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Introduction La pneumonie organisee est une pathologie pulmonaire de presentation clinique et radiologique variable, aux nombreux diagnostics differentiels, dont le diagnostic de certitude est histologique. Ce diagnostic est actuellement obtenu par biopsie transbronchique ou biopsie chirurgicale mais ces deux techniques ont des inconvenients. Le but de ce travail est de discuter la valeur diagnostique de la biopsie pulmonaire transthoracique guidee par scanner pour le diagnostic de pneumonie organisee et de la comparer aux methodes classiques de diagnostic. Methodes Six observations personnelles sont rapportees et discutees, et une revue de la litterature concernant l’interet de la biopsie pulmonaire transthoracique scano-guidee dans le diagnostic de pneumonie organisee a ete realisee. Resultats La biopsie pulmonaire transthoracique scano-guidee a une meilleure rentabilite que la biopsie transbronchique et fournit des prelevements de plus grande taille, minimisant les risques d’erreur diagnostique. Les valeurs diagnostiques sont respectivement de 88-97 % contre 26-55 % dans les lesions pulmonaires localisees et de 42-100 % contre 66-75 % dans les lesions diffuses. Les complications sont rares et le plus souvent benignes. Conclusions La biopsie pulmonaire transthoracique scano-guidee devrait etre evaluee en remplacement de la biopsie transbronchique dans le diagnostic de pneumonie organisee. La biopsie chirurgicale reste la methode diagnostique de reference.

Introduction Le cherubisme est un processus extensif kystique atteignant surtout la mandibule, dont le diagnostic est souvent fait devant un facies joufflu chez l’enfant. Son determinisme familial est anciennement connu. Recemment identifiee, la mutation de l’exon 9 de SH3BP2 permet d’identifier les cas autosomiques dominants de la maladie. Observation Les auteurs rapportent le cas d’un enfant de 14 ans, de grade I, de diagnostic tardif. Une recherche genetique familiale, faite a Berlin, n’a pas retrouve la mutation du gene candidat. Discussion A cote des cas familiaux dominants, on retrouve des cas sporadiques, parfois lies a une neo-mutation identifiee du gene codant pour SH3BP2. Le cas presente a des antecedents familiaux de troubles de l’homeostasie osseuse et dentaire qui peuvent argumenter l’hypothese d’un mode de transmission autosomique recessif ou d’un autre gene candidat.

Introduction Le neurothecome est une tumeur rare. Elle est benigne mais doit etre distinguee de certaines tumeurs malignes notamment les tumeurs fibro-histiocytaire a cellules plexiformes, les melanomes neurotropes et les sarcomes a cellules claires. Observation Une enfant de 11 ans a consulte pour la recidive d’une tumeur sous-cutanee mentonniere, operee 4 mois plus tot. L’exerese avait ete incomplete. Une reprise chirurgicale au large a permis de controler l’evolution. L’etude histologique a etabli le diagnostic de neurothecome en particulier en raison de l’existence d’atypies et de mitoses, de nodules cellulaires et de l’extension a l’hypoderme. La confirmation a ete apportee par l’immuno-histo-chimie. Discussion Le neurothecome cellulaire est une tumeur benigne de la femme jeune, qui atteint souvent la face. Elle se presente comme un nodule dermique avec respect de l’epiderme. Ses caracteristiques immuno-histo-chimiques specifiques permettent le diagnostic differentiel avec la forme myxoide et les tumeurs melanocytaires et nerveuses. Le traitement est l’exerese chirurgicale.

A nationwide retrospective study of exogenous lipid pneumonia (ELP) was carried out to update the data on this disease, with emphasis on thoracic computed tomography (CT) scan and bronchoalveolar lavage (BAL) findings. The inclusion criteria were: 1) presence of abnormal imaging features compatible with the diagnosis of ELP; 2) presence of intrapulmonary lipids; and 3) exogenous origin of the lipid pneumonia. Forty four cases were included (20 males and 24 females; mean age 62 +/- 11 yrs), of which four were occupational (chronic inhalation of cutting mist or oily vapour in an industrial environment). Thirty of the 40 nonoccupational cases were related to aspiration of liquid paraffin used for the treatment of constipation. A condition possibly favouring oil aspiration or inhalation was present in 34 patients (77%), most commonly gastro-oesophageal reflux (n = 20) and neurological or psychiatric illness (n = 14). Fever (39%), weight loss (34%), cough (64%), dyspnoea (50%) and crepitations (45%) were the most frequent symptoms. BAL was performed in 39 cases: 23% had a lymphocytic alveolitis; 14% neutrophilic alveolitis; and 31% a mixed alveolitis (lymphocytic and neutrophilic). Alveolar consolidations (57%), ground glass opacities (39%), and alveolar nodules (23%) were the most common radiological abnormalities. The changes were bilateral (79%), predominant in the posterior and lower zones of the lobes concerned (74%), hypodense (71%), and spared the subpleural zones (52%). In 13 cases, hypodensity was retrospectively established on CT scan by the presence of a "positive angiogram". This sign may be of diagnostic value when the density measurement is either not possible or not reliable. In conclusion, this study provides an update of the clinical, biological and radiological profile of exogenous lipid pneumonia and, in particular, confirms the diagnostic benefit of computed tomography scan, which revealed bilateral and hypodense changes in a large majority of cases.

Summary Background Organising pneumonia is a pulmonary disease with variable clinical and radiological features and with many differential diagnoses. Diagnosis is based on histology obtained by either transbronchial or surgical lung biopsy but these techniques have several disadvantages. The aim of this study was to evaluate the diagnostic yield of CT-guided transthoracic lung biopsy in organising pneumonia and to compare it to the usual diagnostic tools. Methods Six cases of organising pneumonia diagnosed with CT-guided lung biopsy are reported and discussed. The role of CT-guided lung biopsy in the diagnosis of organising pneumonia was also reviewed in the literature. Results CT-guided transthoracic lung biopsies provided a higher rate of adequate samples than transbronchial biopsies (92–100% versus 77–86%). The samples were larger, which reduced the risks of misdiagnosis and increased the diagnostic yield (88–97% versus 26–55% in pulmonary nodules and 42–100% versus 66–75% in diffuse pulmonary disease). Complications were rare and generally not serious. Conclusion CT-guided transthoracic lung biopsy may be considered in place of transbronchial biopsy in the diagnosis of organising pneumonia. Surgical lung biopsy remains the gold standard method for diagnosis.

Organizing pneumonia is a pulmonary disease with variable clinical and radiological features and with many differential diagnoses. Diagnosis is based on histology obtained by either transbronchial or surgical lung biopsy but these techniques have several disadvantages. The aim of this study was to evaluate the diagnostic yield of CT-guided transthoracic lung biopsy in organizing pneumonia and to compare it to the usual diagnostic tools.Six cases of organizing pneumonia diagnosed with CT-guided lung biopsy are reported and discussed. A review of literature concerning the role of CT-guided lung biopsy in the diagnosis of organizing pneumonia was performed.CT-guided transthoracic lung biopsies provided a higher rate of adequate samples than transbronchial biopsies (92-100% versus 77-86%). The samples were larger, which reduced the risks of misdiagnosis and increased the diagnostic yield (88-97% versus 26-55% in pulmonary nodules and 42-100% versus 66-75% in diffuse pulmonary disease). Complications were rare and generally not serious.CT-guided transthoracic lung biopsy may be considered in place of transbronchial biopsy in the diagnosis of organizing pneumonia. Surgical lung biopsy remains the reference method for diagnosis.

Pulmonary involvement observed during atheroem- catheter was inserted. bolic disease is usually due to congestive heart failure Ten days after admission the patient became dysand not to pulmonary cholesterol crystal emboli. Very pnoeic. He was able to lie flat and had no peripheral few cases of biopsy-proven specific pulmonary involve- oedema, fever, sweats, chills, or cough. Inspiratory ment have previously been reported in the course of crackles were heard over the lower one-third of both atheroembolic disease [3,4]. We describe a case of lungs. Echocardiography was normal. Arterial-blood atheroembolic disease associated with endogenous gases were the following while breathing oxygen at 6 lipoid pneumonia. Because spumous macrophages are litres per minute: partial pressure of oxygen, 8 kPa; observed in the inflammatory reaction surrounding partial pressure of dioxide 4.5 kPa; pH 7.35. A radiocholesterol crystal clefts in diVerent sites, we hypothet- graph of the chest showed bilateral infiltrates more ize that their presence in brochoalveolar fluid may prominent in right mid- and lower lung fields. reflect the presence of cholesterol crystal emboli in the Computed tomography revealed extensive bilateral pulmonary parenchyma. infiltrates. Flexible fibreoptic bronchoscopy examination did not reveal endobronchial lesion. A bronchoalveolar lavage was performed. Bronchoalveolar fluid

Cherubism is an extensive kystic process of the mandibula. The diagnosis is often established in children presented swelling of the jaws. The familial determinism of cherubism is well-known, and recently autosomal dominant cases have been described with detection of the exon 9 - SH3BP2 mutation.We describe the case of a 14-year-old boy with grade I cherubism diagnosed late. The familial genomic analyze conducted in Berlin was negative for the recently identified candidate gene.Apart from dominant cases cherubism sometimes occurs sporadically, some of the cases resulting from a neomutation of the candidate gene. The present case with familial bone homeostasis and dental disorders is an argument for the recessive transmission hypothesis or for another candidate gene.

Neurothekoma is a rare benign tumor which must be distinguished from certain malignant tumors such as fibrohistiocyte tumors or plexiform cell tumors, neurotropic melanomas and clear-cell sarcoma.An 11-year-old girl consulted for a recurrent subcutaneous tumor of the chin which had been operated 4 months earlier. The resection was incomplete. A wider revision resection successfully stopped recurrence. The histology study established the diagnosis of neurothekoma due to the presence of mitosis atypia, cellular nodules, and extension to the hypodermis. Immunohistochemistry confirmed the diagnosis.Neurothekoma is a benign tumor observed in young women, mainly on the face. It occurs as a dermal cohesive mass without infiltration of the epidermis. The typical immunohistochemical pattern enables differential diagnosis with myxoid neurothekoma, melanocytic and nervous system tumors. Surgical resection is indicated.

Resume Les auteurs rapportent le cas d'un patient de 35 ans presentant un dermatopolymyosite associee a une tracheopathie osteochondroplastique. L'evolution sera fatale avec infection pulmonaire favorisee par les troubles de la deglutition due a la polymyosite et a la perturbation de la clairance mucociliaire secondaire a la tracheopathie. La tracheopathie osteochondroplastique est une affection rare diagnostiquee exceptionnellement avant 50 ans. L'association de deux pathologies rares n'est pas le fait du hasard. Elle invite donc a rechercher les circonstances etiologiques communes qui pourraient etre chez ce patient l'inhalation de particules de silice.

The 'Standards, Options and Recommendations' (SOR) project, started in 1993, is a collaboration between the Federation of the French Cancer Centres (FNCLCC), the 20 French cancer centres and specialists from French public universities, general hospitals and private clinics. The main objective is the development of clinical practice guidelines to improve the quality of health care and outcome for cancer patients. The methodology is based on literature review and critical appraisal by a multidisciplinary group of experts, with feedback from specialists in cancer care delivery.To develop clinical practice guidelines according to the definitions of the Standards, Options and Recommendations project for the management of stage I and II non small cell lung carcinoma treated by radiotherapy alone.Data were identified by searching Medline and personal reference lists of members of the expert groups. Once the guidelines were defined, the document was submitted for review to independent reviewers, and to the medical committees of the 20 French cancer centres.The main recommendations for the management of stage I and II non small cell lung carcinoma treated by radiotherapy alone are: 1) The curative external irradiation with a continual course is an alternative to surgery only in the case of medically inoperable tumors or because the patient refuses surgery; 2) The external irradiation of the primary tumor only without the mediastinum could be proposed in peripheral stage IA. In proximal stage IA and IB, external irradiation should be carried out only as part of prospective randomised controlled trials comparing a localised irradiation of the primary tumor with a large irradiation of the mediastinum and the primary tumor. The treated volume must include the macroscopic tumoral volume with or without the microscopic tumoral volume and with a security margin from 1.5 to 2 cm; 3) There is a benefit to delivering a total dose in the primary tumor higher than 60 Gy in so far as the proposed irradiation, taking into account the respiratory function, does not increase the likelihood of severe adverse events due to radiation; and 4) The change in fractionation, the radiochemotherapy combination, the endobronchial brachytherapy with high dose rate alone or with external irradiation could be proposed only as part of prospective controlled trials for tumors classified as stage IB or II.

The aim of this study was to analyze SCLC patients beyond 30 months, particularly their outcome, their way of life, and factors which could influence relapses, second-primary cancers and death.Between January 1986 and May 1995, 263 SCLC patients who survived longer than 30 months were included from 52 French institutions. The analysis was performed on the 155 cases confirmed by a pathologic review.Physical, mental and psychological states were considered as normal at 30 months in respectively 70.3%, 87.7% and 67.7% of patients, not influenced by prophylactic cranial irradiation, number of chemotherapy cycles, CCNU or cisplatin. Therapeutic sequelae were neurological impairment (13%), pulmonary fibrosis (18%) and cardiac disorders (11%) at 30 months. Return to work was possible for 40% of patients in the first two years following diagnosis. Among 43 relapsing patients, 33 benefited from a second-line treatment. Their median survival was 12 months since retreatment, and seven patients have survived again longer than 30 months. Age60 at the time of diagnosis was found as an independent factor increasing the risk of relapse beyond 30 months (OR = 2.46, IC 95% (1.16-5.26), P = 0.01). The risk of relapse became less than 10% beyond five years. Twenty patients (13%) developed a second primary cancer in a mean time of 58.6 months. The risk of second primary cancer was increased by a number of chemotherapy cycles6 (OR = 3.25, IC 95% (1.08-9.8) P = 0.02) and by an age60 (OR = 2.92, IC 95% (1.07-7.97), P = 0.03). Five- and 10-year survival rates were respectively 68% and 44%. In these patients having reached a 30-month survival, three independent factors were predictive of a survival longer than five years: ageor = 60 at the time of diagnosis (OR = 2.85, IC 95% (1.23-6.6), P = 0.01), chest radiotherapy (OR = 3.1, IC 95% (1.28-7.69), P = 0.006) and absence of relapse (OR = 4.5, IC 95% (1.75-12.5), P = 0.002). This study suggests that: 1) therapeutic sequelae are rather mild, allowing return to work in 40% of patients; 2) relapsing 30-month survivors can benefit from second-line treatment; 3) SCLC cure can be achieved with a 10-year follow-up.

We report a case of a 35 year-old-man with dermatomyositis associated with tracheopathia osteoplastica. The swallowing perturbation secondary to myositis and airway involvement by tracheopathia induced fatal outcome. Tracheopathia osteoplastica is a rare disease and occurs exclusively in men over the age of 50. The association of two rarest disease is not a fortuitous event. The common pathogenic factor may be, in this case, the occupational exposure to silicon.

The authors report a new case of bronchio-alveolar carcinoma which developed on a background of iatrogenic exogenous lipid pneumonia; this association is extremely rare (less than 20 cases in the literature) and poses the problem of possible cancer developing in the centre of the lipid pneumonia.

The long-term survivors of SCLC are described in 3 different types of study: analysis of prognostic factors of phase II and III chemotherapy trials (3,4,5,6,7,17,18), epidemiological studies (8) and medical registries of LTS (9,10). A small number of patients with small cell lung cancer achieve long-term survival. Most of these patients have a disease limited to the chest at the time of diagnosis. The major concerns of these LTSs are: the relapse of the SCLC, the occurrence of a second primary tumour and the occurrence of a disease related to tobacco consumption. About 20% of the LTSs die of non-cancer related causes and this exceeds the age adjusted mortality. There is a high risk of relapse in the first 4 years after the diagnosis; this risk decreases later, but relapses may be seen until 7 years. Nearly 8% of LTSs developed a SPTs are alive at 8 years; this indicates that cure is possible in SCLC, however, these patients account for less than 3% of the overall population.

Orthotopic liver transplantation has been considered as one of the therapeutic tools in certain patients with hepatocellular carcinoma. In our liver Unit in Besançon, 17 patients with hepatocellular carcinoma were treated by orthotopic liver transplantation between March 1986 and December 1991. This series included 14 men and 3 women. The mean age was 51 years (range: 36-62). In 11 cases, hepatocellular carcinoma was multifocal and larger than 5 cm, in 6 cases, the tumor was encapsulated, and was well differentiated in 11 cases. Lymph node invasion was observed in 2 cases and the portal vein was invaded in 5 cases. Before orthotopic liver transplantation, alpha foetoprotein was increased in 10 cases. Kaplan-Meier actuarial survival was 76%, 53%, 40%, at one, two and three years, respectively, and remained unchanged at four years. There were 9 recurrences of hepatocellular carcinoma. There were 8 deaths, 4 due to a tumor recurrence. A tumor larger than 5 cm was the only parameter statistically associated with recurrence in this study.

We report the results of a morphological analysis of 60 pulmonary biopsies gathered from a multi center study, organised by the clinico-pathological research group on Wegener's Disease under the auspices of the French Language Society of Thoracic Medicine. Forty of the sixty cases analysed were retained after indexing the histological aspects in order to specify their diagnostic value. Two groups of lesions were distinguished, which had different significance. Group A: These include the three major diagnostic criteria, which reinforce one another as they associate: 1) The polymorphoneutrophil microabscesses with limited central necrosis or an extended necrosis like the contours of a relief map. 2) An angiitis (arteries, veins, capillaries) with eccentric focal parietal crescent-shaped microabscesses. 3) Polymorphous granulomas with giant cells. Group B: In this group are the minor morphological observations (table II) of a lesser value and significance. 1) Acute or chronic lesions with alveolar haemorrhage, endogenous lipid pneumonia, xanthomatous granulomas, an organising pneumonia with an alveolitis. 2) Bronchial lesions: Bronchitis and necrotising bronchiolitis, which is more rarely follicular. 3) Sero-fibrinous or infiltrative neutrophil pleural lesions with focal microabscesses, elastolysis and elastophagia with giant cells in the elastic lamina. Thirteen cases presented with misleading lesions, which was a possible source of diagnostic error and led to a discussion of several associated disorders (Goodpasture's syndrome, and collagen disorder syndrome) or there may be systemic angiitis (Giant cell or lymphocytic) or also systemic or tissue eosinophilia (Churg-Strauss syndrome, bronchocentric granulomatosis) or necrotising bronchitis (atrophic polychondritis) or other forms of nodular interstitial fibrosis, such as histiocytosis X. We would like to stress the great polymorphic variation of the lesions and the difficulties which confront pathologists in the diagnosis of Wegener's Disease, above all when it is localised to the lung. There is value in finding at least one major diagnostic criteria which is associated with a minor criteria and with the help of the C.ANCA levels may lead to a narrow clinicopathological correlation and allows for a fairly precise approach to the diagnosis and identification of early or unusual lesions and thus to the early treatment of patients before irreversible renal failure appears.

Authors present a case of a cutaneous T. cell lymphoma (mycosis fungoides) complicated by a cauda equina syndrome. Crural and sciatic radicular pain were due to epidural lumbar involvement by tumoral process. After surgical decompression completed by chemotherapy the evolution was uneventful. This case is the second one reported in the literature.

The authors report the case of a 69 year old man who developed a disease clinically, radiologically and pathologically indistinguishable from bronchiolitis obliterans organizing pneumonia (BOOP) as described by Epler (1985). The patient responded completely to corticosteroids.

The authors present a case of Abrikossoff's tumor located in the segmental bronchus and detected on a systematic chest radiography, diagnosed during an endoscopy is reported. The CT study specified the tumoral extension. Bronchial distal affections is very rare. A confrontation with the anatomopathological data allowed a better understanding scanographic aspect.

In the case described, the onset of haemoptysis led to the discovery of a rounded opacity at the left base. All investigations being negative, thoracotomy was performed. The diagnosis could then be made by histology. Bronchopulmonary "tumourlets" are rarely seen (184 cases) masses of epithelial cells which are usually discovered by chance within areas of altered pulmonary tissue. Forms with clinical or radiological manifestations are extremely rare, only 4 other cases having been described. The pathogenesis of these tumours has been the object of recent ultrastructural studies which have shown the relationship which exists between "tumourlet" and carcinoid tumour. It would thus seem reasonable to classify "tumourlets" amongst the tumours of the diffuse endocrine system. The authors nevertheless feel that it would be premature to name them as bronchial carcinoids of tumourlet type. The concept of "tumourlet" as a separate entity should be retained.

The authors report a case of Wegener's granulomatosis, whose initial manifestation was an isolated endobronchial stenosis. In connection with this observation, it calls to mind, within the scope of the pulmonary granulomas (in view of such a rare clinical presentation) the difficulties of differential diagnosis, in particular with bronchocentric granulomatosis, and stresses the need for pulmonary histological examination to open lung biopsy.