Leser-Trélat syndrome secondary to non-small-cell lung carcinoma.

The syndrome of Leser-Trélat (LT) is a rare paraneoplastic syndrome. However, patients presenting with the sign of Leser-Trélat should be considered to harbor an occult malignancy or a progressive tumor disease until "proven" otherwise. Herein, we present two cases of non-small-cell lung carcinoma associated with LT syndrome.

Predominance of the Rare EGFR Mutation p.L861Q in Tunisian Patients with Non-Small Cell Lung Carcinoma.

Objectives: Several new cancer therapies targeting signaling pathways involved in the growth and progression of cancer cells were developed as personalized medicine. Our study aimed to identify epidermal growth factor receptor (EGFR) mutations for TKI treatment in non-small-cell lung cancer (NSCLC) Tunisian patients. Methods: Analysis of the TKI sensitivity mutations in exons 18 to 21 of the EGFR gene and exon 15 of the B-raf gene was performed in 79 formalin fixed-paraffin embedded (FFPE) NSCLC samples using pyrosequencing. Results: EGFR mutations were detected in 34 cases among 79 (43%), with the predominance of the L861Q in exon 21 found in 35.3% of the cases (12 out of 34). Deletions in exon 19 were found in 8 cases (23.5%), and only one young male patient had the T790M mutation. Three patients harbored composite EGFR mutations (p.E746_A750del/p.L861R, p.E746_S752>V/p.S768I, and p.G719A/p.L861Q). Furthermore, the EGFR mutated status was significantly more frequent in female patients (p = 0.019), in non-smoker patients (p = 0.008), and in patients with metastasis (p = 0.044). Moreover, the B-raf V600E was identified in 5 EGFR negative patients among 39 analyzed samples (13.15%). Conclusion: The p.L861Q localized in exon 21 of the EGFR gene was the most common mutation identified in our patients (35.3%), whereas the "classic" EGFR mutations such as Del19 and p.L858R were found in 23.5% and 11.7% of the cases, respectively. Interestingly, most of p.L861X mutation-carrying patients showed good response to TKI treatment. Altogether, our findings suggest a particular distribution of the EGFR-TKIs sensitivity mutations in Tunisian NSCLC patients.

Primary thoracic synovial sarcomas: A case report.

Primary pulmonary synovial sarcoma (SS) is a rare neoplasm. Its clinicoradiologic attributes are not yet well defined. We report the observation of a patient followed for primary pulmonary synovial sarcoma. We report the radio-clinical features of this rare tumor.

Metastasis of small cell bronchopulmonary cancer in the submandibular gland: a new case.

The metastases of bronchopulmonary cancer in the submandibular gland are infrequent. We report a case of metastasis in the submandibular gland in a patient with small cell lung cancer who had chemotherapy with disease stability.

Analysis of Genetic Alterations in Tunisian Patients with Lung Adenocarcinoma.

The identification of the mutations that drive lung cancer have furnished new targets for the treatment of non-small cell lung cancer (NSCLC) and led to the development of targeted therapies such as tyrosine kinase inhibitors that are used to combat the molecular changes promoting cancer progression. Furthermore, biomarkers identified from gene analysis can be used to detect early lung cancer, determine patient prognosis, and monitor response to therapy. In the present study we analyzed the molecular profile of seventy-three Tunisian patients with lung adenocarcinoma (LAD). Mutational analyses for EGFR and KRAS were performed using direct sequencing, immunohistochemistry or MassARRAY. Anaplastic lymphoma kinase (ALK) rearrangement was evaluated by immunohistochemistry using the D5F3 clone, and p53 expression was also assessed. The median age of patients at diagnosis was 61 years (range 23-82 years). Using different methodologies, EGFR mutations were found in 5.47% of patients and only exon 19 deletions "E746-A750 del" were detected. KRAS mutations were present in 9.58% of cases, while only one patient was ALK-positive. Moreover, abnormal immunostaining of p53 was detected in 56.16% of patients. In conclusion, the detected rates of EGFR and KRAS mutation and ALK rearrangement were lower than those found in European and Asian countries, whereas, abnormal p53 expression was slightly more frequent. Furthermore, given the small sample size of this study, a more comprehensive analysis of this patient set is warranted.

How does serum brain natriuretic peptide level change under nasal continuous positive airway pressure in obstructive sleep apnea-hypopnea syndrome?

BACKGROUND: Obstructive sleep apnea-hypopnea syndrome (OSAHS) is associated with cardiovascular morbidity and mortality, which can be improved by using continuous positive airway pressure (CPAP) therapy. However, the pathophysiological links between the two kinds of disease and the mechanism of the CPAP effect remain incompletely understood. We aimed to inquire into the myocardial involvement in this relationship. We suggested that serum brain natriuretic peptide (BNP) is sensitive enough to detect myocardial stress caused by OSAHS. DESIGN AND METHODS: Sixty-four subjects without cardiovascular disease (21 controls, 24 normotensive OSAHS patients, and 19 hypertensive OSAHS patients) were analyzed for serum BNP at baseline and serially over 6 months. CPAP was applied to 23 patients with severe OSAHS. RESULTS: At baseline, the serum BNP levels were significantly higher (p=0.0001) in the OSAHS group (22.3±14.79 pg/ml) than in the control group (9.2±6.75 pg/ml). Increased serum BNP levels were significantly associated with mean transcutaneous oxygen saturation (SpO2) (p<0.0001), minimal SpO2 (p=0.002), oxygen desaturation index (p=0.001), and total sleep time spent with SpO2 lower than 90% (p=0.002). All patients with elevated BNP levels (≥37 pg/ml) had moderate or severe OSAHS (11/43 OSAHS patients). The more severe the OSAHS, the higher the BNP levels were. However, only the difference between severe and mild OSAHS was statistically significant (p=0.029). Hypertensive OSAHS patients had the highest baseline BNP levels (27.7±16.74 pg/ml). They were significantly higher (p=0.001) than in normotensive OSAHS patients (18±11.72 pg/ml) (p=0.039) and the controls (9.2±6.75 pg/ml). As compared with baseline, treatment with CPAP significantly decreased BNP levels in both hypertensive and normotensive OSAHS patients (respectively, from 36±16.10 to 29.7±14.29 pg/ml, p<0.001, and from 20±10.09 to 16±8.98 pg/ml, p<0.001). In contrast, the BNP levels slightly increased in the controls (from 9.2±6.75 to 9.5±7.02 pg/ml, p=0.029), but there was no statistically significant difference in comparison with the baseline value. The effect of CPAP on BNP levels was more marked in patients with higher baseline BNP levels and those with the most prolonged nocturnal desaturation (p=0.001, r=0.65). It was also more marked in hypertensive OSHAS patients (p=0.015, r=0.72) in comparison with normotensive OSAHS patients (p=0.03, r=0.62). CONCLUSION: BNP seems to be sensitive enough to detect myocardial stress caused by OSAHS. As such, it is a potential marker for screening of preclinical cardiovascular damage in patients with untreated OSAHS. Application of CPAP decreases levels significantly in normotensive and particularly in hypertensive OSAHS. These findings are consistent with previous results suggesting the potential benefits of CPAP on cardiovascular outcome in OSAHS patients.

Do inhaled corticosteroids increase the risk of Pneumocystis pneumonia in people with lung cancer?

Pneumocystis pneumonia (PCP) is a life-threatening infection in immunocompromised patients. It is relatively uncommon in patients with lung cancer. We report a case of PCP in a 59-year-old man with a past medical history of chronic obstructive pulmonary disease treated with formoterol and a moderate daily dose of inhaled budesonide. He had also advanced stage non-small lung cancer treated with concurrent chemo-radiation with a cisplatin-etoposide containing regimen. The diagnosis of PCP was suspected based on the context of rapidly increasing dyspnea, lymphopenia and the imaging findings. Polymerase chain reaction testing on an induced sputum specimen was positive for Pneumocystis jirovecii. The patient was treated with oral trimethoprim-sulfamethoxazole and systemic corticotherapy and had showed clinical and radiological improvement. Six months after the PCP diagnosis, he developed a malignant pleural effusion and expired on hospice care. Through this case, we remind the importance of screening for PCP in lung cancer patients under chemotherapeutic regimens and with increasing dyspnea. In addition, we alert to the fact that long-term inhaled corticosteroids may be a risk factor for PCP in patients with lung cancer. Despite intensive treatment, the mortality of PCP remains high, hence the importance of chemoprophylaxis should be considered.

[Thoracic involvement revealing lymphomas: report on 17 cases]. / Manifestations thoraciques revelatrices de lymphomes: a propos de 17 cas.

BACKGROUND: Thoracic manifestations of lymphomas are frequents and various. They are frequently described in secondary than as a primary manifestation. AIM: This work aims to analyze the clinical, radiological, and prognostic characteristics of initial thoracic features of lymphomas. The authors stress the diagnosis and therapeutic difficulties which they encountered. METHODS: We report a retrospective work of initial thoracic involvement of lymphomas and their radiologic features observed in 17 patients hospitalized in pneumology service of Sfax. RESULTS: There were 10 cases of non Hodgkin's lymphoma and 7 cases of Hodgkin disease. For the patients with non Hodgkin's lymphoma, the mediastinal involvement was present in 8 cases. The parenchymal involvement was noted among 2 patients such as nodules or masses, with concomitant mediastinal lymph nodes. The pleural effusion such as pleurisy or solid mass was seen in 3 cases. Pericardial and parietal involvements were noted in one case. For the patients with Hodgkin's disease, the mediastinal lymph nodes were seen among 6 patients. The parenchymal involvement was noted in one case. One case of primary thymic lymphoma involvement and one case of tracheal involvement were noted. No case of pleural effusion was deplored. CONCLUSION: Initial thoracic involvements, rarely described, cause a variety of features which are often nonspecific. The diagnosis, often difficult, has beneficiated, from imaging and endoscopy's progress. However, treatment should be optimized in order to improve the prognosis which is also reserved particularly in non Hodgkin lymphoma.

[Narcolepsy]. / La narcolepsie.

Narcolepsy is a chronic neurologic disease whose main features are excessive daytime sleepiness and cataplexy. It is a rare disorder which is most frequently sporadic. The age of onset shows tow peaks. Both genetic and environmental factors play in its pathophysiolgy. Association with a specific HLA antigen and implication of the hypocretin system are very important findings. Narcolepsy's diagnosis is based on clinical findings but polysomnographic monitoring and multiple sleep latency test are usually necessary. Treatment of narcolepsy has been substantially modified with the advent of Modafinil which is effective as amphetamine with less undesirable effects. New etiologic treatments, such as hypocretin agonists, are currently being developed.

[A rare primary pulmonary tumor: pleomorphic carcinoma]. / Une tumeur primitive rare du poumon: le carcinome pléomorphe.

INTRODUCTION: Pleomorphic carcinoma is a rare malignancy in the family of non-small cell lung cancers. CASE: This 65-year-old woman, a nonsmoker, was hospitalized for hemoptysis and weight loss. Computed tomography showed a peripheral tumor of the right upper lobe, and the radiography showed signs of malignancy. Pathologic examination of the transparietal biopsy confirmed the tumor diagnosis, classifying it as stage III A. The patient underwent 2 cycles of induction chemotherapy that combined gemcitabine and cisplatin. Disease progression continued, however, and distant metastases were observed. The patient died 4 months after diagnosis. DISCUSSION: Pleomorphic carcinoma is identified by purely histologic criteria: the concomitant presence of malignant epithelial and homologous sarcomatoid spindle-cell components. Like the other non-small cell lung cancers, treatment is primarily surgical, and the invasive character of this tumor makes it very difficult. Pleomorphic carcinoma has a poorer prognosis than conventional non-small cell lung cancers despite surgery, irradiation and chemotherapy, because relapse occurs early.

[Toward optimization of small cell lung cancer management]. / Vers une optimisation de la prise en charge des carcinomes à petites cellules du poumon.

Small cell carcinoma represents 20 to 25 % of all lung cancers. It is characterized by his quickly dissemination. Chemotherapy is the comer stone of small cell lung cancer therapy. However, therapeutic approach are depending on patient's performans states and the disease's extension. The role of radiotherapy is established in the treatment of limited disease. However irradiation's modalities are still discussed. Surgery, usually, contraindicated is, actually, possible in early stages (T1T2N0). Recent advances in molecular biology have created new molecular targeted therapies for SCC but the benefits are not still evaluated. In this article, we review briefly classic strategies that have been evaluated in the management of patients with small cell carcinoma. This review will, also, focus in the main novel cytotoxic drugs and radiotherapy's modalities and for their clinical implication.