The 476 children treated for solid malignant tumor from 1947 to 1968 at the Institut Gustave-Roussy, and living NED at 5 years, were reviewed after 5 to 36 years of follow-up (median 17 years). Two children were affected in the sibship in 9 families. Thirty patients died after 7 to 32 years, 15 from late evolution of their primary tumor, 7 from second cancer, and 8 from other causes. Late sequellae (3 lethal), mainly neurologic, orthopedic, endocrine, and others were observed in 271 patients, and not known in 48. A second tumor (19 malignant, 46 benign) occurred from 7 to 26 years after treatment, more than half in the previously irradiated area. These patients have presently 164 known children; two died in infancy, two have malformations. None has had tumor up until now. The long-term cure of cancer remains true in 95% of cases in our span of follow-up, with sequellae compatible with a useful life in most cases.

The present report discusses the main constraints influencing the choice in the method in the epidemiological approach of host factors and family factors. It briefly describes 3 of the usual methods in this field, i.e. the migrant studies, the twin studies and the family studies. The practical problems and the conceptual limitations met by each of these 3 methods are reviewed. A brief review of the literature provides an opportunity for examining the main misunderstandings between geneticists and epidemiologists in their study of human groups when analysing the role of such factors in the etiology of cancer.

The c-myc oncogene was characterized and its expression analyzed in 32 mammary adenocarcinomas and in 2 benign breast tumors from 34 untreated patients. Southern blot hybridization experiments have demonstrated the amplification of the oncogene (3 to 30 fold) in 3 carcinomas. The analysis of total RNA by Northern blot revealed the presence of a 2.4 kb c-myc RNA band. In 7 out of 10 carcinomas from patients with 3 or more than 3 lymph node metastases the level of c-myc expression evaluated by dot blot analysis was 4 to 14 fold greater than that of normal human tissues. In only 5 out of 22 carcinomas from patients without lymph node metastases or less than 3 invaded nodes the level of c-myc expression was also higher (4 to 10 fold). The level of c-myc expression was not significantly enhanced in the 2 benign tumors. It is suggested that the c-myc gene activation could be associated to a higher degree of malignancy of mammary carcinomas.

Squamous cell lung carcinomas from 10 untreated patients were examined for the state of the oncogene c-myc. Blot hybridization experiments have demonstrated the amplification of the oncogene of about six fold in only one tumor. The oncogene amplification was not detected in normal tissues of patients. The analysis of RNA by Northern blot revealed the presence in the seven tumors examined of a 2.4 kb c-myc RNA band. The level of c-myc expression evaluated by dot blot analysis was 5 to 14 fold greater in tumors than that of histologically normal lung of the same patients.

Among 16 leukemia patients with abnormalities of the short arm of chromosome 12 (12p) were found 5 patients with an increased number of marrow basophils and a special M2 cytological feature. This new correlation between 12p abnormalities and M2-baso phenotype is presented. The localisation of c-k ras 2 genes at the same 12p site suggests a possible mutation of this c-oncogene.

The authors report a retrospective study of 129 children with retinoblastoma treated from 1963 to 1977 at the Institut Curie by enucleation of the worst eye and conservative irradiation of the other eye; this irradiation was performed either with Stallard plaque (19 cases) or with electrons (110 cases). In 8 familial cases, no enucleation has been performed. T.E.M. was used from 1964 to 1973 and iterative photocoagulation since 1968. With a 5 years follow up, 88 children (68%) are living NED, 6 are lost. There was 34 treatment failures (26%) and 1 death from second malignant tumor. At 10 and 15 years, the results are stable despite the occurrence of two other second primary tumors. Irradiation preserved 73/94 (78%) of the irradiated eyes. The technical aspects of the radiotherapy with electrons and both ocular and vital prognostic factors are discussed.

Chromosome studies on five cases of large bowel adenocarcinoma show a systematic rearrangement of chromosome No. 17 after breakage in its juxtacentromeric region (band 17 q 11). A frequent involvement of chromosome No. 8 (juxtacentromeric break) and the loss of chromosome No. 18 are also noticed. A review of the literature strengthens the hypothesis of a preferential involvement of these chromosomes in large bowel cancer.

A molecular rearrangement of the proto-oncogene c-myc located downstream of the exon III 3' end has been found in a cell line derived from KE37 cell line established from an acute T-lymphoblastic leukemia. This rearrangement resulted from a chromosomal translocation t(8; 14)(q24; q11). Since the 14q11 chromosomal band has been found to be involved in several T-cell leukemias and lymphomas, the importance of the rearrangement of c-myc discovered in the KE37 cell line lies in the possibility of analyzing chromosome 14 DNA near the breakpoint involved in the translocation.

Posterior fossa is the main location of hemangioblastomas of the CNS. Etiological, gross anatomical, clinical and thérapeutic study performed by F. Resche et al. is based on analysis of 20 large series and 624 separated cases of the literature and on results of a cooperative study of the S.F.N.C. (Société française de Neurochirurgie) which collected 262 cases. It is the largest series gathered up to the present. Among the S.F.N.C. series there were 151 males and 111 females. The age ranged from 2-71 with two peaks in 31-35 and 46-50 in the male population. The mean age at the time of diagnosis is 38 but lower in the female group than in the male one. The mean age is significantly lower in familial cases. Cerebellum is the main location of infratentorial hemangioblastomas. Brain stem hemangioblastomas occurred in less of ten per cent of cases (3,77% in the S.F.N.C. series). Solitary tumors are located in the cerebellar hemipheres in about 80 per cent of cases, where they are of macrocystic type (type 2) in a proportion of two third; vermian tumors are equally of cystic and solid (type 3 and 4) types. Whatever their gross anatomic type and location, infratentorial hemangioblastomas usually have a superficial margin at the leptomeningeal layer. Associated axial and extra-axial lesions are analysed. Angiomatosis retinae are in 30-40% of cases the first manifestations of disease, occurring before infratentorial tumor. Pathogenic features are studied. Lethal potential of renal carcinomas and pancreatic nesidio-blastomas of Lindau's disease is pointed out. Histo and cyto pathologic aspects of infratentorial hemangioblastomas are analysed by J. Hassoun. Morphological (photonic and ultra-structural) characteristics are seen and an attempt on histogenetic interpretation is given. From a clinical point of view it is important to note that hemangioblastomas, although they are vascular tumors, are exceptionally revealed by an intrathecal bleeding. The most common initial symptoms are manifestations of increased intracranial pressure without or only with light signs of cerebello-vestibular disturbances. (F. Resche et al.) Potential occurrence of polycythemia in cerebellar hemangioblastoma is a well-known fact. J.P. Caron et al. report a case of a cerebellar hemangioblastoma with polycythemia where plasmatic, C.S.F. and saline extract tumor erythropoietin levels have been measured. Elevate erythropoietin levels were found in the C.S.F. and the tumor suggesting true ectopic hormonal production which is responsible for the polycythemia encountered in this patient.(ABSTRACT TRUNCATED AT 400 WORDS)

The PFT cell line was established in 1969 from diploid cells of the inner lining of a uterine tube of a 2 year-old sow and has been continuously subcultured more than 500 times over a decade. Three chromosomal rearrangements have occurred during this time. The first translocation was shown at the 100th passage with the concomitant and spontaneous release of an endogeneous type C virus. The second translocation was observed at the 290th passage along with the appearance of gap junctions and the induction of malignant tumors in athymic nude mice following the inoculation of PFT cells. The third translocation was found towards the 470th passage with the simultaneous appearance of annulate lamellae. Since the translocations were accompanied by the spontaneous release of a retrovirus and then by malignancy of PFT cells when inoculated in athymic nude mice, it is likely that the chromosomal abnormalities are associated with the viral carcinogenesis of the cell line. The third translocation appears to confirm the perenniality of the multistep evolution hypothesis of malignancy.

Genetic susceptibility (HLA types), clinical and pathological findings, amount of acetylcholine receptor antibodies and T lymphocyte subpopulations were studied in 63 patients with Myasthenia Gravis (MG). The frequency of HLA-DR5 was increased among patients (0.50 versus 0.23 in controls, pc less than 0.01, relative risk 3.3) and that of HLA-DR3 previously described as associated with MG was slightly increased (0.31 versus 0.20 in controls). The relative frequencies of two T cell subpopulations (T4 helper and T8 suppressor/cytotoxic lymphocytes) were normal in HLA-DR5 positive patients while the ratio T4/T8 was increased in other MG patients, who were HLA-DR3 (p less than 0.005). The high rate was due to an increase in the absolute number of T4 lymphocytes (p less than 0.001). HLA-DR3 patients were mostly women with early onset of a severe form of the disease, marked by the presence of thymic follicular lymphoid hyperplasia. A third genetic susceptibility to this disease was recently described in patients treated with D-penicillamine, the antigenic frequency of HLA-Bw35, DR1 is significantly increased. These 3 types of association between HLA and myasthenia gravis can be related to three different physiopathological mechanisms: the first two are probably linked to individual immunity (inductor/suppressor disequilibrium), in the third association, the mechanism is immunopharmacological.

A patient with Ph1 positive chronic myeloid leukemia developed accelerated phase with cytological abnormalities of platelets and megakaryocytes, and persistence of a high platelet count, after the failure of a course of intensive chemotherapy. During this phase, the karyotype analysis revealed two different Ph1 positive clones: one with a pericentric inversion of chromosome 3, the other with the same abnormality and a paracentric inversion of the second chromosome 3. Cases of acute leukemia with normal thrombopoiesis and abnormalities of chromosome 3, especially paracentric inversion, have already been reported. The significance of this association is discussed.

Secondary bone sarcomas are frequent among children who have been treated previously for a retinoblastoma. The main point is that these bone sarcoma occur almost always after a bilateral retinoblastoma and more often in the irradiated area; from these, the classical concept of "radiocancer" was generally admitted. However, since many authors reported bone sarcomas occurring in a different location from the irradiated area, radiotherapy cannot be by itself the causative factor of these secondary bone sarcomas. Therefore the genetical factor is probably one of the predisposing factor. The authors report 6 cases of bone sarcomas with a review of the literature of bone sarcomas occurring in the irradiated field or not, for children "cured" of retinoblastoma.