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A girl with retinoblastoma, mild psychomotor retardation, bifid uvula and clinodactyly of the fifth fingers is described. A chromosome analysis revealed a long arm deletion of a D chromosome. An autoradiographic analysis, as well as G- and Q-banding analyses indicated a 13q- chromosome. Measurements of length, surface and relative reflection of the different light and dark bands in the normal and abnormal chromosome 13 pointed to an interstitial deIetion, mainly of band q21 (46, XX, de1(13) (pterq14:: q22qter) The chromosome findings and the clinical features of the patient are discussed and compared with other reported cases.

A 51/2-year-old boy is presented with chronic granulocytic leukaemia and blastic transformation whose clinical course is complicated by meningeal leukaemia. In the authors' opinion prophylactic central nervous system therapy should be part of the initial therapy of blastic transformation.

Two hundred and seventeen patients, 1-50 yr old, with acute lymphoblastic leukemia in complete remission were randomized to receive a 1-yr consolidation chemotherapy of either type P, comprising 7 different drugs, or type M, consisting of methotrexate interspersed with prednisone and vincristine. Thereafter, they were randomized a second time to receive a 4-yr maintenance of either chemotherapy or immunotherapy, comprised of allogeneic blasts and bacillus Calmette-Guérin (BCG). Consolidation P caused more toxicity than consolidation M. However, comparison between the consolidation therapies P and M showed no significant difference, neither for disease-free interval nor for duration of survival. Chemotherapy showed more lethal toxicity in adults than in children. Comparison between chemotherapy (C) and immunotherapy (I) as maintenance treatment showed a significant (p = 0.016) superiority of C for disease-free interval (DFI). The difference was even more pronounced (p = 0.009) in the group with less than 8 g/dl of hemoglobin (Hb) at diagnosis before therapy. On the other hand, for patients with more than 8 g/dl Hb at diagnosis, presumably those with T-ALL, no difference in DFI was seen. No difference has been seen so far between maintenance therapies I and C concerning the duration of survival. The patients who were receiving maintenance I when they relapsed and who were consequently retreated by chemotherapy, survived longer from relapse than those patients retreated for relapse while receiving maintenance C.

The details of 37 children presenting a malignant tumor in the ENT-region (period 1950-1983) are presented and discussed. The prevalence was higher in boys than in girls and the most common tumors were non-Hodgkin lymphoma (n = 14) and rhabdomyosarcoma (n = 13). The most common primary sites were the nasopharynx (9 cases), the paranasal sinuses (7 cases) and the soft tissue in the parotid region (7 cases). In 23 children the presence of a painless tumor was the first symptom, in 11 pain was predominant. Only a minority (5 children) presented a limited tumor; in 20 children there was a local extension of the tumor and in 10 children metastases were present at the first visit. The beneficial role of chemotherapy and the necessity of a good teamwork in the treatment of these children is outlined. Of the 37 children, 14, (38%) are surviving; of these 14 children, 12 are off therapy and cured. It is demonstrated that the prognosis improved considerably since the last 14 years.

Children with acute lymphoblastic leukemia frequently show skeletal changes. We examined 50 children with acute lymphoblastic leukemia with reference for skeletal pain as well as the nature and the degree of skeletal changes. 14 children had bone pain. The pain did not correlate with the roentgenographic findings. Some children had characteristic bone changes without bone pain. Roentgenographic findings were observed in 35 of the 50 children (70%). The major lesions observed were: transverse metaphyseal lucent bands, osteolytic lesions, osteosclerotic lesions, periosteal reaction and osteoporosis. Different pathologic findings in leukemia have been given a prognostic value. This was also done for the bony changes but the literature is not coherent on this point. In the group of 17 children with evident skeletal changes, 15 had a non B- non T-cell leukemia. Also the age of the patient, apart from the immunological cell type, seems to be an important factor in the incidence of initial skeletal roentgenographic findings.

From January 1981 through July 1983, 141 children with newly diagnosed acute lymphoblastic leukemia were registered in a cooperative clinical study whose objective was to evaluate the toxicity and the feasibility of the German Berlin-Frankfort-Munster (BFM) protocol. The results were comparable with those reported by the BFM group. For the 133 patients (94%) who achieved complete remission, the actuarial disease-free survival was 67% at 4 years. These results were obtained in spite of a high rate of deaths in complete remission during the initial year of the study. Subsequently, probably as a result of improved expertise in the handling of the protocol, the proportion of toxic deaths declined sharply. Although the current BFM protocol adjusts for aggressiveness of therapy according to the volume of the liver and spleen, splenomegaly (but not hepatomegaly) remained of prognostic significance. Moreover, for patients with bad risk features, an initial high hemoglobin level was found to represent an additional factor of negative significance.

Dans 18 cas de neuroblastome humain la noradrenaline (NA) et la dopamine-β-hydroxylase presentent des profils de distribution intracellulaire identiques, suggerant un stockage dans la meme particule. Le contenu en NA de ces particules n'est pas inferieur a celui des particules du nerf splenique; on peut en conclure qu'un stockage defectueux de NA dans les cas de neuroblastome est peu probable.