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Biomedical subjects

E S Baum

Publications and source records attributed to E S Baum.

At least 19 recordsLinked to original sources

Therapy-related myelodysplastic syndrome and acute myeloid leukemia in children: correlation between chromosomal abnormalities and prior therapy.

We have studied 20 children with therapy-related myelodysplastic syndrome (MDS) or acute myeloid leukemia (AML) who were 3 months to 16 years old at diagnosis of their primary neoplasm and 1 to 24 years old at diagnosis of their secondary neoplasm. The median interval from initial treatment for the first malignancy to diagnosis of therapy-related MDS or AML was 46 months (range, 12 to 116 months). Twelve patients had chromosomal abnormalities resulting in loss of material from the long arm of chromosomes 5 and/or 7, three patients had abnormalities of chromosome 11 band q23, one patient had both classes of abnormalities, three patients had other abnormalities, and one patient had a normal karyotype. Ten of 12 patients with chromosome 5 and/or 7 abnormalities had been exposed to an alkylating agent, and two of three patients with 11q23 abnormalities had been exposed to an epipodophyllotoxin. The patient with both classes of abnormalities had been exposed to both types of therapy. We conclude that abnormalities of chromosomes 5 and/or 7 are common in children with therapy-related MDS or AML. The proposed relationships between exposure to alkylating agents and abnormalities of chromosomes 5 and/or 7 and between exposure to epipodophyllotoxins and abnormalities of 11q23 are supported in this pediatric series.

Adolescent

Therapeutic radiation at a young age is linked to secondary thyroid cancer. The Late Effects Study Group.

We estimated the risk of thyroid cancer among 9170 patients who had survived 2 or more years after the diagnosis of a cancer in childhood. As compared with the general population, patients had a 53-fold increased risk (95% confidence interval, 34-80). Risk increased significantly with time since treatment for the initial cancer (P = 0.03). Detailed treatment data were obtained for 23 cases and 89 matched controls from the childhood cancer cohort. Sixty-eight % of the thyroid cancers arose within the field of radiation. Radiation doses to the thyroid of greater than 200 cGy were associated with a 13-fold increased risk (95% confidence interval, 1.7-104). The risk of thyroid cancer rose with increasing dose (P less than 0.001), but this was derived almost entirely from the increase from less than 200 to greater than 200 cGy. The risk of thyroid cancer did not decrease, however, at radiation doses as high as 6000 cGy.

Adolescent

Carboplatin in childhood brain tumors. A Children's Cancer Study Group Phase II trial.

Between October 1985 and March 1988, Children's Cancer Study Group institutions entered 95 patients with recurrent brain tumors into a Phase II trial of carboplatin 560 mg/m2 every 4 weeks. Complete or partial responses were observed for one of 19 evaluable children with brainstem glioma, two of 14 with ependymoma, six of 19 with medulloblastoma or central nervous system primitive neuroectodermal tumor (PNET), and none of 15 with high-grade astrocytoma. Of 33 children with medulloblastoma, ependymoma, or central nervous system PNET, five of 12 with no prior cisplatin exposure had responses, and two of 21 with prior cisplatin exposure had responses (P = 0.03). Thirty-four percent of patients had absolute neutrophil count nadirs less than 500/microliters, and 37% had platelet count nadirs less than 25,000/microliters. Sixteen percent had moderate to severe otoxicity, 10% had nausea and vomiting, and none had nephrotoxicity.

Adolescent

A phase II study of cisplatin therapy in recurrent childhood brain tumors. A report from the Childrens Cancer Study Group.

Thirty-six children with brain tumors were treated with surgery, radiation and/or adjuvant chemotherapy. After tumor recurrence, cisplatin (60 mg/m2/day IV X 2) was given every three to four weeks. CT scans were used to measure drug response prior to the first, third and fifth courses. Complete and partial responses were demonstrated in nine of 31 evaluable patients. Dose limiting toxicities were renal and auditory. Seven patients developed the syndrome of inappropriate antidiuretic hormone secretion. This study confirms that cisplatin is active in a spectrum of brain tumors.

Adolescent

The relationship of various aspects of surgical management to outcome in childhood nonmetastatic osteosarcoma: a report from the Childrens Cancer Study Group.

Recently, much attention has been focused on the role of adjuvant chemotherapy in the treatment of osteosarcoma. Surgery, however, remains the primary modality for the ablation of this disease. In this report, we examine the relationship of various aspects of surgical management of osteosarcoma to prognosis for disease-free survival (DFS) in a randomized study of 234 pediatric patients. Attention is restricted to 166 patients with nonmetastatic disease confined to an extremity and who were randomized to receive one of two chemotherapeutic regimens. No advantage with respect to DFS was attributable to the various aspects of surgical management considered: interval from first symptoms to definitive surgery, surgical sequence, and type of surgery. Only two local recurrences were seen. One occurred in an above knee amputation stump and the other occurred in a patient receiving a tibial allograft. One of these patients died of pulmonary metastases within 6 months of recurrence; the other patient is alive without evidence of disease at last contact after resection of the recurrence followed by chemotherapy.

Adolescent

Nephroblastomatosis: comparison of CT with US and urography.

A review of the imaging studies of five children with Wilms tumor and nephroblastomatosis demonstrated that contrast-enhanced computed tomography (CT) can show the multifocal involvement of nephroblastomatosis more accurately than either excretory urography or real-time ultrasound (US). In all five children, CT demonstrated multiple areas of nephroblastomatosis along the renal cortex. US showed a single intrarenal mass in each child (one bilaterally) but did not show the cortical nephroblastomatosis. Excretory urography also demonstrated only the dominant mass of the Wilms tumor. The superiority of CT is attributed to better spatial resolution and soft-tissue contrast differentiation and clear demarcation between nonenhanced nephroblastomatosis and enhanced normal renal parenchyma. Contrast-enhanced CT is therefore recommended for the evaluation of clinically abnormal and contralateral kidneys in all children with suspected Wilms tumor.

Child, Preschool

Microcomputer-assisted data management for multiinstitutional pediatric clinical cancer trials.

Microcomputer assisted data management techniques, utilized in the oversight of two multiinstitutional trials for children with previously untreated acute lymphoblastic leukemia and unfavorable prognostic features, are presented. In the first study, such oversight hastened identification of unexpectedly prolonged delays, so that treatment could be successfully modified. In the second study, inferiority of one therapy was rapidly demonstrated, and after careful review that therapy was halted. Performance monitoring is discussed in terms of consideration of the overall quality of a clinical trial, namely, how complete are the data submitted and how precisely was protocol therapy administered; measurement of the quality of institutional participation, and identification of deficiencies in reporting or performance in the records of individual patients.

Acute Disease

Pediatric phase I trial of carboplatin: a Childrens Cancer Study Group report.

Carboplatin is one of a series of cisplatin analogs now undergoing clinical investigation. Phase I and II trials in adults demonstrate activity in a number of human cancers and less toxicity than might be expected with the parent compound. This phase I trial was undertaken to establish the maximum tolerated dose and the recommended phase II dose in children treated by a 1-hour iv infusion every 4 weeks. Twenty-nine patients with recurrent or progressive tumor were entered in this study at the Children's Hospital of Los Angeles and Children's Memorial Hospital in Chicago between April 12, 1983, and November 27, 1984. Beginning with a dose of 350 mg/m2 (about 80% of the adult phase II dose), we escalated the dose in groups of patients to 670 mg/m2; dose-limiting myelosuppression was encountered at this dose. Fifty-seven infusions are at least partially evaluable for toxicity. Asymptomatic hypomagnesemia, hypocalcemia, and ototoxicity were observed infrequently, and nausea and vomiting were mild. One patient with a mixed glioma of the posterior fossa achieved a good partial response lasting 9 months. Stable disease for greater than or equal to 6 months was observed in three patients: one each with ependymoma, brain stem glioma, and spinal cord astrocytoma. The recommended pediatric phase II dose is 560 mg/m2 given as a 1-hour iv infusion every 4 weeks.

Adolescent

Recurrence of Wilms tumor after apparent cure.

The recurrence of Wilms tumor after a 5-year disease-free interval is rare. We present two patients who had recurrent disease after a disease-free interval of greater than 7 years. Three additional patients, registered with the National Wilms Tumor Study who had a recurrence after 5 years, are also described. Of these three patients, two had nephroblastomatosis. Because more patients are achieving long-term survival, careful surveillance after apparent "cure" is recommended, particularly if nephroblastomatosis is identified in the original nephrectomy specimen.

Child, Preschool

Primary cutaneous T-cell lymphoma in a child.

Primary cutaneous lymphoma in childhood is extremely rare. An 11-year-old girl had lesions that were treated as infected insect bites until a biopsy was performed and the diagnosis of primary T-cell lymphoma was made. In contrast to the typically indolent course of cutaneous T-cell lymphoma in adults, the condition in children tends to disseminate rapidly.

Adolescent

Renal sarcomas of childhood. A clinicopathologic and ultrastructural study.

Of 230 consecutive primary renal tumors of childhood collected over a 26-year period, 19 (8.2%) were sarcomas. The histologic criteria devised by the National Wilms' Tumor Study for diagnosis of "sarcomatous variants" of Wilms' tumor could be applied to all but three cases, namely, a rhabdomyosarcoma, a spindle-celled tumor, possibly leiomyosarcoma, and an unclassified primitive tumor. The most prevalent subtype was the bone-metastasizing, "clear cell" sarcoma. Correlative histologic and ultrastructural study supports the concept of subsets of tumors within the category now called "sarcomatous Wilms' tumor." Although this designation has the practical value of alerting the oncologist to the need for aggressive therapy, an origin in nephrogenic mesoderm cannot be cogently proposed for all of these tumors. This study also shows that the histologic features of the different subtypes of renal sarcomas may overlap. Hence, electron microscopy is indispensable for appropriate subclassification of these tumors.

Bone Neoplasms

Continuous infusion of 5-azacytidine as induction for acute nonlymphocytic leukemia in patients with previous exposure to 5-azacytidine.

5 children with acute nonlymphocytic leukemia in relapse who had received 5-azacytidine as a part of previous multiagent chemotherapy received continuous infusion of 5-azacytidine, 150-200 mg/m2/day, for 5-7 days every 2 weeks. 2 achieved remission marrows with good peripheral counts (duration 1 and 3 months). 1 achieved a transient remission marrow but remained pancytopenic and 1 achieved reduction from 91% blasts to 18% blasts in the marrow. Principal toxicities were severe myelosuppression, diarrhea, and phlebitis.

Adolescent

Wilms' tumor.

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Child

Bleomycin-induced pulmonary fibrosis mimicking recurrent metastatic disease in a patient with testicular carcinoma: case report of the CT scan appearance.

Pulmonary fibrosis is a well-recognized complication of bleomycin therapy. Detection of bleomycin-induced pulmonary fibrosis by computed axial tomographic scanning (CT) has not been reported. We report on a patient in whom the development of lesions on chest CT scan following cessation of chemotherapy (including bleomycin) was interpreted as representing recurrent metastatic disease. At bilateral thoracotomy, only pulmonary fibrosis was found. In this patient, the CT scan appearance of bleomycin-induced pulmonary fibrosis mimicked recurrent disease.

Adolescent

Current trends in treatment of childhood rhabdomyosarcoma of lower genitourinary tract.

Treatment of childhood embryonal rhabdomyosarcoma of the lower genitourinary tract is in the process of undergoing drastic changes. Extensive surgery with excision of the lesion and its contiguous structures leaving the patient with significant physical disability is being replaced by biopsy and aggressive combined chemotherapy along with radiation therapy. Although it would appear that survival may be improved by this approach, caution is recommended and extirpation advised should the tumor fail to respond rapidly to chemotherapy.

Biopsy, Needle