[Palliative care--objectives and accomplishments of the Association for the Development of Palliative Care]. / Soins palliatifs--Objectifs et réalisations de l'Association pour le développement des Soins Palliatifs.

Associations involved in Palliative Cares want to develop a better use of therapeutic resources and a better support of patients threatened in their Lives. They want to help developing of adjusted structures to a better global tacking charge of patient and his family in hospital and at home. The Association for development of Palliative Cares (ASP) founded 1984 has chosen from the beginning the concept of continuous care and has set since 1986 the first coordinated team of voluntary "Support", selected and trained, in a curative care unit cancer patients with fatal prognostic, after a possible remission. They participated, the ASP also, to the architectural conception and the financing of the first unit of palliative cares in France. The ASP conceived a help to home support for patients in the end of their life, by a team reinforced by a referent nurse, in collaboration with a home hospitalisation unit and a pain consulting. The ASP is now taking action in Paris and its area, with its 180 volunteers and 12 teams in 11 Hospitals and one at home.

[Predictive value of urinary cytology in the diagnosis of urothelial tumors]. / Valeur prédictive de la cytologie urinaire dans le diagnostic des tumeurs urothéliales.

Between November 1991 and June 1993, 315 patients with urethral lesions were divided into two groups: group 1 included 215 patients with urethral tumours and group 2 included 100 patients with non-tumoural lesions. In all patients, urethral smears (performed on 3 consecutive days), pyelography and uretrocystoscopy (with bladder biopsy of observable lesions) were performed in all. The sensitivity of urine cytology examinations in the diagnosis of bladder cancer was found to by 80% with a specificity of 90%. The positive predictive value was 94.5% and the negative predictive value 67.7%. Urine cytology agreed with the diagnosis of urethral tumours especially well in advanced stage ulcerated tumours. These results underline the importance of cytology examinations in the exploration and follow-up of patients with urethral tumours.

[Tuberculous reactions]. / Réactions tuberculiniques.

Intradermoreaction made with reliable tuberculin is the reference test: it is the only one that allows a therapeutic decision. The other tests can only be used as pre or post vaccinal tests, or as a technical approach for new born. In France, the reading of the IDR is difficult because BCG is almost always applied during small childhood. However it is possible to admit tuberculosis infection for vaccinated person with clear increase of the IDR positivity compared to a measured reference IDR, or when the IDR is superior to 14 mm for persons vaccinated a long time ago. For immunocompetent persons, a tuberculosis infection admitted on an isolated IDR (not associated with tuberculosis disease) does not need chimiprophylaxy for children under five, or in case of clear and recent increase of IDR, or in a context of contamination. For a HIV positive person, a tuberculosis infection admitted on an isolated IDR implies a systematic chimioprophylaxy, but the IDR looses its signification as the immunodepression progresses. An attempt to study the prevalence of tuberculosis infection at twenty in France for vaccinated persons has been realized. In 1984 the admitted rate is over 8%, superior to the calculated rate from Styblo and Sutherland tables. That seems to mean that tuberculosis infection is under evaluated in France.

[Data of broncho-alveolar lavage and pulmonary histiocytosis X]. / Données du lavage broncho-alvéolaire et histiocytose X pulmonaire.

The data obtained from broncho-alveolar lavage in 12 cases of pulmonary histiocytosis X explored between 1978 and 1986 are reported. The usefulness of this simple and reliable examination to the diagnosis and follow-up is emphasized. The diagnosis, suggested by radiography of the chest, may be suspected on cytological findings of hypercellularity, macrophage alveolitis and eosinophilia. The occurrence of neutrophilic hypergranulocytosis indicates an unfavourable course towards fibrosis. An increase of cells marked with monoclonal OKT6 antibodies (70% of the cases in this series) contributes to the diagnosis of histiocytosis X and is a good indicator of granuloma activity during the follow-up period. Electron microscopy of the alveolar fluid often confirms the diagnosis by showing the specific organelle: the X granule (60% of the cases in this series), thus avoiding exploratory thoracotomy in many patients.

Aneurysm of the inferior mesenteric artery associated with occlusion of the celiac axis and superior mesenteric artery.

The authors report a case of aneurysm of the inferior mesenteric artery encountered in a 38-year-old man, associated with occlusion of the celiac axis, the superior mesenteric artery and the inferior mesenteric artery distal to the aneurysm. All three arteries were revascularized. In spite of failure in the bypass of the superior mesenteric artery, the patient remained symptom free until his demise four years later, from a probable myocardial infarction. Only 11 cases of aneurysms of the inferior mesenteric artery have been reported in the literature. The causes, diagnosis and treatment of these uncommon lesions are discussed. When occlusion of the celiac axis is associated with that of the superior mesenteric artery, a complete mesenteric revascularization should be attempted whenever possible.

[Bone involvement in histiocytosis X in adults. 11 cases]. / L'atteinte osseuse de l'histiocytose x chez l'adulte. 11 observations.

The authors report 11 cases of bone lesions associated with histiocytosis X in adults, with a total of 25 bone sites: 22 were detected on X-rays and 3 were detected on scintigraphy when the corresponding X-rays were normal. They stress the high incidence of cranio-facial involvement, predominantly in the maxillae, with premature localised loosening of the teeth. They recommend X-rays in the staging of histiocytosis X and reserve scintigraphy for the cases with normal or doubtful X-rays. The bone lesions do not affect the prognosis of histiocytosis X, which depends on the associated visceral lesions. It generally has a favourable outcome and requires careful treatment: limited to a localised procedure for the purposes of histological diagnosis or to control the local underlying visceral risk or the functional complications. Bone lesions do not usually participate in the decision to treat histiocytosis X by chemotherapy.

[Intravascular bronchiolo-alveolar tumor. Study of a form with angiosarcomatous tendency]. / Tumeur bronchiolo-alvéolaire intra-vasculaire I.V.B.A.T. Etude d'une forme à tendance angiosarcomateuse.

Intravascular bronchiolo-alveolar tumor (IVBAT) is a rare pulmonary neoplasm of assumed endothelial nature. We report a new case of peculiar interest because of the association of a diffuse pulmonary involvement with multiple visceral localisations, which has scarcely been proved during life. We found out a striking endothelial differentiation on microscopic examination. The presence of F VIII RAg in many tumor cells as well as the ultramicroscopic findings support the endothelial differentiation. Thus, we regard our case of IVBAT as an aggressive form of the disease behaving as a real angiosarcoma.

[A frequent malignant tumor of the soft tissues, malignant fibrohistiocytoma. Current interest: apropos of 2 cases disclosed by vascular complications]. / Une tumeur maligne fréquente des parties molles, Le fibrohistiocytome malin (M.F.H.). Intérêt actuel: à propos de 2 cas révélés par des complications vasculaires.

Malignant fibrohistiocytoma represents one of the most common malignant tumors of soft tissues. Previously difficult to diagnose with precision, it can now be detected pathologically as a result of recent techniques for investigating cellular development. Treatment is by wide exeresis, prognosis remaining reserved and requiring prolonged postoperative surveillance.

[Primary cutaneous histiocytosarcoma. Incidence, characterization, course]. / Les histiocytosarcomes cutanés primitifs. Fréquence, caractérisation, évolution.

A case of true cutaneous histiocytosarcoma --or Crosti's reticulosis--is reported, and provides the basis for a discussion of the techniques at present used in defining the histiocytic origin of lymphomas, criteria for assessing the malignancy of histiocytic infiltrates, and clinical data and course of histiocytic sarcomas. Particular emphasis is laid on the characteristics of Crosti's reticulosis, which the authors consider to be a form of histiocytic sarcoma, usually with a favorable outcome. They also attempt to isolate pathological criteria for assessing the prognosis of the disease, which appears to vary substantially from case to case. Setting aside cases which had not been studied by both ultrastructural and enzymo-immunological techniques, the authors found only the twenty-two cases of primary cutaneous histiocytosarcoma in dermatological literature, including their own.

[Malignant systemic angioendotheliomatosis. Apropos of a case with an ultrastructural and histo-immunologic study]. / L'angio-endothéliomatose (maligne) systémique. A propos d'une observation avec étude ultrastructurale et histo-immunologique.

Clinico-pathological data in a typical case are compared with those of 54 published reports of systemic angioendotheliomatosis. In this rare disease the lesions, characterized by clusters of abnormal cells in the small vessels, usually have multivisceral localizations, with chiefly cutaneous and encephalic manifestations and a rapidly fatal course. However cases are known with lesions restricted to the skin and a protracted if not regressive course; about half of these cases are associated with an endocarditis. In the case reported, the results of ultrastructural and histo-immunological studies (f. VIII, lysozyme, anti-protease, Ig) are in favour of the endothelial origin of the abnormal cells, a now widely accepted hypothesis.

[Cerebral and pulmonary histiocytosis X. Neurologic manifestations disclosing a pseudotumoral formation on the floor of the 4th ventricle]. / Histiocytose X cérébrale et pulmonaire. Manifestations neurologiques révélatrices d'une formation pseudo-tumorale du plancher du IVe ventricule.

The authors report a case of histiocytosis X which presented with neurological manifestations in a 20 year old man: episodes of headache with vomiting, followed, three months later, by the development of paralysis of the right 6th and 7th cranial nerves associated with nystagmus. The CT scan revealed a tumour-like lesion on the floor of the IVth ventricle with a long axis of 18 mm. The chest x-ray revealed diffuse nodular opacities in the pleural and apical regions with features suggestive of histiocytosis X. The diagnosis was confirmed by surgical biopsy of the typical pulmonary nodules which were rich in histiocytes with X bodies on electron microscopy. The neurological signs disappeared after one month of treatment with Prednisone (1 mg/kg/day) and Vincaleukoblastine (10 mg/week). By the 3rd month, the pulmonary lesions were reduced and the intra-ventricular formation had regressed by 40%. In the authors' series of 29 cases of confirmed histiocytosis X in adults, the present case is the only one with a clinical neurological presentation, apart from 3 cases of diabetes insipidus. A review of the literature confirms the rarity of this type of presentation. The suggestion of the diagnosis by the chest x-ray appearance enabled a dangerous neuro-surgical operation to be avoided.

[Lymphocytic interstitial pneumonia in Gougerot-Sjögren syndrome with scleroderma]. / Pneumonie interstitielle lymphocytaire au cours d'un syndrome de Gougerot-Sjögren avec sclérodermie.

The authors report the case of a 70 year old woman with a 15 year history of the Gougerot-Sjogren syndrome, Raynaud's phenomenon and sclerodactyly. Progressive respiratory symptoms in this case were not due to pulmonary fibrosis secondary to scleroderma but to diffuse interstitial lymphocytic pneumonia (ILP) secondary to the Gougerot-Sjogren syndrome. The authors emphasize the diagnostic value of the significant, permanent lymphocytosis in the aspirate after broncho-alveolar lavage. The differential diagnosis is difficult because the radiological and clinical signs of pulmonary complications of the Gougerot-Sjogren syndrome and of scleroderma are very similar. Reports of the triple association of ILP-Gougerot-Sjogren syndrome-scleroderma are uncommon in the medical literature. However, prospective studies suggest that paraclinical stigmata of the Sjogren syndrome are relatively common in patients with scleroderma. These observations suggest that some patients with clinical "pulmonary fibrosis" may in fact have ILP secondary to slowly progressive Sjogren's syndrome.