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35 records · Page 2Linked to original sources
PubMed1976

Gallium-67 scans in children with solid tumors.

Twenty pediatric patients with malignant disease were studied by whole body gallium scans. Seven had Hodgkin's disease, five had soft tissue sarcomas, three had neuroblastomas, two had Wilm's tumors, and one each had a hepatoblastoma, embryonal cell carcinoma, and sacrococcygeal teratoma. Of 50 sites studied, there was a true positive result in 17%, and in 58% there were false negative results. The method served best in patients with Hodgkin's disease, where the true positive rate was 64%. In no instance did the gallium scan affect the clinical management of a patient.

Adolescent↗
PubMedDate not supplied

cis-Dichlorodiammineplatinum(II) in childhood cancer.

cis-Dichlorodiammineplatinum(II) (cis-platinum) was evaluated in three separate studies at Roswell Park Memorial Institute in children and adolescents with cancer. In the first study, 16 patients with a variety of solid tumors were treated. Objective responses were seen in patients with neuroblastoma, osteogenic sarcoma, seminoma, and medullary carcinoma of the thyroid. In the second study, five of eight patients with far-advanced osteogenic sarcoma showed objective responses to cis-platinum. In the third study, cis-platinum and Adriamycin were employed as primary adjuvant chemotherapy along with surgery in osteogenic sarcoma. Nine of ten patients have remained disease-free from 8 to 31 months (mean, 19 months). cis-Platinum is an active agent in pediatric tumors.

Adolescent↗
PubMed2026

Clinical, Histopathological, and Molecular Characterization of Pediatric MN1::ZNF341-Associated Cancer.

A lethal round-cell malignancy with an MN1::ZNF341 fusion has recently been reported in three infants. Here, we describe four further tumors, three in newborns (including monozygotic twins), and one in an adolescent. Detailed clinical, radiological, and histopathological data differentiate these tumors from their main mimics, neuroblastoma and round-cell sarcomas. Single-cell RNA sequencing confirms the tumor to be transcriptionally distinct from neuroblastoma, instead exhibiting steroidogenic differentiation. Whole genome and targeted DNA sequencing yield no further driver events. Our work reveals a broader clinicopathological phenotype than previously appreciated and corroborates suggestions that this tumor is a distinct and aggressive childhood cancer.

Humans↗
PubMed2026

A Clinically Integrated Pediatric Patient-Derived Xenograft Program Enables Evaluation of Cohort and Patient-Specific Biology and Therapeutic Strategies.

UNLABELLED: Preclinical translational research has increasingly utilized patient-derived xenograft (PDX) models for mechanistic and experimental therapeutic studies, yet most existing models have been developed from adult cancer types. We describe the establishment of a PDX program to expand the availability of pediatric-specific PDXs for preclinical research and enable studies of pediatric cancer histologies, including ultrarare diseases. Processes for PDX generation were integrated into established clinical workflows to facilitate universal model generation. Methodologies for tissue procurement, processing, and cryopreservation were optimized to enable intra- and interinstitutional PDX model generation. Over a 6-year span, 388 PDX tumor models representing more than 40 diagnoses were generated, including ultrarare tumors and longitudinal models established from pretherapy, posttherapy, and relapse tumors from the same patient. Genomic characterization of these PDXs demonstrates excellent concordance and recapitulation of molecular alterations of the source tumor. Successful PDX generation was enhanced from relapsed samples, was higher in sarcomas compared with other solid tumor types, and was a negative prognosticator for clinical outcome. With a broad portfolio of molecularly annotated models, we demonstrate utility for validating cross-histology biomarker-driven therapeutic strategies by demonstrating antitumor activity of an MAT2A inhibitor in MTAP-deficient PDXs. Universal model creation also allows for experimental validation of therapeutic hypotheses on a patient-specific basis, as we describe the characterization of a novel RAF1 fusion (EPB41L2::RAF1) in an osteosarcoma PDX. Development of a diverse collection of pediatric PDX models enables hypothesis-driven and cross-histology studies that expand our understanding of cancer biology and aid ongoing drug prioritization efforts in rare tumors. SIGNIFICANCE: A clinically integrated, genomically annotated pediatric PDX portfolio supported by systematic benchmarking of model generation facilitates exploratory biomarker-driven and patient-specific translational studies.

Humans↗
PubMed1978

Computed tomography of tumors of the musculoskeletal system in children. Clinical applications.

Studies indicate that computed tomography (CT) has several important clinical applications in the evaluation of tumors of the musculoskeletal system. These include (a) diagnosis of lesions involving difficult areas such as the sacrum and ilium, (b) demonstration of soft-tissue components of bone tumors or primary soft-tissue masses, (c) assessment of bone-marrow involvement, and (d) follow-up after irradiation or chemotherapy. Several selected pediatric cases are described in detail.

Adolescent↗
PubMed1979

ICRF-159 (razoxane) in the treatment of pediatric solid tumors: a Southwest Oncology Group study.

ICRF-159 is active in several animal tumor model systems and human adult malignancies. In this phase II study, ICRF-159 was given on a weekly schedule, 3000 mg/m2/day, orally in three divided doses at 6-hour intervals to 78 children with a variety of malignant neoplasms. Fifty-three patients were evaluable for tumor response. Toxicity was primarily hematopoietic and gastrointestinal. There were no responses in any of the eight patients with osteogenic sarcoma, four with lymphoma, five with Ewing's sarcoma, ten with neuroblastoma, or six with rhabdomyosarcoma. There was a transient partial response in one of four children with Wilms' tumor. Further trials with this drug using this schedule are not indicated for the common childhood solid tumors.

Adolescent↗
PubMed1975

Lymphography in childhood: six years experience with 242 cases.

From January, 1969 - December, 1974, 242 children less than 15 years of age underwent lymphography at the National Cancer Institute, Milan. Successful lymphatic cannulation was accomplished in 97% (440/463) of the sites where it was attempted. No major or permanent complications were encountered, although minor untoward effects might not have been recorded. In those children undergoing biopsy of opacified lymph nodes, lymphographic-histologic correlation was 98% (45/46). Nonspecific reactive hyperplasia lymphographic patterns were encountered in 36% of all studies, confirming its high incidence in the pediatric age group. This study has shown that lymphography in childhood can be as readily performed as in the adult and that its diagnostic accuracy is acceptable. As in adults, it is useful in treatment planning, evaluating results of therapy, and detecting recurrent tumor. The frequent occurrence of nonspecific reactive hyperplasia in the pediatric lymphogram should not be mistaken for evidence of tumor, particularly lymphoma.

Adolescent↗
PubMedDate not supplied

[Intrathoracic tumors in children].

Intrathoracic tumors in pediatric ages are unfrequent. A review of 138 cases in patients under 15 years of age showed in the first place, the same as in adults, malignant neoplasms represented by pulmonary metastases. However, in those of primary localization, the histologically benign are more frequent. Out of 69 cases, such tumors came up to 78.2% and 47% of them were cystic malformations. Among malignant primary neoplasms, out of 30.4%, neuroblastoma showed 8.6%, diverse sarcomas were 8.6% and the epithelial type added up to 4.3%. The main symptoms were: frequent bronchopulmonary infections, obstructive respiratory insufficiency and vascular stasis. Radiological examinations are of great diagnostic aid, but a full clinical evaluation is always necessary. A surgical treatment removing the mass leads to radical recovery leaving limited sequelae. The comparative group of 69 cases with pulmonary metastases showed that neoplasms considered of embryonal type are practically always multiple and appear during the final stage of the disease. Surgery may be attempted when they are single and the primary has been removed; however, chemotherapy and radiotherapy are always necessary.

Adenoma↗
PubMed1979

Ultrasound-guided percutaneous nephrostomy in the pediatric patient.

Percutaneous nephrostomy drainage may be established rapidly using ultrasonic guidance in severely ill pediatric patients with obstructive uropathy. B-mode ultrasonography has proved to be rapid, accurate and free of irradiation hazards, making it applicable to children with little or no sedation.

Adult↗
PubMed1977

Radionuclide skeletal survey for pediatric neoplasms.

A prospective study comparing the radionuclide skeletal survey with the radiographic survey was performed for 159 children with known primary neoplasm. Forty-four showed radionuclide evidence of metastases, while only 14 had radiographic evidence. Thus, 68% of metastases were discovered by bone imaging alone. No false negative images for metastases were obtained, though there was one false negative image for primary tumor. Radionuclide skeletal survey is recommended as the primary evaluation for bony metastases in all children with neoplasms. Suspect areas should then receive complete radiographic examination.

Adolescent↗
PubMed1977

Patterns of second malignant neoplasms in children.

A search of the records of 10 pediatric oncology centers revealed 102 children with more than one malignant neoplasm. In this group of 102 patients, all pediatric cancers were seen as initial lesions, but Wilms' tumor and retinoblastoma were over-represented and leukemia and brain tumors underrepresented. Survival variation as well as tumor susceptibility may be responsible for this disproportion. Osteosarcomas and chondrosarcomas were the most frequent second malignant neoplasms (SMN). Embryonal tumors were rare as SMN and adult-type tumors (carcinomas) appeared at earlier than expected ages, whether arising after irradiation or not related to that form of therapy. Radiation was associated with 69 SMN, genetic disease accounted for 27 SMN and both conditions were noted in 15 SMN. In the group of 21 patients for whom neither radiation nor a known genetic disorder could be implicated, there were three with colon carcinoma and glioma and five with leukemia or lymphoma and glioma. These combinations may reflect new tissue-specific hereditary cancer syndromes.

Adolescent↗
PubMed1979

[Behavior of phosphoisomerase and lactate dehydrogenase in pediatric oncological pathology].

Two glycolytic enzymes, PHI and LDH, have been evaluated in 18 children affected by leukemia or solid tumors: 11 patients had just initiated therapy, 3 patients were about to initiate therapy, while 4 patients were out of therapy. The analysis of the data obtained has shown a good correlation with the course of the disease: we have found values above the normal range in patients with a favorable course of the disease (bone marrow relapse or CNS involvement in leukemic children; relapse or metastasis in solid tumors) almost always before it was possible to demonstrate by clinical and laboratory studies the inhanchement of tumoral cells growth. This was true in all patients except two children affected by neuroblastoma, who were in a favorable immunological status (presence in the serum of free specific antibodies), and who were out of therapy. In these patients the abnormal high values of PHI were interpretated as an index of necrotic phenomena of micrometastasis of tumor cells induced by specific committed T-lymphocytes. Values of PHI and LDH in the normal range were found in patients whose disease demonstrated a favorable course. The AA. suggest the introduction of these enzymatic parameters which may be a useful index of the efficacy of the chemotherapy in the follow-up of oncologic patients.

Child↗
PubMed1979

Evaluation of bone-marrow scanning with technetium-99m sulfur colloid in pediatric oncology.

Eighty-six technetium-99m sulfur colloid (Tc-SC) bone-marrow scans in 56 pediatric oncology patients were reviewed. The distribution of the sulfur colloid was similar to that in adult bone marrow in normal children older than 10 yr, and involved progressively more marrow of the extremities in normal children under 10 years of age. After irradiation or chemotherapy there was an extension of the Tc-SC to peripheral marrow sites. There was also diminished uptake of the tracer in sites corresponding to irradiated areas. In most patients there was recovery of these defects by 6 mo after completion of therapy. Tumor replacement of the marrow was reflected in the scans, and the extent of the scan defect paralleled the course of the disease. In four patients, despite normal bone scans and radiographs, marrow-scan abnormalities due to tumor replacement were present and confirmed by needle aspiration and/or biopsy. In two other patients, the marrow-scan abnormality preceded radiographic and histologic evidence of tumor metastasis. Two patients who responded clinically showed persistent defects; biopsy in one revealed fibrosis. Technetium-99m sulfur colloid bone-marrow scanning appears to be a sensitive monitor of marrow alteration caused by metastases, irradiation damage, or tissue fibrosis in children receiving treatment for cancer.

Adolescent↗