Between July 1976 and July 1984, 1106 cold thyroid nodules have been operated on UCL. Teaching Hospital; 128 were diagnosed as carcinoma (11,5%) and 15 were diagnosed as medullary carcinoma of the thyroid gland (1,3% of all the cold nodules and 12% of all carcinomas of the thyroid). This article presents the case of two families exhibiting a characteristic clinical history of Medullary Carcinoma of the thyroid or CMT. The authors describe briefly the clinical signs; they point out that it is absolutely necessary to ask for a blood calcitonine level control in case of cold thyroid nodule or cervical lymphadenopathie. Once the diagnosis of Medullary Carcinoma of the thyroid gland is reached, screening of the Family, at least the first degree relatives should be undertaken, in order to reveal subclinical cases, which could be detected by laboratory investigations at an early subclinical stage. The treatment of the CMT is always surgery; it consists of total thyroidectomy with a modified neck dissection or a radical neck dissection, if lymph nodes are more than 3 cm or are fixed. Any way the recurrent nerve nodes have to be looked for, and if positive a Mediastinal lymph node dissection should be carried out. A control of the calcitonine hormone blood level should be asked for twice a year to detect any metastases or recurrence.

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)