About a spontaneously regressive case of osteo-cutaneous congenital fibromatosis, the authors describe the characteristics of the disease (32 observations). Although the majority of cases are sporadic, 3 familial observations are in favour of a dominant autosomal transmission of low penetrance. Study of the familial cases and analysis of the different localisations demonstrate the unicity of the so-called diffuse forms with visceral involvement and of the so-called generalized forms without visceral involvement congenital fibromatosis is characterized by several fibromas at birth: in two-thirds of the cases, it is a purely cutaneous or osteocutaneous form, which disappears spontaneously; in one third of the cases, it is a cutaneous or osteo-cutaneous form with lethal visceral involvement.

The authors present three cases of multiple, intra-cranial meningiomatosis with contact hyperostosis affecting the grandmother, mother and daughter, in a very stereotypic manner. No other sign of von Recklinghausen's disease was noted, except for two tiny neurofibromas on the left hand in the mother. Only the latter suffered from bilateral deafness, but it was not possible to give definite proof of the presence of an acoustic neuroma in this patient. Anatomical verification in the first case, and surgical intervention in the last, confirmed that it was in fact a meningioma. Thus, a new clinical form of expression of neuro-fibromatosis has been found.

Two cases of acute myeloblastic leukemia were observed in a young 22 year old woman, then two years later, in her brother aged 16 years. The elder brother had died previously probably from the same disease. The very rare families in which more than two members had the same type of leukemia show the possible intervention of a hereditary factor in leukemogenesis.

Two cases of multiple self-healing epithelioma (Ferguson-Smith) are reported in a French family. This dermatosis has been present since the age of 30 in both father and son. The father aged 66 years, farmer, had suffered since 1939 from recurrent spontaneously healing tumors of the face and neck, with deeply pitted scars on the face. He was treated during fifteen years in a cancer institute for multiple well-differentiated " squamous cell carcinoma " on the neck, face, and tongue; local recurrence did not occur after treatment of the active tumors and there was no clinical evidence of secondary lymphnode involvement. His son, aged 30, teacher, came in July with a papular active tumour on the cheek, which was surgically removed; his face and ears soon became affected. In the two cases, numerous biopsies showed in the early stage a great down growth of the epidermis with a central horny plug, like in keratotic papules or small kerato-acan-thomas and in more active lesions, typical appearances of squamous cell epithelioma grade I. The familial incidence is proved in the majority of the 30 cases reported since the first description by Ferguson-Smith in 1934. Only caucasians are concerned, most of them being Scottish. There is no sex predominance. First changes generally occur in young adults on light exposed areas but mucous membranes can be affected. The individual lesions last for several months, but fresh ones appear continuously, so that the disease remains extensive. The cause of these tumors is still unknown; histological and ultrastructural findings cannot explain this condition. Our ultrastructural study is parallel to previous works and fails to find viral particles in keratinocyte nuclei or cytoplasma.

On the basis of a clinical case, the authors review the principal signs of Bourneville tuberose sclerosis and show the renal manifestations which may be encountered. The most common is the presence in both kidneys of tumours which are hamartomas. In addition, this patient suffered from hypertension and renal insufficiency. Their frequency and pathogenesis are discussed in the context of this disease.

The authors report ten cases of multiple malignant tumours noted on a general medical unit : 4 cases of chronic lymphatic leukemia associated with one visceral carcinoma. One case of polycythemia associated with a bifocal colonic carcinoma and a skin carcinoma. 5 double carcinomas mainly bronchial and digestive. They recall the general characteristics of these multiple carcinomas and discuss their pathogenesis with special reference to factors of immunodepression and genetic factors.

This entity, isolated by Gorlin, is characterized by the presence of cutaneous and mucous neuromas, facial disfiguration resembling acromegalia as well as morphological changes to the extremities similar to those produced by Marfan's disease. These factors are also associated with multiple endocrinological neoplasms type II of Sipple's syndrome. In other words, malignant tumors developing from thyroid "C" cells as well as from bilateral benign pheochromocytoma (neoplasias which derive from the A.P.U.D. system). It is considered to be hereditary in nature and transmitted as a dominant trait. Its evolution is triphasic, and the prognosis is extremely dismal. There may exist incomplete or benign forms, in the course of which the endocrinological neoplasms may appear very late or not at all. Generally, the diagnosis is easy. This entity can be calssified with other diseases stemming from the neural ridge, in other words "neurocristopathies" but in spite of these similarities with disease of the same embryological origin, it would seem that this particular entity is genetically autonomous. The only effective treatment is surgical, for thyroid neoplasms, as well as neoplasms occurring in the medullo-adrenal glands. It should be noted that this line of treatment must be undertaken with the utmost precaution.

The evidence in support of the assertion that 80% of human cancers are of environmental origin is reviewed. The environment is used in its broadest sense and is taken to include the widespread general exposures of air and water pollution, the work environment, exposures resulting from personal choice such as smoking and drinking, and the diet. Several examples are chosen to show that a wide range of environmental agents can cause cancer. Consideration of descriptive epidemiological data relating to migrants, geographical variation in incidence, changes in risk over time, correlation studies, clusters and case reports, supports an environmental etiology for many cancers the cause of which is at present unkown. The same data can be used to show that a large proportion of cancer is likely to be due to environmental factors. If the lowest rate observed in a series of populations be regarded as representing a residuum of cancers due to non-environmental causes then it is possible to imagine a hypothetical population with a very low rate indeed. However, it is unlikely that such a level could be achieved even if all causal mechanisms were understood as it is possible that factors leading to one cancer may protect against another. Until evidence to the contrary is aduced it is held that the vast majority of human cancers are due to the environment: the reasons why there has been reluctance to accept this conclusion are discussed.

Two cases of retinoblastoma in two children showing a chromosomal rearrangement in the karyotype were reported. In the first case, a pericentric inversion of chromosome 9 was observed both in the child and his father. In the second case, a reciprocal translocation t (3;12)(q29;q15) present also in the paternal karyotype was identified. A strict connection cannot be established between the tumour itself and the family chromosomal aberrations, but these observations raise the problem of a possible "interchromosomal effect".

During the last 5 years, 7 families suffering from familial polyposis coli have been investigated; we found 23 members affected and we operated 10 of them. One of them died of metastases and 9 members of these pedigrees died of carcinoma which appeared at the average age of 41,5 years. This confirms that this condition is the most serious precancerous illness and needs regular controls of all members of these families. At the occasion of this review, we describe the main features of this condition and briefly our surgical attitude.

The authors have studied 59 cases of Bourneville's tuberous sclerosis, including 44 sporadic cases from healthy parents and 9 familial patients. They stress the very high frequency of spasms in the infants of less than one year (80 %), the frequency and the early appearance of retinal phakomata and areas of depigmentation and the interest in the early detection of intracranial calcification. They discuss the specificity of the individual manifestations: cutaneous, retinal and systemic and they consider the problem of genetic counselling.

Having had the occasion of observing a new case, the authors have started on a new description of a specific autosomal dominant inheritance disease, description which connects an eye trouble with basal cell nevi, with jaw cysts as well as with malformations of the skeleton and nervous lesions, the tumoral lesions having the essential characteristic of possessing and evolutive potential. The authors explain the reasons which have led them to give to this specific disease the name of Gorlin-Goltz phacomatosis.