperitoneal, 1 in the CNS, 1 orbitary and two oronasal), two hepatic tumors (1 hepatoblastoma, 1 hemangioendothelioma, two CNS tumors, two giant nevus (one on a hamartoma), and one each Wilms tumor, infantile fibrosarcoma and myofibroblastic tumor. Treatment was surgical resection alone in 17 cases (68%) and surgery + chemotherapy in 8 (32%) (5 neuroblastomas, one CNS tumor, one Wilms tumor and one presacral teratoma who developed a yolk sac tumor); 3 patients died (11%): one at surgery, one of tumoural airway obstruction at birth and one with craniopharyngioma. Among the 14 tumors that were initially not malignant, two can be locally agressive, one was an immature teratoma, the giant nevus with hamartoma developed in situ melanoma, the other nevus had meningeal melanosis with hydrocephalus, and one mature presacral teratoma developed a yolk sac tumor. CONCLUSIONS: Diagnosis of congenital tumors is performed earlier in recent years due to the wide use of prenatal ultrasound. Their natural history is more benign than in other age groups, except for CNS tumors and very large or obstructing tumors. The histological patern is not determinant of the outcome. Complete surgical excision is the treatment of choice, most cases not need adjuvant chemotherapy. We ought to pass this message on to our colleagues in prenatal diagnosis, so parents get reliable information.

Renal tumors in infants less than 6 months of age.

BACKGROUND/PURPOSE: Renal tumors are rare in infants less than 6 months of age and may have associated paraneoplastic symptoms. To better define the characteristics of these tumors the authors reviewed their 10-year institutional experience. METHODS: The authors searched the pathology database to identify all renal tumors resected at their institution since 1992 (after IRB approval and guidelines). The clinical presentation, operative details, pathology, and outcome for all children < or = 6 months of age were reviewed. RESULTS: Of 101 children who had renal tumors resected during this period, 11 (11 %) were 6 months of age or less. Histopathologic examination showed congenital mesoblastic nephroma (CMN) in 7 patients (4 with cellular features), Wilms' tumor in 3 patients, and ossifying renal tumor of infancy in 1. Renal masses were detected antenatally in 2 patients and during newborn examination in 1 patient; however, the mean age at diagnosis was 72 +/- 18 days. Ten children had a palpable abdominal mass, 3 had gross hematuria, and 6 had hypertension (4 CMN; 2 Wilms'). Only 1 child had hypercalcemia (cellular CMN). Ten infants had nephroureterectomy, and 1 had a partial nephrectomy. All patients had either stage I or II disease. At follow-up (mean 4.2 +/- 1.2 years) 10 patients are alive with no evidence of disease. One newborn with hydrops and a very large congenital Wilms' tumor had abdominal compartment syndrome and died during surgery. CONCLUSIONS: About 10% of renal masses may occur in infants less than 6 months of age. Although mesoblastic nephroma is the most common renal tumor in this age group, Wilms' tumor also may be seen. Paraneoplastic syndromes, such as hypertension and hypercalcemia, are common in these infants and are not specific for tumor type. These tumors generally present at an early stage and have an excellent prognosis overall.

Diagnóstico prenatal de una masa suprarrenal fetal / Prenatal diagnosis of a fetal adrenal mass

El neuroblastoma es el tumor suprarrenal más frecuente en el feto, generalmente en su variedad quística (benigna). El diagnóstico diferencial hay que realizarlo con la hemorragia suprarrenal, que tiene un pronóstico favorable y una resolución espontánea postparto. La glándula suprarrenal es una estructura muy vascularizada con un tamaño relativo 20 veces superior a la del adulto, por lo que un aumento brusco de la presión intravascular o una agresión hipóxica pueden provocar un sangrado intraglandular. La técnica diagnóstica prenatal de elección es la ecografía. Cuando la hemorragia se produce durante el embarazo se diagnostica habitualmente alrededor de la semana 20 de gestación. Cuando se sospecha una hemorragia suprarrenal se aconseja seguimiento ecográfico en espera de su resolución postnatal. Presentamos el caso de una gestante a la que se le detectó ecográficamente en la semana 20 una tumoración suprarrenal fetal izquierda que fue aumentando de tamaño durante el embarazo. El parto se produjo en la semana 41 y tras el mismo se confirmó la presencia de una tumoración, descartándose adenopatías o metástasis. En el estudio ecográfico al mes de vida se observó una disminución del tamaño tumoral con hiperecogenicidad, por lo que se diagnosticó hemorragia suprarrenal fetal en regresión (AU)

Neuroblastoma, the most common adrenal mass, generally presents as a cystic tumour of benign nature. Differential diagnosis should consider adrenal hemorrhage, condition which carries better prognosis and spontaneous resolution after delivery. Fetal adrenal gland, a vasculari in the gland as a result of high pressure or hypoxic injury. The gold standard method of diagnosis is based on ultrasound. When adrenal bleeding occurs during pregnancy, diagnosis of this condition takes place at around twenty weeks. Then, when adrenal hemorrhage is suspected, conservatory management with follow up scans is advised until spontaneous resolution is reached. A case of twenty week pregnancy in which a growing left adrenal tumour was detected is presented. After delivery, the presence of an isolated tumour not associated with metastasis or lymph nodes was confirmed. Ultrasound follow up one month later showed reduction of size and echogenicity of the tumour which led to the diagnosis of adrenal hemorrhage in regression (AU)

Presence of vascular anomalies with congenital hemihypertrophy and Wilms tumor: an evidence-based evaluation.

Congenital hemihypertrophy is an uncommon condition of unknown etiology characterized by unilateral overgrowth of part or all of one side of the body. Hemihypertrophy is known to be associated with certain childhood tumors, most notably Wilms tumor (or nephroblastoma), and for this reason infants with hemihypertrophy are often followed with serial abdominal ultrasounds. Klippel-Trénaunay syndrome (KTS) is the triad of port-wine stain, venous varicosities, and soft tissue and/or bony hypertrophy. Children with KTS typically have localized rather than generalized hemihypertrophy, but occasionally the hypertrophy is more extensive than the vascular anomaly itself. Information is lacking about whether hemihypertrophy in this setting can also be a risk factor for Wilms tumor. We systematically reviewed the medical literature to determine whether well-documented cases of Wilms tumor in the setting of both hemihypertrophy and vascular anomalies have been described, and if found, whether the association was sufficiently frequent that routine screening for Wilms tumor in this setting should be recommended. A review of case reports and case series in the pediatric population was undertaken using specific inclusion and exclusion criteria. We found 4 of 58 subjects with hemihypertrophy and Wilms tumor had a reported vascular anomaly, but in only one case was a clear-cut diagnosis of KTS confirmed. The relationship of the other three vascular anomalies reported was of uncertain significance. In conclusion, our review suggests that the risk of Wilms tumor in the setting of localized soft-tissue hypertrophy in conjunction with a vascular malformation is quite low. More extensive hemihypertrophy extending to body sites remote from the vascular malformation itself could have a higher risk of Wilms tumor, although the magnitude of this risk is uncertain. Our findings suggest that routine serial abdominal ultrasounds in patients with vascular malformations in association with localized soft-tissue hypertrophy are unwarranted.

Congenital and hereditary tumours in domestic animals. 2. pigs. A review.

The literature on congenital and hereditary tumours in pigs was reviewed. One hitherto unreported own case was added. Sporadic cases of congenital tumours included several types found in newborn piglets. Embryonic tumours (nephroblastoma, rhabdomyosarcoma) occurred either in newborn or in juvenile animals. Cardiac rhabdomyomas were provisionally classified as hamartomas. The hereditary tumours, melanomas and lymphomas, were reproducible by experimental matings. These tumours are particularly interesting as models to elucidate genetic and immunologic mechanisms of tumour diseases. Striking is the high degree of regression in porcine melanomas and the associated melanosis.

Prenatal diagnosis of congenital Wilms' tumor (nephroblastoma) presenting as fetal hydrops.

We describe a case of congenital nephroblastoma (Wilms' tumor) presenting at 28 weeks of gestation with fetal hydrops and polyhydramnios. Prenatal diagnosis was made by biopsy. An emergency Cesarean section was performed due to deterioration in the cardiotocograph. A post-mortem examination confirmed the diagnosis of congenital nephroblastoma.

[Neonatal segmental cystic nephroma. A case report]. / Neonatales segmental-zystisches Nephrom. Eine Kasuistik.

UNLABELLED: Multicystic segmental renal dysplasia is rare in early childhood. We report a case with prenatally recognized renal malformation. Prenatally a cystic renal malformation was detected sonographically; postnatally further evaluation was performed by Doppler sonography, contrast enhanced CT and voiding cysto-urethrography leading to the diagnosis of a multicystic segmental nephroma. Due to increasing size in spite of therapeutic and diagnostic sonographic guided punctures and the atypic manifestation the baby underwent heminephrectomy. The final histological diagnosis confirmed preoperativ findings. CONCLUSION: Prenatally recognised cystiform renal malformations should be reevaluated postpartally by ultrasound and--as doubtful findings are found--further imaging might be necessary for follow up and for the decision on conservative or operative treatment.

Wilms' tumor and related abnormalities in the fetus and newborn.

Mesoblastic nephroma, a benign tumor, is the most common renal neoplasm in neonates. Wilms' tumor (WT) may occur in newborn infants, but is more common in older children. The molecular genetics of WT involves one or more genes located on Chromosome #11 and probably other locations not yet elicidated. Germline mutations cause less than 5% of WTs; most WTs are sporadic. Precursor lesions to WT called nephrogenic rests may be detected before evolution to WT by imaging studies. Developmental anomalies comprising several different syndromes are associated with nephrogenic rests and predisposition to WT. Prospective surveillance for WT may be feasible in high risk infants identified on the basis of physical findings followed by testing for predisposing gene defects and periodic imaging of the kidneys and other organs at risk until the period of risk has ended.

Histopathological variations among cases of Wilms' tumor in Bangladesh and its relationship with prognosis.

Recent advancement in the cytopathologic features produced a number of variants of Wilms' tumor which are the primary determinant of survival of Wilms' tumor patients. This study was carried out with 47 patients of Wilms' tumor in different stages in three selected hospitals from 1991 to 1993. Among them 61.7% (29) were in Favorable histopathology and 38.3% (18) were in Unfavorable histopathology group. After managing the patients with multimodal therapy according to the protocol of National Wilms' tumor Study-III the favorable group had shown better prognosis. The difference between two groups was statistically significant (chi-square = 3.2, P < 0.05). Histopathological variations could be easily determined which might improve the overall prognosis of Wilms' tumor.

Solid tumours in newborns and infants.

Thirty two cases of solid tumours in newborns and infants were observed by the authors over a 15 year period. The most common type of tumours were soft tissue tumours (n = 8), neuroblastomas (n = 7), nephroblastomas (n = 5) and germ cell tumours (n = 5). Other types of embryonal tumours such as retinoblastoma and hepatoblastoma were observed in four children and non-Hodgkin's lymphomas in three children. Of 25 children followed up 18 survived. Five children died of their tumours and two children died of causes unrelated to their basic disease. Analysis of the fate of the patients and the effect of the therapy is made. It is concluded that malignant tumours prevail in infancy (90.6% of all cases). The percentage of patients treated successfully with insignificant risk for consequences from radiotherapy and chemotherapy is also high (72%).

Bilateral hyperplastic nephromegaly, nephroblastomatosis, and renal dysplasia in a newborn: a variety of universal nephroblastomatosis.

A preterm boy was born at 34 weeks. Prenatal ultrasonography showed oligohydramnios, fetal ascites, large kidneys, and small thorax. He died 21 h after birth of respiratory insufficiency. Autopsy revealed Potter's-like facies, hypoplastic lungs, ascites, and bilateral nephromegaly (renal weight almost 10 times normal). The kidneys were finely nodular externally, solid, and cerebriform on cut section. Histologically, they showed a diffusely distorted architecture of jumbled lobules, hyperplasia of cortical-type tissue with inconspicuous proximal tubules, relative hypoplasia of medullary tissue, tubulointerstitial dysplasia, and perilobar nephrogenic rests. The renal features represent a variety of the universal or panlobar (also called pancortical or infantile) type of nephroblastomatosis. To our knowledge, this is only the third such case reported. In the brain, each lateral ventricle contained a yellow gelatinous mass. Histologically, the masses consisted of a pseudomyxoid matrix with delicate fibers and focal adipocyte clusters, all confined within choroid plexus. We consider these lesions fibrolipomatous hamartomas.