In a case of white sponge naevus type congenital buccal keratosis, electron microscopic study revealed severe disturbances in filamentogenesis which was insufficient (pale cells) or on the contrary excessive and anarchic (dark cells) and, to a lesser degree, corneal maturation. This was accompanied by slight abnormalities of the cellular junctions, rarely described in such a condition up to the present time. With regard to the delayed epithelial exfoliation typical of this condition, the manner and causes of its development are discussed in the light of ultrastructural findings.

Sipple's syndrome in three members of the same family (three successive generations) is reported, confirming that this syndrome is hereditary and transmitted in an autosomal and dominant way. Whereas pheochromocytoma was clinically detectable, medullary thyroïd carcinoma could only be detected in two cases by systematic search for high levels of calcitonin before and after stimulation. In 6 other siblings, thyroïd carcinoma was eliminated in view of negative stimulation tests. HLA groups were studied, but definite conclusions as to the interest of these data in Sipple's syndrome cannot be drawn from 10 few cases. Persistingly high levels of calcitonin after surgery is known to be of severe prognosis; in the present cases, the very short delay after surgery did not allow definite conclusion, since, according to some authors, several months are necessary to allow a return to normal levels.

We have recently studied a large family of 80 persons in which 47 were examined. The evaluation included history, blood pressure determination, palpation of the thyroid gland and determination of serum carcino-embryonic antigen (C.E.A.) and calcitonin (C.T.). Two members of the kindred had a proven M.C.T. without pheochromocytoma, hyperparathyroidism or Cushing's disease and two others a probable M.C.T. Four members suffered from intestinal occlusion and death occurred in three of them. Our conclusions are: 1) In this family traced through 4 generations: it appears that M.C.T. is transmitted as an autosomal dominant trait with a high degree of penetrance; 2) Our cases associated with those reported in the literature in the past few years point to the existence of a rare but distinct syndrome characterised by the association of M.C.T. and congenital megacolon with hyperplasia of the myenteric plexus; 3) As far as we know, this is the first indication of C.E.A. coupled with elevated calcitonin among several individuals of the same family. We confirm here the conclusions of previous studies: "C.E.A. is a valuable tumour marker which can be used for the detection of M.C.T., particularly if no calcitonin radio-immuno assay is available.

After a brief review of the characteristics of familial malignant melanoma, the authors summarize the literature about the BK mole syndrome. The clinical and histological features of this dermatosis are described. Three personal cases are also reported. The latter tend to demonstrate a certain genetic heterogeneity, while in the literature the BK mole syndrome has been described as an autosomal dominant trait.

The authors report 2 cases of Kaposi's sarcoma in whose immunosuppressive therapy is implicated. In a 47-year-old Italian man, cutaneous lesions were rapidly aggravated after a renal transplant and treatment with corticosteroids and azathioprine; new lesions appeared close to an arteriovenous fistula of the forearm. In a 83-year-old Algerian man, Kaposi's sarcoma appeared during steroid therapy for bullous pemphigoid. The role of acquired immunosuppression, local predisposition and genetical factors are reviewed and discussed for the pathogenesis of Kaposi's sarcoma.

Sister-chromatid exchanges were studied in fibroblasts from a child with del(13)-retinoblastoma. The skin biopsy was performed before the clinical onset of the tumor. The observed frequency of SCE, 19.65 per cell, was significantly increased as compared to that of a normal control, 14.68 per cell (t = 3.57, p < 0.001).

The present observation concerns a female patient with a 47,XX, r(18), + mar karyotype. The size of the ring varies from mitosis to mitosis; generally it is very small. The presence of a small marker ("minute") may explain some phenotypic differences not usually observed in the "r(18) syndrome". This patient is the niece of a trisomic 21, who showed complex chromosome anomalies during the course of an acute leukemia from which he died.

Endocrine polyadenomatosis forms but a part of the larger group of neurocristopathy disorders. This term includes those affections due to lesions of cells, tissues, or organs derived from the neural crest. The common origin of the various neuro-endocrine cells within the neural crest suggests that there is a denominator of embryologic pathogenicity for the different polyendocrine affections. Knowledge of these is essential for early diagnosis of the different neuro-endocrine lesions, together with a systematic search for any familial associations.

The authors present a brief report on a familial case of Wermer's syndrome, and review the principal characteristics of this "multiple endocrine neoplasm" which usually affects the parathyroids, pancreas, and anterior pituitary.

Measurement of sister chromatid exchanges (SCEs) frequency of inbred Rat or nu/nu Mice bone marrow cells, following tumour grafts, have been developed. Increase of SCEs was observed in hosts which present or not metases and with reduced survival rates after malignant tumour grafts. These results suggest a remote control of tumoral tissue by a diffused matter effect.