Crucial questions for health professionals include what constitutes social support, the sources of social support, and the parameters of social support. The preliminary findings from a research project that focused on the care of children dying of cancer indicate that these questions have concerned primary care nurses and physicians. Data acquired in personal interviews administered to 27 physicians and 32 nurses who participated in the first year of the Home Care for the Child with Cancer project provided descriptions of individual sources of social support. The results reveal a range of supportive mechanisms and networks within varied institutional contexts. While there were some within-group similarities in the forms of social support used by nurses and those used by physicians, numerous individual differences were evident. This is an area for extensive further study.

Child↗

PubMed1981

Sarcomas of bone in childhood: pathologic aspects.

Some sarcomas of bone are not characteristically childhood tumors. Within the context of osteosarcomas, telangiectatic and parosteal (juxtacortical) variants deserve recognition. Among chondrosarcomas, which ordinarily are not tumors that occur in children, the mesenchymal variant should be recognized in children especially, this lesion may be extraosseous. Fibrous histiocytoma and its malignant variant have recently emerged as morphologically specific lesions, occasionally osseous, among other spindle cell tumors. Ewing's sarcoma, which is predominantly of osseous origin, consists of undifferentiated fragile cells. The elaborate spectrum of topographic, cytologic, nuclear, and other morphologic features that fall within the designation Ewing's sarcoma are described.

Adolescent↗

PubMed1981

Use of monoclonal antibodies, morphology, and cytochemistry to probe the cellular heterogeneity of acute leukemia and lymphoma.

A combined immunological, morphological, and cytochemical approach to the study of malignant cells in patients with acute leukemia and lymphoma is presented. Newly produced monoclonal antibodies that bind to antigens of human mononuclear cells (TA-1), or B-lymphocytes (BA-1) were used to study malignant cells from patients with acute lymphoblastic leukemia (ALL). acute myelocytic leukemia, acute myelomonocytic leukemia, and chronic lymphocytic leukemia. Results in lymphoid leukemia-lymphoma patients were compared with other immunological markers and indicate that the major groups of ALL and childhood non-Hodgkin's lymphoma are T-ALL, pre-T-ALL, pre-B-ALL, B-ALL, and non-T, non-B-ALL. In addition, each major group had multiple phenotypes when analyzed with seven immunological markers including the erythrocyte rosette receptor, surface immunoglobulin, cytoplasmic immunoglobulin M, the early lymphocyte-acute lymphoblastic leukemia antigen, monoclonal antibody TA-1, monoclonal antibody BA-1, and a monoclonal antibody against HLA-DR. While immunological heterogeneity was demonstrable within each group, distinct biological behavior was observed, with T-ALL and B-ALL generally presenting as "lymphomas" and the others presenting as "leukemias." Morphological analysis using the French-American-British classification provided independent information in the definition of groups with differing clinical behavior. Cytochemical analyses demonstrated focal paranuclear staining of leukemia cells with acid phosphatase in 73% of T-ALLs and 6% of non-T, non-B-ALLs.

Acute Disease↗

PubMed1981

Chromosomal abnormalities in acute lymphoblastic leukemia.

Chromosome abnormalities in acute lymphoblastic leukemia (ALL) and their possible clinical significance are briefly reviewed based upon the literature and 60 cases studied at the University of Minnesota. Almost all cases of ALL appear to demonstrate clonal abnormalities; the major abnormal clone is usually hyperdiploid or pseudodiploid. Among cases of non-T, non-B ALL, at least four translocations appear to be present with an increased frequency: t(9;22); t(4;11); t(11;14); and t(1;3). Patients with these translocations appear to have unique clinical and laboratory findings. Although the presence of abnormal clones does not seem to influence remission duration, the nature of the abnormality does. Patients whose leukemias demonstrate predominantly a pseudodiploid abnormal clone or a translocation have significantly shorter first remissions. Most importantly, among patients with non-T, non-B ALL, the presence or absence of translocations may separate poor responders from good responders.

Acute Disease↗

PubMed1981

Multimodal therapy for the management of primary, nonmetastatic Ewing's sarcoma of bone: an Intergroup Study.

A randomized study of 264 children and adults with previously untreated localized Ewing's sarcoma of bone was undertaken between 1973 and 1978 by 83 institutions of three national study groups: Children's Cancer Study Group, Southwest Oncology Group, and Cancer and Leukemia Group B. The Intergroup Study was designed to determine if the addition of adriamycin (ADR) or bilateral pulmonary radiotherapy (RT) to vincristine, dactinomycin, and cyclophosphamide (VAC therapy) would improve survival and reduce local recurrences and metastases. All patients received RT to the primary lesion, and the survival rate after 3 years was 65%. The most effective treatment regimen was VAC plus ADR; 74% of the patients were free of disease at 2 years. The lengths of disease-free status and survival of patients treated with VAC plus ADR or VAC plus RT did not differ. However, both regimens were significantly superior to treatment with VAC alone. The addition of ADR or bilateral pulmonary RT to VAC was highly advantageous to patients with nonpelvic primaries. Bone and lung were the major sites of distant relapse, but the addition of bilateral pulmonary RT showed no advantage over that of ADR in reducing the occurrence of lung metastases. These recent results should eliminate some of the pessimism that has accompanied a diagnosis of Ewing's sarcoma, although distant metastases continued to be a major reason for failure in the control of this tumor. Survival of these patients can be improved through well-controlled clinical trials designed to determine optimal adjuvant chemotherapy and treatment of the primary lesion.

Adolescent↗

PubMed1981

Radiation therapy in the multimodal management of Ewing's sarcoma of bone: report of the Intergroup Ewing's Sarcoma Study.

This paper is a progress report on the role of radiation therapy (RT) in local tumor control and the decreased incidence of pulmonary metastasis in 251 patients entered in the Intergroup Ewing's Sarcoma Study. All were followed for more that 1 year, and their RT records were reviewed. Doses to the primary tumor in the range of 4,500--6,500 rad were administered over approximately 5 to 6 weeks in combination with 4 drugs, i.e., vincristine (VCR), dactinomycin (DAC), cyclophosphamide (CY), and adriamycin, or only the first 3. One group of patients received the 3 drugs and bilateral pulmonary irradiation (approximately 1,500 rad in 2 wk). Preliminary analysis showed a local primary tumor control of approximately 90%. Patients with lesions in the pelvis and humerus had local failure rates of 13% (7 of 54) and 21.4% (6 of 28), respectively. The treatment groups differed significantly in the incidence of pulmonary metastasis. Patients treated with the 4 drugs (regimen 1) had a 14% incidence, whereas 42% of those treated with only 3 drugs (regimen 2) developed pulmonary metastases. Of all patients treated with 3 drugs and pulmonary irradiation (regimen 3), 18% showed lung metastases. The study indicated that intensive chemotherapy and RT significantly improved the local control and survival of patients with localized Ewing's sarcoma. However, the high incidence of metastasis indicated the need for more effective systemic chemotherapy for further improvement of treatment results. More studies are needed so we can define the volume to be treated and the optimal dose of irradiation to determine a therapeutic strategy that will yield optimal survival and tumor control with the fewest sequelae.

Adolescent↗

PubMed1981

Prognostic factors in children with Ewing's sarcoma.

Patient characteristics of 272 patients entered in a clinical trial conducted by the Pediatric Intergroup Ewing's Sarcoma Committee between June 1972 and November 1978 were examined for their relationship to prognosis. Prognosis was defined as disease-free survival time (time to local recurrence and/or metastatic disease) and overall survival time; all times were measured from the start of treatment. In a multivariate regression model, primary site of disease was the major variable that influenced prognosis, and patients with pelvic sites had the least favorable prognoses, followed by those with proximal and rib sites. The most favorable sites were distal and other. The median disease-free and survival times in weeks by primary site were, respectively: pelvis (69, 112), proximal (102, 141), rib (105, 109+), distal (226+, 240), and other (96+, 199+). Females had better prognoses than males; the median survival times were 197 and 147 weeks, respectively. An abnormal liver function as indicated by an abnormal serum glutamic-oxaloacetic transaminase value (greater than 45 IU) was a bad prognostic sign, although only 8 patients had this finding; their median survival time was 94 weeks. Patients who had resections had a slight advantage in survival compared with those having biopsies, though the difference favoring resection patients was not consistent for both sexes in any primary site. Individual characteristics of the patients that were of prognostic significance were: blood lymphocyte counts (high counts favorable), polymorphonuclear leukocyte counts (high counts unfavorable), and time from symptoms to diagnosis (times less than 1 mol favorable). Patients who received treatment 2 had significantly poorer prognoses than those given treatments 1 or 3. The median disease-free and survival times by treatment were (in wk): 1 (134, 198+), 2 (81, 120), and 3 (123, 182).

Adolescent↗

PubMed1981

Multimodal therapy in metastatic Ewing's sarcoma: an Intergroup Study.

Multimodal therapy consisting of radiation therapy to all areas of gross disease and intensive combination chemotherapy was administered to 44 patients with Ewing's sarcoma. Seven of these patients had regional disease and the others had clinical evidence of distant metastases. The median duration of time on study for all patients was 75 weeks. A complete response occurred in 31 of the patients and 17 are currently free of disease. Four deaths resulted from complications of therapy; 2 were due to infection and 2 due to adriamycin-related cardiomyopathy.

Bone Neoplasms↗

PubMed1981

The immune system and the histiocytosis syndromes.

The histiocytosis syndromes consist of a heterogeneous group of disorders referred to as histiocytosis X or differentiated histiocytosis. Although a large proportion of cases of histiocytosis remains to be of an idiopathic nature, there are cases in which the etiologic mechanism appears to be a defect in the immune system. Provided is a brief review of the literature regarding the immune system in histiocytic disease and a review of the experience at the University of Minnesota with immunologic evaluation of children with histiocytic reactions. Two general hypotheses on the relationship between the immune system and histiocytosis syndromes are discussed.

Child, Preschool↗

PubMed1981

L-Asparaginase as a single agent in acute lymphocytic leukemia: survey of studies form Childrens Cancer Study Group.

A series of studies were undertaken by the Childrens Cancer Study Group between 1968 and 1972 to evaluate the optimum schedule and dosage of L-asparaginase as a single agent in induction and maintenance of children with previously treated acute lymphocytic leukemia. Six different dosages of enzyme were administered (3600-840,000 IU/4 wk/m2). The optimal dose of L-asparaginase was found to be 12,000 IU/m2 given three times per week for a total dose of 144,000 IU/m2. Sixty-three percent of children treated with this dosage achieved a bone marrow remission. The intramuscular method of administration is as effective and produced less significant sensitivity reactions than the intravenous method. Reinduction, after an unmaintained remission, with L-asparaginase was possible in 32% of the cases. L-Asparaginase used as a maintenance agent did not result in a significant increase in remission duration when compared to no maintenance therapy. However, patients who received L-asparaginase maintenance had a median remission duration of 66 days as compared to 41 days for the no-maintenance group.

Asparaginase↗

PubMed1980

Bone sarcoma as a second malignant neoplasm in children: influence of radiation and genetic predisposition for the Late Effects Study Group.

Osteosarcoma or chondrosarcoma developed as a second malignant neoplasm (SMN) in 40 of 188 individuals with SMN whose first neoplasm occurred in childhood. A genetic susceptibility to cancer was found in 23; the SMN developed in an irradiated field in 32; both factors wer present in 16; neither in one. When a genetic predisposition was present, radiation shortened the interval to SMN. The intervals between tumors and the age at which the bone sarcomas developed in relation to genetic disease and therapy were analyzed by a two-mutation hypothesis. Studies of SMN in childhood permit us to make observation about the role of genetic factors and environmental mutagens in cancer etiology.

Adolescent↗

PubMed1980

Thrombotic and hemorrhagic strokes complicating early therapy for childhood acute lymphoblastic leukemia.

Sudden cerebrovascular insults occurred during or immediately following remission induction therapy in 4 children with acute lymphoblastic leukemia. In 3, cerebral infarction was due to thrombosis. In the fourth, an intracerebral hematoma developed representing either frank hemorrhaging or a hemorrhagic infarction. None of the patients had central nervous system leukemia or extreme leukocytosis at the time of diagnosis. Symptoms were obtundation, hemiparesis, seizures, and headache. The induction chemotherapy included L-asparaginase which causes deficiencies of antithrombin, plasminogen, fibrinogen, and factors IX and XI. These hemostatic abnormalities may explain the thromboses and bleeding observed in these children.

Adolescent↗

PubMed1980

Comparison of three methods of central-nervous-system prophylaxis in childhood acute lymphoblastic leukaemia.

A retrospective comparison was made of three methods of central-nervous-system prophylaxis in childhood acute lymphoblastic leukaemia; (1) intrathecal methotrexate only; (2) intermediate-dose methotrexate infusion and intrathecal methotrexate and, (3) 2400 rads cranial irradiation and intrathecal methotrexate. The incidence of primary meningeal relapse was statistically significantly lower in both standard-risk patients (age grear than 24 months and less than or equal to 120 months white-cell count less than 20,000) and increased-risk patients (age less than or equal to 24 months or greater than 120 months and/or white-cell count greater than 20,000) whose central-nervous-system prophylaxis included cranial irradiation. The disease-free and overall survival of irradiated increased-risk patients was significantly better than that of unirradiated increased-risk patients. The disease-free survival of standard-risk patients who received intermediate-dose methotrexate was statistically superior to that of the remaining standard-risk patients. There were no significant differences in overall survival between the three groups of standard-risk patients.

Adolescent↗

PubMed1980

Testicular relapse in childhood acute lymphoblastic leukemia: association with pretreatment patient characteristics and treatment. A report for Childrens Cancer Study Group.

Of 395 male pediatric patients with previously untreated acute lymphoblastic leukemia, 20 (5%) exhibited testicular infiltration prior to or concurrent with their first bone marrow relapse. Fourteen occurred as an isolated relapse and six occurred concomitant with bone marrow and/or central nervous system relapse. Nine of the 20 relapses were in patients who had discontinued therapy after completing three years of continuous complete remission. Factors found to be independently associated with an increased risk of testicular relapse during maintained remission included pretreatment lymphadenopathy, and to a lesser extent, initial hemoglobin level and initial platelet count. Pretreatment splenomegaly and lymphadenopathy appear to imply an increased risk of testicular relapse for those patients who have their maintenance therapy discontinued. Time from testicular relapse to bone marrow relapse or death was significantly shorter for patients with testicular involvement while receiving chemotherapy when compared to patients with testicular relapse after discontinuing therapy. In those patients achieving three years of continuous complete remission, subsequent testicular relapse occurred significantly more often in patients who discontinued therapy than a similar group who continued therapy. In a group of 76 males who received presymptomatic gonadal radiation immediately after achieving an initial marrow remission, protection appears to have been provided against the manifestation of testicular leukemia during maintained remission.

Age Factors↗