BACKGROUND: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.METHODS: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.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 organizing pneumonia. Surgical lung biopsy remains the reference method for diagnosis.

Background 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.Methods 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.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 organizing pneumonia. Surgical lung biopsy remains the reference method for diagnosis.

Le chérubisme est un processus extensif kystique atteignant surtout la mandibule, dont le diagnostic est souvent fait devant un faciès joufflu chez l’enfant. Son déterminisme familial est anciennement connu. Récemment identifiée, la mutation de l’exon 9 de SH3BP2 permet d’identifier les cas autosomiques dominants de la maladie. Les auteurs rapportent le cas d’un enfant de 14 ans, de grade I, de diagnostic tardif. Une recherche génétique familiale, faite à Berlin, n’a pas retrouvé la mutation du gène candidat. À côté des cas familiaux dominants, on retrouve des cas sporadiques, parfois liés à une néo-mutation identifiée du gène codant pour SH3BP2. Le cas présenté a des antécédents familiaux de troubles de l’homéostasie osseuse et dentaire qui peuvent argumenter l’hypothèse d’un mode de transmission autosomique récessif ou d’un autre gène candidat. 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.

INTRODUCTION: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.OBSERVATION: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.DISCUSSION: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.

Le neurothécome est une tumeur rare. Elle est bénigne mais doit être distinguée de certaines tumeurs malignes notamment les tumeurs fibro-histiocytaire à cellules plexiformes, les mélanomes neurotropes et les sarcomes à cellules claires. Une enfant de 11 ans a consulté pour la récidive d’une tumeur sous-cutanée mentonnière, opérée 4 mois plus tôt. L’exérèse avait été incomplète. Une reprise chirurgicale au large a permis de contrôler l’évolution. L’étude histologique a établi le diagnostic de neurothécome en particulier en raison de l’existence d’atypies et de mitoses, de nodules cellulaires et de l’extension à l’hypoderme. La confirmation a été apportée par l’immuno-histo-chimie. Le neurothécome cellulaire est une tumeur bénigne de la femme jeune, qui atteint souvent la face. Elle se présente comme un nodule dermique avec respect de l’épiderme. Ses caractéristiques immuno-histo-chimiques spécifiques permettent le diagnostic différentiel avec la forme myxoïde et les tumeurs mélanocytaires et nerveuses. Le traitement est l’exérèse chirurgicale. 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.

INTRODUCTION: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.CASE REPORT: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.DISCUSSION: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.

In France, primary lung cancer (all types combined) is the leading cause of cancer mortality in men, and the third in women, after breast and colorectal cancer.In 1995, lung cancer was responsible for 23.5% of cancer deaths in men and 6.4% in women.The incidence of lung cancer is higher in men than in women, and the mortality rate is nine times higher in men than in women.The lung cancer mortality rate is constantly increasing in France, particularly in the north of the country.The increase in incidence between 1975 and 1995 was more marked in women.In France, the 5-year survival rates (11.5% for men and 16% for women) are among the highest in Europe.

CONTEXT:The "Standards, Options and Recommendations" (SOR) project, started in 1993, is a collaboration between the Federation of French Cancer Centers (FNCLCC), the 20 French cancer centers and specialists from French public university and general hospitals and private clinics. Its 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.OBJECTIVES:To develop, according to the definitions of the Standards, Options and Recommendations, clinical practice guidelines for the management of non small cell lung carcinoma patients.METHODS:Data were identified by searching Medline and the personal reference lists of members of the expert groups, then submitted for review to independent reviewers. This is a short version of the SOR guideline covering diagnosis, treatment and follow-up and includes the algorithms for the management of patients with non-small cell lung cancer.

CONTEXT:The "Standards, Options and Recommendations" (SOR) project, started in 1993, is a collaboration between the Federation of French cancer centers (FNCLCC), the 20 French cancer centers, 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 the outcome of cancer patients. The methodology is based on a literature review and critical appraisal by a multidisciplinary group of experts, with feedback from specialists in cancer care delivery.OBJECTIVES:To develop clinical practice guidelines for non small cell lung cancer patients according to the definitions of the Standards, Options and Recommendations project.METHODS:Data were identified by searching Medline , web sites, and using the personal reference lists of members of the expert groups. Once the guidelines were defined, the document was submitted for review to independent reviewers.RESULTS:This article presents the chapter "Prognosis significance of oncogenes and tumor suppressor genes" from the full report "Standards, Options and Recommendation for non small cell lung cancer" validated in August 2000. The main recommendations are: 1) No clear clinical prognostic value of oncogenes and tumor suppressor genes (p53, bcl-2, Ki-ras, c-erbB-2, Rb, p16) in non small cell lung cancer, can be established from the available evidences (standard, level of evidence C). 2) Prospective multicenter studies should be performed to assess prognostic significance of oncogenes and tumor suppressor genes in non small cell lung cancer.

CONTEXT: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. OBJECTIVES: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. METHODS: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. RESULTS: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.

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

Background: 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.Patients and methods: 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.Results: 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. Age > 60 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 cycles > 6 (OR = 3.25, IC 95% (1.08-9.8) P = 0.02) and by an age > 60 (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: age less than or equal to 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.

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.

Les auteurs rapportent le cas d'un patient de 35 ans présentant un dermatopolymyosite associée à une trachéopathie ostéochondroplastique. L'évolution sera fatale avec infection pulmonaire favorisée par les troubles de la déglutition due à la polymyosite et à la perturbation de la clairance mucociliaire secondaire à la trachéopathie. La trachéopathie ostéochondroplastique est une affection rare diagnostiquée exceptionnellement avant 50 ans. L'association de deux pathologies rares n'est pas le fait du hasard. Elle invite donc à rechercher les circonstances étiologiques communes qui pourraient être chez ce patient l'inhalation de particules de silice.

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 rite occurrence of a disease related to tobacco consumption. About 20% of the LTSs die of noncancer 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.

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 occuptional 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.