Diagnosis Of Wilms Research Articles

Cystic nephroma

The clinical and pathologic findings of four cases of cystic nephroma (so-called "renal multilocular cyst") in four infants nephrectomized with the diagnosis of Wilms' tumor are presented. Each lesion consisted of an encapsulated mass composed of cysts separated by thin septa. Histologically the cysts were lined by epithelium, and the septa contained foci of immature metanephric blastema (abundant in one case and sparse in another). rhabdomyocytes and mature tubules. Neither embryonic ducts nor cartilage were seen. One cystic lesion was adjacent to a nephroblastoma which formed a separate nodule. No recurrences of metastases developed, and the only death was due to postoperative sepsis. These lesions are considered by some authors to be dysplasia, while others interpret them as benign neoplasms. Our findings and a review of the literature probably represent the differentiated counterpart of nephroblastoma.

Pulmonary metastases in Wilms' tumour

In a series of 87 patients with a diagnosis of Wilms' tumour seen at the Great Ormond Street Hospital for Sick Children between 1960 and 1973, 15 patients had pulmonary metastases at the time of diagnosis and a further 22 patients developed pulmonary metastases within 18 months of their initial nephrectomy. The incidence of later development of metastases was very much greater in those patients with locally advanced primary tumours than in those with a completely resectable, encapsulated tumour. In both groups of patients the disease-free survival rate was closely related to initial local tumour staging. In the whole series there were 11/37 (42-5%) patients with pulmonary metastases who survived disease-free for two or more years following treatment. The survival rate was highest in those patients who had a solitary pulmonary metastasis which was surgically resected (5/6); this type of lesion was seen only in those patients who had a localised primary tumour. In those patients with multiple pulmonary metastases, treated with irradiation to the whole lungs and single-agent chemotherapy, there were only 6/31 disease-free survivors; this group of patients all had advanced primary tumours and there was a high incidence of associated abdominal recurrence. The need for multiple agent chemotherapy in conjunctin with surgery and radiotherapy, both as prophylaxis against and in the treatment of metastases from Wilms' tumour is stressed.

Prognosis and treatment of wilms' tumor at great ormond street hospital for sick children—1960–1972

Eighty-one children with a diagnosis of Wilms' tumor who were treated at the Great Ormond Street Hospital for Sick Children between 1960 and 1972 are reviewed. The National Wilms' Tumor Study group staging system is used. Distribution by age and sex in each stage together with survival rate is given. Treatment and prognostic factors are discussed. Wilms' tumor is most common in children below the age of 3 years. There was a survival rate of 87% in Stage I, 36% in Stage II, 8% in Stage III, and 33% in Stage IV. The survival was worse in patients with locally advanced tumor than in patients who had pulmonary metastases in association with a locally resectable tumor. Whole abdominal irradiation is necessary in all patients in whom the tumor has breached the renal capsule. Single agent chemotherapy has not proved effective in preventing relapse in patients with advanced tumors. More intensive therapy is needed in this group to prevent both local recurrence and distant metastases. The addition of multiple drug chemotherapy to nephrectomy and whole abdominal irradiation is already improving the disease-free survival.

The incipient Wilms' tumor

The diagnosis of Wilms' tumor is usually made by excretory urography after the patient presents with an abdominal mass. A diagnosis prior to the appearance of an obvious mass might result in an improved cure rate and less morbid therapy. The three cases described illustrate the difficulty in making an earlier diagnosis since pyelograms were "normal" prior to detection of the abdominal mass.

Bone metastases in Wilms' tumour

In a series of 81 patients with a diagnosis of Wilms' tumour seen at the Hospital for Sick Children, Great Ormond Street, between 1960-1972, two patients developed histologically proven bone metastases. In one patient a lump on the head was the presenting symptom of his Wilms' tumour. In the second patient an isolated metastasis developed in the iliac bone 2 3/4 years after complete removal of an encapsulated Wilms' tumour. The bone lesion was outside the previously irradiated volume. Response to treatment was poor and both patients died with further widespread disease. A review of other series confirms the low incidence of bone metastases from Wilms' tumour and the poor prognosis of this complication. This contrasts with a high incidence of pulmonary metastases in which the response to treatment and subsequent survival is good. The value of skeletal surveys and scans in detecting this relatively rare site of metastases from Wilms' tumour is discussed.

Benign renal tumor in a 16-month-old boy treated with pre-operative radiotherapy

In a 16 months old boy a diagnosis of Wilms' tumour was made by aortography. Radiotherapy was given pre-operatively. After removal of the kidney the histologic examination showed benign dysontogenetic tumour. Preoperative radiotherapy in small children with radiologic diagnosis of nephroblastoma is discussed.

Considerations in the radiation therapy of Wilms' tumor.

A review of 156 children with a diagnosis of Wilms' tumor seen and treated at the CHMC—CCRF from January 1, 1960 to December 31, 1970 is presented. No demonstrable effect of age at presentation on subsequent prognosis could be identified. A staging system is presented which is readily used and correlates well with prognosis. Ninety-one of 156 children survive without evidence of Wilms' tumor. Sixty-eight of 93 (73%) children who received all therapy at CHMC—CCRF survive. Large primary size and major extra-renal vascular involvement did not affect the prognosis within a given stage. Tumor spill noted at operation requires subsequent whole abdominal irradiation to adequate doses to prevent local abdominal recurrence. A correlation of vascular involvement with pulmonary metastases is made. The incidence, sites, time, and treatment of relapse is presented. Optimum survival for the child with Wilms' tumor requires a thorough interdisciplinary approach of surgical, medical, and radiation oncologists. Careful follow-up is necessary so that when relapse occurs, especially in the lungs, it can be detected at the earliest possible time, assuring the maximum possible salvage in these patients.

BILATERAL NEPHROMEGALY IN A CHILD WITH HEMIHYPERTROPHY

A newborn with hemihypertrophy and bilateral palpable abdominal masses due to markedly enlarged kidneys is reported.The kidneys showed intrinsic distortion of the collecting systems by intravenous urography and a preoperative diagnosis of Wilms’ tumor was made. Open wedge biopsies of each kidney evinced normal tissue. Despite large kidneys the patient has remained well for 3 years.

Total-body opacification in the diagnosis of Wilms' tumor and neuroblastoma: A note of caution : Gerald J. Kurlander and Evrett E. Smith. Radiology 89:1075–1076. December 1967

Total-body opacification in the diagnosis of Wilms' tumor and neuroblastoma: A note of caution : Gerald J. Kurlander and Evrett E. Smith. Radiology 89:1075–1076. December 1967

Combined Liver-Kidney Isotope Scans

A combination of chlormerodrin Hg 197 kidney scan and rose bengal sodium I 131 liver scan can facilitate the diagnosis of upper abdominal masses. This technique was used in the diagnosis of Wilms' tumor in a 2-year-old boy.

Total-body opacification in the diagnosis of Wilms's tumor and neuroblastoma. A note of caution.

O'connor and Neuhauser in 1963 described a method of total-body opacification with relatively large doses of intravenously injected radiopaque contrast medium (3). The injected medium mixes with the blood and is uniformly distributed throughout the blood vascular compartment in less than sixty seconds. The added density of the various body tissues is proportional to the blood supply. An avascular or hypovascular lesion will acquire a radiolucency in contrast to adjacent structures with greater blood supply which contain blood mixed with the radiopaque contrast medium. The total-body opacification effect can be produced best in infancy but requires excellent radiographic technic. It is particularly useful in defining cystic, hemorrhagic, and necrotic lesions (2). Although the originators of the technic recognized the limitation of the method in differentiating necrotic or poorly vascularized neoplasms from cysts, others have suggested that the demonstration of an absolutely avascular renal mass in infancy ...

WILMS'S TUMOR AND ASSOCIATED ANOMALIES.

WILMS'S tumor, one of the most common abdominal neoplasms of childhood, has been reported to be associated with certain congenital anomalies. Miller et al1of the National Institute of Health, reviewed a series of 440 cases of Wilms's tumor in association with aniridia, hemihypertrophy, pigmented nevi and hemangiomas, hypospadias, cryptorchidism, horseshoe kidney, and duplication of the upper urinary tract. The more common anomalies associated with aniridia and Wilms's tumor, and the cases reported in the literature, are noted in the Table. A survey by Miller1indicates that only six cases of Wilms's tumor have been reported having aniridia as one of the associated congenital anomalies. The presence of Wilms's tumor with aniridia, cataracts, recurved pinna of the ear, and duplication of the upper urinary tract has occurred in one of our patients. A diagnosis of Wilms's tumor should be suspected and considered in the differential diagnosis when a

WILMS'S TUMOR IN THE ADULT.

THE OCCURRENCE of Wilms' tumor in adults is thought to be rare. In actuality it is not as rare as has been presumed. Esersky et al reviewed the literature in 1947 and found 54 cases to which they added three of their own. 1 By 1960, Smith was able to collect 105 cases from the literature. 2 Although not as frequent as other types of malignancy in the adult kidney, it cannot be considered a rare disease. Some degree of confusion still exists because of the considerable variation in histologic structure, and it has been difficult to evaluate treatment and prognosis in the cases in the literature. This report discusses the disease and treatment in 12 adults observed in one institution. Material From January 1949 to June 1963, a total of 130 patients with a pathologic diagnosis of Wilms' tumor were seen at Memorial Hospital. Of these, 12 (9.2%)

Urologic problems in pediatric x-ray diagnosis.

Functional failure of most organ systems of the body usually gives some indication of the organ at fault. If the heart is abnormal, there are murmurs and perhaps cyanosis; if the gastro-intestinal system is involved, there are vomiting, distention, abnormal stools, etc.; if the endocrine system is affected, there are alterations of body contours, changes in the skin and its appendages, and abnormalities of the secondary sexual characteristics. In addition, there is often an associated failure of growth. But, if the urinary system is defective, the first sign may be only failure to grow, with no reference to the source of the difficulty. The alert pediatrician, confronted with physical retardation in a child without obvious cause, will immediately direct his attention to the urinary tract and call upon his radiologic and urologic colleagues for assistance. The presence of an abdominal mass in infancy and childhood, particularly under the age of five years, involves the differential diagnosis of Wilms' embr...