Biomedical subjects
Publications and source records attributed to G Chomette.
A case of mycosis fungoides initiating in the cavum is reported. Results of biopsy of specimens taken from this region were negative, and diagnosis was established only after the appearance of lymph node and cutaneous lesions. The etiology is discussed, particularly in relation to the possible viral nature of the acquired immunodeficiency sundromes and more especially the "gay syndrome".
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Arterial angiodysplasias constitute an ill-defined entity comprising conditions of apparently very variable natures. Apart from arterial fibrodysplasia, the clinical features of which, if not the etiology, are well established, there exists a number of different arterial abnormalities, stenotic or aneurysmal, the significance of which remains unclear (dolicho-arteries, the Moya-Moya syndrome, etc.). However, the role of genetic factors and metabolic abnormalities in these conditions is uncontestable. They demonstrate both the authenticity of this group of diseases and the importance of metabolic disorders of the interstitial tissues in the majority of these vascular abnormalities.
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Hemangiopericytoma, an unpredictable tumour, is not rare in the buccal cavity. Nine cases, most of which have been followed-up over a long period, show that, in this localisation, this neoplasm shows the unpredictable nature, though to a lesser degree (recurrences and metastases) of those situated in other parts of the body. Histological and ultrastructural analysis shows, apart from its characteristic perivascular features, much cellular polymorphism responsible for the diagnostic problems. A variety of cells, endothelial, fibroblastic and smooth muscle are observed next to the pericytes. The predominance of one or another cell type significantly affect the prognosis; immature forms with an endothelial predominance have a recurrent course, whilst fibrous forms have a much better prognosis. This diversity tends to support the histogenetic concept of one blastomatous cell responsible for the variable maturative possibilities.
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The authors report on a new case of leiomyosarcoma of the inferior vena cava, to add to the sixty five cases already mentioned in the literature. The patient had been suffering from subcostal pains for 12 years, and an adenomyoma of the gall bladder was diagnosed. The tumor was discovered in the course of a cholecystectomy. The vena cava was resected and ligated below the renal veins. After describing the case, the authors go on to review the literature and stress the fact that the prognosis, which is often severe, could be improved by earlier diagnosis.
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465 patients with broncho-pulmonary malignant tumors have been autopsied. Small cell carcinoma was diagnosed in 22.5 per cent of these patients. The histo-cytological variants of these tumors (lymphocytoid, polygonal, fusiform and polymorphic) had the same general characteristics (age, sex, survival) and a similar clinical course. Grossly and histologically, the bronchial tumor, always located in proximal bronchial tree, largely involved the mediastinum. Metastases were peculiarly frequent to the liver (69%), to bone (64.2%) and to the central nervous system (36.2%). Three Schwartz-Bartter syndromes and two Denny Brown sensitive neuropathies were noted in this statistical study.
Ventricular tachycardia with left bundle branch block appearance in adults without coronary artery disease may be due to arrhythmogenic right ventricular dysplasia. This condition forms part of a spectrum with the complete form of Uhl's anomaly at one end and arrhythmogenic right ventricular dysplasia at the other. Both conditions have similar histological appearances representing the same congenital defect but the macroscopic appearances are quite different. Arrhythmogenic right ventricular dysplasia seems to be a slowly progressive condition with one or more localisations in the right ventricle where the myocardium is replaced by adipose tissue with progression of lesions from the epicardium towards the endocardium. The subepicardial layers resemble a plexiform structure of partially degenerated myocardial fibres within fibro-adipose tissue. These fibres may be the site of slowing of activation and the anatomical basis of intraventricular reentry phenomena. A similar appearance is also found in UHl's anomaly, but the severe dilatation of the ventricle and almost complete absence of muscle fibres only leaves a few zones with surviving, partially degenerated myocardial fibres. Clinically, a cardiothoracic index of over 0.60 is more common in Uhl's anomaly whilst arrhythmogenic right ventricular dysplasia is usually associated with a normal cardiac silhouette or only slight cardiomegaly. The differential diagnosis is important if surgery is being considered. An appropriate technique must be chosen to avoid supraventricular arrhythmias which can lethal in patients with Uhl's anomaly.
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In a series of 331 minor salivary gland tumors (malignant in about 55,3% of cases), mucoepidermoid tumors, after cystic adenoïd carcinomas, are the most frequent malignant tumors (21,5% of cases). They are much more common in women than in men. The average age of patients at presentation (52,2 years) is higher than that of pleomorphic adenomas. They occur more frequently in buccal floor, tongue and gums. By a half-quantitative study of 71 mucoepidermoid tumors, these neoplasms are ranged in 3 main groups : differentiated epidermoid or glandular tumors, intermediate cell tumors with predominant oncocytic, clear glycogenic or basophilic cells and rare adenosquamous carcinomas. These 3 groups are well demonstrated by histoenzymological investigations, which show high level of oxydative enzymes activity in oncocytic cells and high level of ATPase and alkaline phosphatase activities around basophilic sheets. Besides, an ultrastructural study shows, in addition to well differentiated glandular or epidermoid cells, 3 forms of intermediate cells : young basophilic ribosome-rich cells and more differentiated oncocytic or glycogenic cells. The various structural features of mucoepidermoid tumors are positively correlated with clinical course and behaviour, after long term follow-up studies. Differentiated forms and intermediate clear or oxyphilic cell tumors are of low grade malignancy. Intermediate basophilic cell tumors grow rapidly or metastasize and a lethal course is often noted in these cases.