Immunologic chimerism as evidence of bone marrow graft acceptance in an identical twin with acute lymphocytic leukemia.

Bone marrow from a well child was infused into her identical twin who had acute lymphocytic leukemia. In an attempt to provide an immunologic tag for use in twin transplantation, the donor twin was immunized to Keyhole limpet hemocyanin (KLH) and yellow fever virus prior to the marrow infusion. Subsequent immunological chimerism in the recipient twin gave evidence for graft acceptance.

Malignant histiocytosis in childhood: morphologic considerations.

Eight cases diagnosed over a ten-year period as malignant histiocytosis (MH; histiocytic medullary reticulosis) were reviewed to clarify diagnostic criteria for the childhood disease and to identify sources of diagnostic confusion. Five of the eight cases met the authors' criteria for diagnosis; i.e., they were characterized by loose mixed infiltrates composed of three cell types--well-differentiated histiocytes, prohistiocytes, and malignant histiocytes--and they had no leukemic phase. Three cases did not share these features and were reclassified. The liver was found to be the organ most useful in premortem diagnosis, and immunoperoxidase staining for immunoglobulins and lysozyme was also helpful. The clinical and morphologic features of the five cases confirm the authors' view that diagnoses of MH should be limited to cases in which there is a loose pleomorphic population of all three types of histiocytes and that cases with monomorphous populations of aggregated malignant cells should be classified as lymphomas.

Toxicity study of cytosine arabinoside and methotrexate in the maintenance therapy of childhood leukemia. A Southwest Oncology Group study.

In a toxicity study to determine the feasibility of treating patients with acute lymphocytic leukemia (ALL) using an intravenous combination of cytosine arabinoside (Ara-C) and methotrexate (MTX), the drugs were given either simultaneously or sequentially every two weeks. Twenty-nine patients were studied, 17 treated simultaneously, 12 treated sequentially. The tolerated doses of Ara-C and MTX were 60 mg/m2 and 90 mg/m2, respectively, for the simultaneous treatment schedule and 90 mg/m2 and 150 mg/m2, respectively, for the sequential treatment schedule. The dose-limiting factor of the drug combination was gastrointestinal toxicity. The observed recurrent vomiting on both schedules rendered the treatment unsuitable for maintenance therapy.

Changing concepts in management of pelvic rhabdomyosarcoma in children.

Survival with embryonal rhabdomyosarcoma of all sites has improved dramatically in recent years with the increased use of long-term, cyclic, multidrug chemotherapy. Protocols have been established and are currently being evaluated by the Intergroup Rhabdomyosarcoma Study. The management of embryonal rhabdomyosarcoma of the pelvic viscera, though, remains troublesome. Limited surgical excision is rarely possible and high-dose radiotherapy to the bony pelvis may cause severe and disabling growth disorders. Yet, survival with these lesions is increasing as with rhabdomyosarcoma from all sites and is directly related to a well-planned and aggressive multidisciplinary program. We have seen 12 cases of pelvic rhabdomyosarcoma within the last seventeen years at this institution. These cases will be reviewed in regard to varying modes of therapy and survival. Our current therapeutic approach, based on national and local experience, will be presented.

Chemotherapy of histiocytosis-X.

This article discusses the efficacy of various chemotherapeutic agents in the treatment of histiocytosis-X. Although these agents alone and in combination have improved the prognoses for children with histiocytosis-X, the need for more effective methods of treatment still exists.

Symptoms and signs of cancer in the school-age child.

The American Cancer Society's seven warning signs of cancer are: 1. Unusual bleeding or discharge. 2. A lump or thickening in the breast or elsewhere. 3. A sore that does not heal. 4. Change in bowel or bladder habits. 5. Hoarseness or cough. 6. Indigestion or difficulty in swallowing. 7. Change in size or color of a wart or mole. These signs apply to children as well as to adults. Cancer in children, however, is often more insidious than in adults and may well mimic many other diseases, developmental processess, or childhood psychologic problems. The knowledge that cancer kills more children than any other disease and the awareness of the presenting symptoms and signs may well save a child's life. Early detection with prompt, aggressive therapy is of paramount importance in achieving cures in childhood cancer.

Curing children with leukemia in West Virginia.

Leukemia is the most common cancer in childhood with acute lymphoblastic leukemia (ALL) the most common subtype. While once uniformly fatal, today leukemia is a highly curable disease. To determine the outcomes of children with acute lymphoblastic leukemia in West Virginia, we performed a retrospective analysis of the results of treatment of children and adolescents with B-lineage ALL diagnosed between 2/86 and 1/91 and treated by the pediatric oncology teams at Morgantown or Charleston. Forty-one children with B-lineage ALL were identified and treated by a uniform protocol. Twenty-nine (71%) have remained disease-free for more than two years off therapy and are considered cured. Of the 10 patients who relapsed, five have now been off rescue therapy for greater than two years and are likely to be cured. Thirty-five of the original cohort of 41 children are alive and disease-free yielding an overall survival of 85%. The results of treatment of childhood leukemia in West Virginia are comparable to national data. Children with ALL diagnosed and treated by pediatric oncology teams in West Virginia have a very good chance of being cured.

A survey of pediatric malignancy in West Virginia.

The occurrence of malignancy in the pediatric age group is an uncommon but serious event. Since little data are available on the extent, nature or referral patterns of childhood cancer in West Virginia, we conducted a survey of 782 primary care physicians and 17 regional referral centers. The results showed that 249 cases of malignancy in the pediatric age group were reported and that 68% of children with newly diagnosed childhood malignancy were referred to institutions within West Virginia. We conclude that the incidence and distribution of types of malignancy in childhood in West Virginia parallels that of the nation, although there is some regional variation within the state.

Immunotherapy in acute leukemias. Possible applications in children.

The role of immunotherapy in maintenance of remission in children with acute leukemias is briefly reviewed. With few exceptions, the bulk of clinical trials of immunotherapy in children with acute lymphoblastic leukemia have failed to demonstrate a beneficial effect. Immunotherapy trials for acute myelogenous leukemia mainly have involved adults. Though the results of many studies are incomplete or inconclusive, an increased median survival time has been frequently observed. The mechanisms of action remain obscure. However, further immunotherapy trials in children with acute myelogenous leukemia may be a reasonable alternative, in view of the generally poor long-term results with conventional chemotherapy.

Cis-diamminedichloroplatinum (NSC-119875) in childhood malignancies: a Southwest Oncology Group study.

Children with malignancies resistant to conventional therapy were treated with cis-diamminedichloroplatinum (PDD), 15 to 20 mg/m2, given daily by rapid intravenous infusion for 5 days at 3-wk intervals. Eleven of 24 children with acute lymphocytic leukemia (ALL) received two or more courses; among these no remissions occurred. Fifty-four children with solid tumors were treated: 25 neuroblastoma, 9 rhabdomyosarcoma, 4 Ewing sarcoma, 2 testicular embryonal carcinoma, 2 retinoblastoma, and 12 miscellaneous tumors. One complete remission, 3 partial remissions, and 2 improvements were observed in children with neuroblastoma. One girl with metastatic osteogenic sarcoma achieved a partial remission. One child with metastatic testicular embryonal carcinoma showed improvement. The side effects were vomiting controlled by antiemetics in 26 children and transient elevations of serum creatinine and BUN in 14 children. Nineteen of 39 children with solid tumors, who received more than one course of PDD, had moderately severe myelosuppression caused by PDD. In summary, PDD is a promising agent in neuroblastoma, osteogenic sarcoma, and testicular embryonal carcinoma, and an ineffective agent in ALL. The effect of PDD on other types of solid tumors should be evaluated in the future.

Doxorubicin and cisplatin therapy in children with neuroblastoma resistant to conventional therapy: a Southwest Oncology Group Study.

Fifteen children with metastatic neuroblastoma resistant to vincristine and cyclophosphamide were treated with two drugs which were known to be effective as single drugs against neuroblastoma. The drugs were given in courses every 3 weeks. Doxorubicin (50 mg/m2 iv) was given on Day 1 and cisplatin (50 mg/m2) was administered on Day 2 as an 8-hour infusion, using a forced diuretic-hydration program. Three of the 15 children achieved partial or complete remission. Three children showed improvement. The other children either did not respond to the therapy or had progressive disease. The combination of doxorubicin and cisplatin given in the sequence outlined is no more effective than either drug given singly. The side effects of the drug combination were tolerable and were in keeping with previously described toxicity.