Twenty-two children with malignant ovarian tumors (12 malignant teratomas, six endodermal sinus tumors, and four embryonal carcinomas) were studied from 1960 through March 1975. Comparison is made between different modalities of therapy, such as surgery alone or surgery in combination with either radiation or chemotherapy. Because of the poor results obtained with the aforementioned treatment methods, a new approach, consisting of more aggressive initial surgery followed immediately by radiation therapy and an intensive chemotherapeutic regimen called the T2 protocol, was begun in 1971 with encouraging results. To date six of ten patients are alive and free of disease with a median observation period of 22+ months. A new grouping defining the extent of disease is also provided. Of the ten patients on the T2 protocol, only one was a Group I; eight were Group III and one was Group IV at the time treatment was initiated. An analysis of the mode of spread, as well as a discussion of the results obtained with previous modalities of therapy in relation to initial grouping and pathology, constitute the background for the proposed treatment.
A variety of surface markers, terminal deoxynucleotidyl transferase (TdT) activity, morphologic appearance, and cytochemical composition were studied in a group of 16 patients (13 children, 3 adults) with acute lymphoblastic leukemia (ALL). In 9 children, no surface markers were detected on lymphoblasts (null-type ALL). Leukemic blasts of 4 children formed E-rosettes. These E-rosette-forming blasts from childhood ALL, and E-rosette-forming lymphoblasts from 3 adult patients with ALL, were studied for the presence of Fc receptors. Of the leukemic blasts from these 7 patients, 2–76% expressed receptors for IgG Fc. Only 3 of 7 patients showed 9–42% receptors for IgM Fc. In addition, complement receptors were investigated in 6 of those 7 patients with T-cell ALL. Complement receptors were detected on 9–70% of the E rosette forming blasts from all 6 patients. TdT activity was elevated in T-cell ALL and in children with null-type ALL. The heterogeneity of Fc receptor expression on leukemic blasts in these patients demonstrates a malignant proliferation of T cells in different stages of differentiation or maturation. This observation might be helpful in subclassifying T-cell leukemias with regard to prognosis and the response to therapy. Cancer 46:45–49, 1980.
Adjuvant chemotherapy was added to local radiation therapy for patients with Ewing's sarcoma to treat widespread microfoci of disease presumed to be present at the time of diagnosis. Since June 1970, 12 children have been treated with radiation therapy and sequential adjuvant chemotherapy: dactinomycin, adriamycin, vincristine, and cyclophosphamide, continued for 2 years. Two children developed reversible congestive heart failure after cumulative adriamycin doses of 905 mg/m2 in one patient and 720 mg/m2 in another patient who had mediastinal irradiation. Adriamycin is now generally limited to cumulative doses of 720 mg/m2 but is further limited to 500 mg/m2 in children who receive mediastinal or pulmonary irradiation. Of 12 children entered into this study and followed for from 10 to 37 months, all continue in disease-free remission without evidence of recurrent tumor, metastatic tumor, or central nervous system disease.
Forty-one previously untreated children with Stage IV non-Hodgkin's lymphoma were studied from January 1971 to April 1979. All patients had bulky disease histologically proven to be non-Hodgkin's lymphoma with bone marrow involvement. They were separated into two groups according to the extent of bone marrow involvement. Group IVA included 14 patients with 25% or less lymphoblasts in the bone marrow. Group IVB included 27 patients with more than 25% blasts in the marrow. Their clinical characteristics with regard to age, sex, hemogram, histology, primary site, and blast morphology are compared. All were treated with the LSA2-L2 protocol with radiation therapy to one or more bulky sites of involvement. The disease-free actuarial survival for Group IVA was 64% with a median observation time of 49 months while that for Group IVB was 65% with a median observation time of 66 months. There was no statistical difference in the survival rates between the two groups. Hence, we conclude that the extent of bone marrow involvement does not affect the prognosis in Stage IV non-Hodgkin's lymphoma. It appears that radiation therapy may have contributed to the improved survival in our series. Furthermore, a subset of patients in Group IVB (24/27) who could be considered as high-risk acute lymphoblastic leukemia on the basis of age, initial leukocyte count, hemoglobin, mediastinal mass or T- or B-cell markers showed an improved survival (73% versus 43%) when compared to patients treated with conventional leukemia regimens.
Abstract Sixty‐three pediatric patients with germ cell tumors are presented with details of symptoms, histological findings, staging, serological markers, treatment, and response to therapy. The primary sites were: ovarian 32, testicular 17, presacral 7, mediastinal 3, intraabdominal 2, vaginal 1, and right inguinal canal 1. These patients were treated with T2 (sequential use of dactinomycin, doxorubicin, vincristine, and cyclophosphamide, with or without radiation), T6 (combination chemotherapy with cyclo‐phosphamide, bleomycin, dactinomycin, doxorubicin, methotrexate, vincristine), or VAB treatment protocols (velban, dactinomycin, bleomycin, cisplatin). The cure rate for stage I ovarian and testicular germ cell tumors was 100%; for stage III, all primary sites, 82% and for stage IV, all primary sites, 75%. Histology was prognostic in ovarian tumors of the immature malignant teratoma type; the neural type immature teratoma, grades II and III, had the worst prognosis. Initial debulking surgery in combination with chemotherapy and radiation plays an important role in germ cell tumors. Stages II, III, and IV germ cell tumors require aggressive treatment with surgery, radiation, and chemotherapy. For stage I patients, with primary ovarian malignant tumor, cure with surgery alone can be achieved in 50% of the cases and in testicular tumors in about 70% of the patients. For those with stage I and elevated serological markers, it is feasible to follow these markers and give no treatment until there is evidence of persistent elevation or a rise in titers after an initial fall. In those without elevated serological markers, one should take into consideration the size of the tumor and the histological type before taking the “wait and see” approach. These stage I tumors are highly curable when they first present but, if allowed to recur, chemotherapy may not offer the patient such a favorable response and cure rate.
