The present study reports just 4 cases of cancer of the ovary which occurred in 3 families with a history of cancer of the ovary. The authors reviewing the literature of the last 15 years have tried to indicate the present status of knowledge of the features of ovarian familial cancer. At present, as there are no specific enzymatic, immunological, reliable genetic markers nor screening tests, the only prophylaxis that is available is bilateral oophorectomy. As the epithelium of the ovary and of the peritoneum are embryologically derived from the same sources, this prophylactic procedure cannot completely abolish the risk of the development of cancer of the peritoneum in these families.

Triploidy in a common chromosomal abnormality that gives rise to early abortion in most cases. Rarely triploidies carry on past the end of the first trimester. When they do they are always accompanied by severe intra-uterine growth retardation with occasionally molar changes in the placenta. These changes are different from hydatidiform moles but they can become malignant. The formal diagnosis of triploidy depends on the fetal caryotype.

A case of granulocytic breast sarcoma in a 19 year-old-girl is reported. Surgical excision of the tumor was followed by radiotherapy of the breast and lymph nodes. Then 3 months later acute M2 myelocytic leukemia was diagnosed, complete remission being attained using rubidazone-cytarabine and maintained by monthly reinduction courses. Trisomy of chromosome 22 was present in leukemic cells and disappeared during remission. Its pathogenic significance is briefly discussed.

An exceptional case of acute myeloblastic leukemia (M2) with t(8;21) relapsed as an acute monoblastic leukemia (M5). At that time only a minority of metaphases exhibited a t(8;21) and/or abnormality of chromosome No 11 long arm whereas various other chromosomal changes were present. The existence of a relapse of M2 with t(8;21) as M5 is discussed in relation to the commitment of the cells which became leukemic, and to the possibility of a second leukemia.

The chromosome localization of a 5.4 kb DNA genomic probe of proto-oncogene c-ets 1 has been analysed in an acute monocytic leukemia with t (1; 11) (q21; q23) translocation. The c-ets probe has been translocated onto the rearranged chromosome 1, suggesting the involvement of the proto-oncogene in leukemias with chromosome rearrangements at band 11 q23.

The case of a multiple myeloma secreting IgG kappa in both mother and daughter is reported and discussed in the light of previous reports of familial myeloma and myeloma occurring in spouses. In March 1981, the 60-year-old daughter complained of fatigue leading to the discovery of anemia. The diagnosis of myeloma was based on the association of a monoclonal IgG kappa (57.5 g/l) with low serum levels of IgM and IgA and of an increase (38%) in bone marrow plasma cells. She was treated by chemotherapy and by radiotherapy on bone lesions which appeared during the course of treatment. She died in June 1983. In October 1984, the 84-year-old mother was also found to have anemia. The diagnosis of myeloma was based on the association of a monoclonal IgG kappa (24.5 g/l) with low serum levels of IgM and IgA, 0.6 g/l of Bence-Jones protein in urine, an increase (12%) of atypical plasma cells, and of multiple lytic lesions on X-ray. Familial myeloma has been exceptionally reported. We have found only one case in mother and daughter where the myeloma protein was respectively IgA and IgG. The light chain isotype was not determined. Cases of myeloma occurring in spouses have equally been rarely reported in the medical literature. Genetic transmission of chromosomal determinants for myeloma or a type of response to environmental factors have been suspected as well as environmental determinants for myeloma. Genetically abnormal regulation of immunoglobulin synthesis is another possibility.

A t(1; 14) (p32; q11) translocation has been found by the cytogenetic study of a T cell acute lymphoblastic leukemia patient. Molecular hybridization with a probe (D14S7) which recognizes a fragment of the alpha chain T receptor gene has shown a rearranged EcoRI 11.0 kb band in addition to the germline 3 and 8 kb bands. This translocation infrequently described until now is a new example of an alpha chain gene rearrangement in T cell malignancy. Moreover the breakpoint on chromosome 1 may also involve the newly isolated protooncogene, L-myc, normally localized on the chromosomal band 1 p32.

Cytogenetic analysis of two human ovarian adenocarcinomas show identical specific anomalies. These two tumors exhibit, in all the analysed mitosis, a paracentric inversion of chromosome 3 and a translocation between chromosomes 2 and 5. A relationship between these markers and the location of human oncogenes on chromosomes 2, 3 and 5 should be considered.

Numerous vascular dysplasiae belong to the group of genodysplasiae. Most arteriovenous dysplasiae (cirsoid, racemosum aneurysms,...) are the substratum of various regional "angiomatosis". Stenotic or ectatic arterial dysplasiae can be associated with genodysplasiae. In addition, lymphatic dysplasiae (congenital elephantiasis, etc...) also exist. Among benign tumours, angiomas are sometimes hardly distinguishable from dysplasiae. Some tumors with intermediate malignancy have an uncertain prognosis: chemodectoma, hemangiopericytoma. As for malignant vascular tumours, they fall into 3 varieties: angiosarcoma, Kaposi's sarcoma and sarcoma of vascular walls.

Familial gastrointestinal polyposis constitutes a multiplication of the principle varieties of common polyps, not only in the colon but also in other parts of the gastrointestinal tract. These are hereditary and familial diseases with autosomal dominant transmission. The genetic defect can also be responsible for associated extra-gastrointestinal lesions. The classification of these familial polyposes is based on the histological features of the polyps. The adenomatous polyposes consist of familial recto-colonic polyposis (FRCP), which is the most frequent form, and Gardner's syndrome. This variety of polyposis selectively involves the recto-colon, but also presents gastric and duodenal lesions. The defining feature of Gardner's syndrome is the extra-gastrointestinal mesenchymal lesions, osteomas, skin tumours and, most importantly, mesenteric fibromatosis which may complicate abdominal operations. The prognosis of FRCP and Gardner's syndrome is determined by the almost obligatory and often multicentric malignant degeneration of the polyps, which justifies routine total proctocolectomy. Peutz-Jeghers' syndrome is syndrome is characterised by hamartomatous polyposis selectively involving the upper part of the small intestine. It generally has a benign course. Similarly, juvenile polyposis consists of benign inflammatory polyps and is accompanied by congenital anomalies in one third of cases. The diagnosis of familial polyposis should be proposed whenever polyps are detected, which appear to be unusual in terms of their number or their distribution. A family survey should be performed in such cases.

Authors report one family with three cases of myeloblastic acute leukaemia in three generations. In this family, without chromosomal abnormalities (the only one possible laboratory examination), the risk is certainly increased, but it remains, fortunately, low.

To determine whether or not alpha 1-antitrypsin phenotype PiMZ is a predisposing factor for hepatocellular carcinoma alpha 1-antitrypsin phenotype was studied in 83 French patients with hepatocellular carcinoma compared with 1,030 blood donors. In 66 patients, alpha 1-antitrypsin phenotype was determined by isoelectric focusing which allows the distinction between subtypes of phenotype M. No difference has been shown between the two groups for alpha 1-antitrypsin alleles or subtypes of allele Pi*M. The authors conclude that phenotype PiMZ is not a significant predisposing factor for hepatocellular carcinoma in French people. As well, a review of literature suggests that, in contrast with the conclusion of previous reports, the link between alpha 1-antitrypsin phenotype PiMZ and hepatocellular carcinoma is either nonexistent or very weak.

2 cases of multiple myeloma, both with IgG kappa, are reported in a man and his daughter. 33 other cases of myeloma involving two or more first degree relatives have been reported in the literature. Reported cases showed no major specific characteristics of the myelomas involved. Monoclonal protein family members were rarely identical. Our observation of identical monoclonal protein in two family members has only been found in 4 other reports. Occurrence of several cases of myeloma in one family is unlikely to be due to chance alone and the possibility of familial myeloma must be considered. Reports of benign monoclonal gammapathy in other members of the family of a patient with myeloma is also in favor of genetic factors. HLA-typing has shown an increase in frequency of the 4c complex and HLA-A9 in myeloma, and the latter was present in our patients. A genetic predisposition is further supported by experimental observations in mice. Finally, the short delay between onsets of myeloma in family members, less than 5 years in 20 families out of 27, and 4 years in our case, suggests a possible role of environmental factors.