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Biomedical subjects

J Chatten

Publications and source records attributed to J Chatten.

At least 55 records · Page 3Linked to original sources

Hydropic cell variant (clear cell variant) of Wilms' tumor.

We report an unusual clear cell tumor of the kidney in infancy. The presence in our patient of a discrete mass with foci of primitive nephroblastic cells indicates the neoplastic nature of the clear cells. We discuss its distinction from clear cell sarcomas and carcinomas, and we suggest that this lesion may be inadequately separated from pediatric renal carcinomas in the literature. We recommend the term hydropic cell variant as a less confusing and more accurate descriptive designation.

Adenocarcinoma

HLA class I and beta 2-microglobulin expression in frozen and formaldehyde-fixed paraffin sections of neuroblastoma tumors.

Ten solid neuroblastoma tumors were examined for beta 2-microglobulin (beta 2-m) and HLA-class I expression in an immunocytochemical assay. Blood vessel endothelium and a small population of cells (less than 6% of small round cells) were consistently stained in frozen sections of each tumor. No beta 2-m or HLA-class I reactivity was detected in the remainder (greater than 94%) of the small round cells in each tumor. The localization of most of the positive cells near stromal tissue and blood vessels suggests that most of these cells may be of non-tumor origin. The patients from which the tumors were taken varied in degree of disease involvement, yet no differences with respect to beta 2-m and HLA-class I expression were found among the tumors. A procedure for visualizing beta 2-m and HLA-class I molecules in formaldehyde-fixed, paraffin-embedded tissue is described. This confirmed the results in frozen sections. These findings extend previous reports of weak beta 2-m and HLA-class I levels in cells of neuronal origin. The clinical implications are discussed.

Child

Histopathologic prognostic factors in neuroblastic tumors: definition of subtypes of ganglioneuroblastoma and an age-linked classification of neuroblastomas.

Histopathologic prognostic factors of 295 pretreatment tumors of a total 641 neuroblastomas and ganglioneuroblastomas were studied with the use of the following proposed tumor classification. The tumors were divided into 2 groups: stroma-poor (235 cases) and stroma-rich (60 cases) according to their organizational pattern (stromal development). The stroma-poor group was classified further into 2 subgroups: favorable stroma-poor (84% survival) and unfavorable stroma-poor (4.5% survival) according to the patient's age at diagnosis, degree of maturation, and nuclear pathology [mitosis-karyorrhexis index (MKI)] of the neuroblastic cells. The stroma-rich group was further classified into 3 subgroups: well differentiated (100% survival), intermixed (92% survival), and nodular (18% survival) on the basis of morphology of the immature element in the tumor tissue without regard to patient's age or quantitative maturation. Favorable stroma-poor and well-differentiated and intermixed stroma-rich groups seem to make good prognosis groups (87% survival), which show gradual progression along a maturational sequence according to the age of the patient. Unfavorable stroma-poor and nodular stroma-rich groups form poor prognosis groups (7% survival) and show morphological evidence of malignant or aggressive behavior, such as inappropriate immaturity for age, higher MKI, and gross nodule formation by immature neuroblasts.

Adrenal Gland Neoplasms

Extraskeletal osteogenic sarcoma after treatment for Wilms' tumor.

A large proportion of children with Wilms' tumor will become long-term disease-free survivors. A small number of these children are at risk of developing second malignant neoplasms. There have been no previous reports of osteogenic sarcoma of the chest wall following treatment of Wilms' tumor. Our patient was age seven years when he received surgery, radiation therapy and chemotherapy for Wilms' tumor, eight years when he received radiation and chemotherapy for pulmonary metastases of Wilms' tumor, and 13 years when he developed osteogenic sarcoma of the chest wall.

Child

Cancer in neonates: the experience at the Children's Hospital of Philadelphia.

Among 22 neonates treated at the Children's Cancer Research Center of Philadelphia, 11 had neuroblastoma, which in two cases was widely metastatic. There were three infants with teratoma, three with sarcoma, three with leukemia, one with Wilms' tumor, and one with parotid carcinoma. Nine of eleven patients (82%) are long-term survivors following complete surgical excision of tumor, whereas only one of eight (13%) has survived following incomplete surgical excision. All three neonates with leukemia died. The overall two-year actuarial survival is 45% (10/22). The problems associated with treating neonates with chemotherapy, radiation therapy, or both are especially difficult because of the immaturity of the organs and structures. Surgical excision alone has been the treatment of choice for solid tumors. Chemotherapy or radiation therapy, when indicated, require careful monitoring for both acute toxicities and potential long-term morbidities.

Female

Fibrous lesion of the bowel complicating the management of bilateral Wilms' tumor.

A 31 month old black male with known bilateral Wilms' tumor, who had right nephrectomy and left lower pole heminephrectomy developed a palpable mass in the left lower quadrant. Several studies could not clearly determine the nature of the mass lesion, a laparotomy was performed, a keloid-like lesion was found in the bowel wall.

Child, Preschool

Acute pancreatitis and increased intracranial pressure.

A retrospective review of 41 patients with post-mortem evidence of acute pancreatitis revealed that 19 patients (46%) showed concomitant evidence of increased intracranial pressure (ICP). A prospective analysis of five patients with increased ICP demonstrated two patients with evidence of acute pancreatitis. The pathogenesis of acute pancreatitis in these patients appears to be dependent on the following risk factors: increased intracranial pressure, steroids, hypovolemia, morphine infusion and hypothermia. These factors increase vagal stimulation, predispose to pancreatic duct obstruction and enhance cellular hypoperfusion. Acute pancreatitis occurring in Reye's Syndrome is probably a consequence of increased ICP and the therapy instituted.

Acute Disease

Treatment strategies for infants with malignant sacrococcygeal teratoma.

Twelve children with malignant sacrococcygeal teratoma containing embryonal carcinoma, were treated at the Children's Hospital of Philadelphia between 1971 and 1980. Their ages at diagnosis ranged from 2 days to 23 mo; 8 of the 12 (67%) were girls. Five of the 12 patients presented with localized tumors which were grossly completely excised. Four received no further therapy, and all 4 recurred with histologically documented embryonal carcinoma. Despite subsequent treatment with radiation therapy (RT) and chemotherapy (vincristine, actinomycin D, and cyclophosphamide, collectively called VAC, in all 4 plus adriamycin in 3, only 1 has survived free of disease, 8 yr from diagnosis. The other 3 died of tumor (2) or pneumonia after pulmonary RT (1). The fifth patient in this group received VAC with adriamycin (total dose, 350 mg/sq m) and died of autopsy-proven cardiotoxicity without tumor. The remaining 7 patients presented with either unresectable local tumor (3) or distant metastases (4). One of these 7 died 6 days after biopsy with massive liver metastases. The other 6 children were treated after operation with VAC (5) or vincristine and actinomycin D (1) chemotherapy, and 5 also received RT to the pelvis. One has survived free of disease at 6 yr after treatment with VAC and adriamycin and pelvic RT, but the other 5 died of tumor (2) or of pneumonia after pulmonary RT (3). Surgery alone is inadequate for successful management of children with malignant sacrococcygeal teratoma. Chemotherapy with VAC, with or without adriamycin, can be effective when combined with radiation, but fatal pulmonary toxicity can result from such combined therapy. The optimal therapeutic program for children with malignant sacrococcygeal teratoma is still evolving.

Coccyx

Multilocular renal cysts: radiologic-pathologic correlation.

Multilocular renal cyst, a well-encapsulated benign lesion consisting of multiple noncommunicating cysts, is an uncommon entity of uncertain nature. A spectrum of histologic findings and multiple theories of pathogenesis have resulted in numerous names for the lesion, including multilocular cyst, benign cystic nephroma, cystic hamartoma, cystic lymphangioma, cystic Wilms tumor, and Perlmann tumor. We believe these names, and others, refer to the same entity, namely multilocular cyst. Radiographically a multicystic renal mass which is often calcified and which may partially protrude into the renal pelvis is seen. Although angiography cannot definitively exclude a malignant tumor, a distinctive sonographic appearance-multiple cystic masses separated by highly echogenic septa-is highly suggestive of multilocular renal cyst. Inclusion of this lesion in the preoperative differential diagnosis of a solitary and presumed malignant renal mass may have significant therapeutic implications.

Adolescent

A review of 17 IV-S neuroblastoma patients at the children's hospital of philadelphia.

The records of 207 neuroblastoma patients seen at the Children's Hospital of Philadelphia between 1944 and 1977 were reviewed to study some of the features associated with the unusually good prognosis found in patients with Stage IV-S neuroblastoma. Initially, 22 patients appeared to fit the criteria of small primary tumor and distant disease in liver, skin, and/or marrow without evidence of bone metastases; 5 patients were subsequently rejected as being incorrectly staged. The remaining 17 patients had abdominal primary tumors and hepatic disease; in 12 of the 17, an enlarged liver was the presenting sign. Six patients had skin lesions, 4 had disease in the marrow on routine smear, and additional sites of spread were pancreas and bowel serosa. The treatment given was not systematic, and it was not possible to correlate any specific form of therapy with a satisfactory outcome. Eleven of 17 patients survived; 6 of 11 survivors had spontaneous regression of all or part of their diseases, 5 of 6 who died received irradiation, chemotherapy, or both. Death usually occurred in the first month as a complication of the local disease; 1 patient succumbed to radiation nephritis. This study establishes that the special pattern of widespread neuroblastoma termed Stage IV-S does exist, and that is associated with a good prognosis. Careful consideration should be given before selecting treatment for the Stage IV-S child because spontaneous regression is likely to occur in most of them. In patients with rapidly enlarging livers, renal or pulmonary complications may develop because of liver bulk or coagulopathies. Treatment should be directed to the liver in these cases because distant metastases seldom supervene. Low-dose irradiation, mild chemotherapy, and possibly surgical release of intraabdominal pressure using a silastic patch have all been effective. Unfortunately, patients occasionally succumb to local disease in spite of these and more aggressive measures.

Adrenal Gland Neoplasms

Ossifying tumor of infantile kidney: report of two cases.

Two patients, both male infants, presented with hematuria and were found to have small calcified intrarenal masses interpreted as calculi radiographically. These were found at surgery to be firmly adherent to parenchyma. Dense, stone-like cores, after decalcification, were found to be composed of bone, while the periphery was composed of uniform, undifferentiated cells. We believe these tumors were derived from urothelium rather than the metanephros, and that they represent yet another benign variant of pediatric renal tumors.

Diagnosis, Differential

Pulmonary manifestations of juvenile rheumatoid arthritis. A report of eight cases and review.

Pleuropulmonary disease was seen in 4 per cent of patients with juvenile rheumatoid arthritis. Roentgenographic abnormalities seen in association with juvenile rheumatoid arthritis include: transient pneumonitis, interstitial reticular and nodular infiltrates, pleural and pericardial effusions, and patchy pleural infiltrates. Pathologic abnormalities seen in association with juvenile rheumatoid arthritis include pulmonary hemosiderosis, lymphoid follicular bronchiolitis, and lymphocytic interstitial pneumonitis. Patients with juvenile rheumatoid arthritis and pleural disease recover fully. In children with parenchymal disease, residual abnormalities include roentgenographic evidence of interstitial fibrosis and minimal abnormalities of pulmonary function.

Arthritis, Juvenile

Coarctation resection in children with Turner's syndrome: a note of caution.

Eight children were recognized to have Turner's syndrome, among 353 patients over 1 year of age who had undergone surgical treatment for coarctation of the aorta. Of these eight children, three developed a significant perioperative hemorrhage from aortic rupture, resulting in one death and one instance of paraparesis related to a period of prolonged hypotension. In two of the other five patients with Turner's syndrome, a decision was made to perform an angioplasty rather than a resection of the coarctation because of apparent friability of the aortic wall. In contrast, only one of the 345 patients without Turner's syndrome died as a result of surgical treatment, and none developed spontaneous perioperative aortic rupture or neurologic deficit. This experience suggests that the operative risk for coarctation of the aorta in this subgroup of patients is considerably greater than that in patients without Turner's syndrome (p < 0.001). Special precautions should include use of rubber-jaw vascular clamps, choice of technique to avoid tension at the anastomotic suture line, and careful control of systemic blood pressure intraoperatively and postoperatively. Indications for surgical treatment of coarctation as well as the type of operative procedure must be individualized cautiously in patients with Turner's syndrome.

Adolescent

Nodular renal blastema in trisomy 13.

The autopsy records and material of 14 cases of karyotype-proved trisomy 13 were reviewed. There were one or more foci of nodular renal blastema in six cases. Patients with nodular renal blastema had lived up to five days; patients in whom foci were not found at autopsy had lived six to 37 days. The presence of nodular renal blastema did not substantially correlate with other renal anomalies or the number or severity of extrarenal anomalies. The presence of nodular renal blastema in trisomy 13 expands the spectrum of syndromes associated with nodular renal blastema and supports its relationship with disorders of growth regulation. The presence of the lesions only in very young infants suggests that most, or all, foci of nodular renal blastema regress with age.

Abnormalities, Multiple

Mesenchymal hamartoma of the liver in infants.

Mesenchymal hamartoma of the liver is a rare benign tumor of childhood which has been confused with various other benign liver lesions, particularly vascular hamartomas which are mesodermal but not mesenchymal. The mass, frequently cystic, is characterized by an admixture of epithelial structures in a loose connective tissue stroma with fluid accumulation suggestive of lymphangiomatous channels. Four patients are presented and reviewed with 25 previously reported cases. The patients, usually asymptomatic, present during the first two years of life with progressive abdominal distention, which may be rapid because of increasing fluid content in the connective tissue stroma and cysts. Exploration and biopsy may be necessary before definitive excision. The present cases include a newborn, the youngest known, and a case in which radiation resulted in hyalinization of the mesenchyme, decrease in fluid content, and easlier resection. Respiratory distress and signs of vena caval obstruction due to intra-abdominal pressure were noted. Prognosis after extirpation is very good.

Diagnosis, Differential