Twenty-five cases of a special pattern of chronic myeloid leukemia characterized, at the first evaluation, by excessive amount of blasts in blood or bone marrow (myeloblasts greater than or equal to 20%), Ph1 chromosome, and short evolution (median survival = 14,5 months) are reported. Age, spleen volume, white blood cell count are in keeping with those found in usual chronic myeloid leukemia (C.M.L.). However, clinical course is more severe with fever, bone pains, and anemia. Myelogibrosis, high circulating basophil polymorphonuclear count, platelet and megacaryocyte abnormalities (in morphology and number) are frequently associated with blastic excess. Subacute myeloid leukemia Ph1 positive (L.M.S. Ph1+) is proposed as an appellation for these cases in order to distinguish them from chronic myelocytic leukemia (C.M.L.) and other subacute myelogytic leukemias. The association of Ph1 chromosome excess of blasts and bone marrow fibrosis distinguishes L.M.S. Ph+ from: 1 degree C.M.L. with myelogibrosis; 2 degrees unusual cases of Ph1 positive myelofibrosis with myeloid metaplasia (M.M.M.); 3 degrees h1 positive acute leukemia. Forms of L.M.S. Ph+ with heavy blastic involvement are probably transitional cases with Ph1 acute myeloblastic leukemia. Prognosis is poorer than in C.M.L. but better than in acute blastic crisis occurring after chronic phase, which can be very similar to L.M.S. Ph1+, when seen for the first time.

A transformed and malignant cell line was obtained by treatment of secondary hamster embryo cells with 7-methyl benz(a)anthracene (10 mug/ml of medium). Later on these cells acquired the capacity to grow spontaneously in suspension and form "spheroids". The evolution of this cell line showed similarities between the carcinogenic process in vitro and in vivo.

The authors report an observation in which four siblings were affected by both multiple cutaneous epitheliomatosis and complex but relatively stereotyped neurological disorders. Clinically, the main syndrome was cerebello-spinal ataxia with involvement of the anterior horns of the spinal cord with less marked pyramidal and extra-pyramidal features. Neuropathological examination of one of the cases revealed lesions of essentially cerebello-spinal degeneration suggestive of Menzel's disease. The possible connection between the neural and cutaneous lesions is discussed. All the various etiological categories possible have been ruled out; not one being entirely satisfactory, except for the very broad category of genetic neuro-dermatoses.

138 cases of spinal haemangioblastomas have been reviewed (30 original cases). The first part of this study is devoted to a general survey concerning history, pathological anatomy, genetics and nosology of haemangioblastomas of the central nervous system. Microscopic anatomy of these tumors has been a much debated question and is responsible for the numerous denominations proposed: angiomatosis, capillary angiomas, angioreticulomas, haemangiomas, haemangioendotheliomas, haemangiopericytomas, angioblastic meningiomas; haemangioblastoma seems the most correct. Recent studies in optical and mainly electron microscopy do not allow exact typing of tumoral cells and histogenesis remains uncertain. It is generally accepted that the tumor arises in vascular nesenchyme as a result of dygenetic abnormality. This theory provides a rational explanation for visceral localizations and classification of Lindau disease among phakomatoses. An hereditary factor is present in 25 percent of cases, mainly in multifocal localizations. In the second part, interest is focused on spinal haemangioblastomas, 17 extradural, 121 subdural haemangioblastomas are studied and listed. Extradural tumors are always solitary, mostly in the dorsal area. Subdural tumors may be out of the cord (10 tumors), on the posterior roots (28 tumors), or within the cord. The lesions are situated mainly in the cervicodorsal and dorsolumbar regions. Intramedullary haemangioblastomas are situated in nearly all cases in the dorsal part of the cord behind the central canal. They are close to the dorsal surface of the cord (60 percent) or partly out of the cord (6 percent) or totally intramedullary (30 percent). Cysts close to the tumor or at a distance are present in nearly 50 percent of cases and their origin is still questionnable. Clinical patterns have no special peculiarities, syrinomyelic symptomatology occurs in only 25 percent of cases. Neuroradiology and mainly spinal angiography are a very important step. Angiography was performed in 23 cases (36 tumors). Feeding vessels arise from the posterior system (33 cases) and the anterior system (19 cases). A dark blush indicates the tumor, disappears within 16 to 35 seconds, and can be easily differentiated from arterio venous malformations. The opportunity for spinal angiography depends on clinical and radiological criteria as well as the natural history of the disease. Total removal is the only effective treatment of haemangioblastomas of the cor. Accurate localization of feeding arteries and venous drainage through spinal angiography, preoperative embolization if possible and microsugical techniques applied to cord lesions allow thie ideal treatment. But a lot of problems remain unsolved.

The authors presents two cases of basocellular nevomatosis with multiple maxillary cysts. He thought it important to point out the presence of a cyst in the left styloid apophysis, as up to now, epidermoid cysts have never been observed in other facial bones apart from the maxillae in BCN. In conclusion, the author presents three casses, which although showing multiple cysts or pseudocysts of the maxillae, do not belong to the BCN syndrome.

Thirty-four cases of corticosurrenaloma with clinical onset before 15 years of age have been studied. Higher frequency in girls (65 percent of the cases) and in young age (80 percent before 5 years of age, 43 percent before 2 years), association with personal or familial other tumors and malformations, are noticeable features. Virilism is the major manifestation in children (83 percent of the cases), either isolated or associated with hypercortisolism and/or feminization. Hormonal assays are of little value, and the dynamic adrenal tests are of some help only in small tumors or isolated hypercortisolism. Radiological diagnosis by urography with cavography, arteriography, is easy in most cases. Evaluation of prognosis is very difficult. Clinical and biological data are not significant, histological data are often of little help. Only a very large mass, and occurrence of metastases (liver, lungs) evidence malignancy. This series does not allow to assess definitely the best therapeutic regimen. Surgery has to be performed as soon as possible. The effects of radiations have not yet been ascertained. High doses of op'DDD have led to a sustained remission of the tumor or metastases in 2 patients, and perhaps to a longer survival in 4 others.

A complex genetic system controls the susceptibility to virus induced leukemia. A good example is provided by AKR mice in which several genes acting in association are involved. The control is demonstrable at several levels: a) virus expression, b) virus replication, c) antitumor immune response. In addition one or two other genes could be involved. The "Leukemia-permissive" genotype would be probably rarely present and the AKR provides the model of a mice in which all genetic locus bear the permissive alleles.

The authors report two new cases of functional parathyroid carcinoma, the course of which varied between 5 and 7 years. The main fact was their familial character, as the disease affected two siblings. No similar case was found in the world literature. These two cases were characterised by symptoms in the bones or pancreatic symptoms which dominated the whole clinical course. Finally, the second case was studied by angiography which permitted the authors to localise a lymphnode metastasis on the opposite side. As far as treatment was concerned, the frequency and severity of local and regional spread justifies wide removal at an early stage. This attitude is necessary in any case of parathyroid tumor with an infiltrating character.

It is often difficult to establish a valid pedigree in a family, a member of which shows one or another phakomatosis. It is indispensable to examine all the members of the family in order to detect the "formes frustes" or the atypical and abortive forms, which are frequent, and in order to establish a genetic prognosis and to give a genetic counseling, taking the penetrance into account.