Granular cell tumor of the lung and tracheobronchial tree: Two case-presentation with a review of the literature.

Pulmonary granular cells tumors (CGT) are rare tumors, that derive from Schwann cells. In the tracheobronchial and pulmonary tree, they remain a diagnostic challenge. There are no well-established criteria to differentiate between benign, atypical, and malignant GCT. Moreover, its real frequency in the respiratory tract is still unknown. Here, we represent 2 cases of bronchial and lung GCTs. We aim to highlight the frequency of all clinicopathological characteristics of this rare tumor in the tracheobronchial and pulmonary tree location based on our cases and the available literature in a large systematic review.

Is critical thinking potential correlated to critical appraisal skills of medical literature?

INTRODUCTION: Critical appraisal of medical literature is a mandatory skill to practice evidence-based medicine. The relation between the critical appraisal skills and the critical thinking potential has been rarely assessed in the literature. AIM: To assess the relation of critical thinking potential to critical appraisal of medical literature competencies. Besides, they tried to highlight the variation of the critical thinking potential according to the students' level. METHODS: The authors conducted a mixed study associating a quantitative and a qualitative approach. The study included second year (SYME), third year (TYME) and postgraduate medical students (CME) and family doctors (FD) from the same faculty of medicine. All the students received the same active training focused on critical appraisal of medical literature. They were asked to fulfil a pre-requisite test and a self-assessment questionnaire before the training session and a final test after the training. The self-assessment questionnaire was conceived by an expert committee and assessed the main characteristics of critical thinking consisting of sensitivity to context, self-correction and search for criteria. Three months after the training, the students were interviewed using a semi-structured interview. The SPSS software 16.0 was used. RESULTS: In our study, 58.9% of the students presented a high critical thinking potential profile. Their scores varied according to their levels with better scores in SYME and FD and intermediate scores in TYME and CME. The pre-requisite test scores varied according to the critical thinking profile and the level. On the other hand, final test scores didn't differ according to the critical thinking profile or the level. CONCLUSION: Our results put emphasis on the variation of the critical thinking potential according to the students' levels. The better results of the SYME students in comparison to those in the TYME put emphasis on the necessity of changing curriculum in order to enhance the sensitivity and the inclination of the students.

Spindle cell hemangioma of the lung: An unusual presentation.

Spindle cell hemangioma (SCH) is a benign vascular tumour, first identified by Weiss and Enzinger in 1986. Habitually, the SCH affects almost exclusively the dermis and subcutaneous tissues of distal extremities. So far, only 2 cases have been described in the lung. We describe herein the third case of SCH occurring in the lung in a 47 year-old woman. The patient was successfully treated by right lower lobectomy. The histopathological and immunohistochemistry examination of the excised tumour leads to the definitive diagnosis. Our case is instructive by its different clinical and radiological presentation compared to the previous two cases.

Collision lung tumor associating typical carcinoid tumor to sclerosing hemangioma.

Collision tumors associating carcinoid tumor and sclerosing pneumocytoma have rarely been reported in the literature. The clinical presentation may be challenging especially in cases showing multiple and bilateral nodules. This case illustrates the association of both tumors diagnosed incidentally and illustrates a full spectrum of neuroendocrine lesions and sclerosing pneumocytoma. The authors present the case of a 52-year-old patient presenting an abdominal pain revealing a vesicular lithiasis and multiple pulmonary nodules. Radiologic follow-up of the asymptomatic lung lesions revealed the stabilization of a left lobe lesion with a disappearance of the other lesions. A lobectomy with a mediastinal lymph node curettage was performed. The microscopic examination revealed both tumor components associating a typical carcinoid tumor to a sclerosing pneumocytoma in association to lesions of diffuse neuroendocrine hyperplasia present in the peri-tumoral parenchyma. This case illustrates radiologic, gross, and microscopic features of a rare pulmonary tumor.

About a lymphoepithelioma-like carcinoma of the lung with an endotracheal localization.

INTRODUCTION: Lympho-epithelioma like carcinoma is a rare lung tumour that acounts for less than 1% of non small cell lung carcinomas. It is defined as a special entity among the 2015 World Health Organization. AIM: Our aim was to describe a completely illustrated new case of lymhpo-epithelioma like carcinoma. We describe a new case diagnosed in our Department of Pathology. CASE PRESENTATION: The authors describe a case of a 22-year-old woman without a particular past medical history who presented non specific respiratory symptoms. Radiologic investigations revealed a tracheal tumor with enlarged mediastinal lumph nodes. A first biopsy was performed revealing a malignant tumor with a squamous differentiation highlighted by immunohischemistry. A surgical biopsy was performed and the final microscopic diagnosis revealed a lymphoepithelioma-like carcinoma of the lung. This diagnosis was retained after ruling out a possible metastasis of a nasopharyngeal carcinoma whose microscopic features are similar to this subtype of lung tumor. CONCLUSION: This case points out the rarity of this diagnosis, especially in a young and caucasian patient and highlights the diagnostic dilemma caused by this kind of tumor.

Prognostic impact of the 2015 world health organisation classification of lung adenocarcinoma : a study in departement specialized in thoracic pathology.

BACKGROUND: The 2015 WHO classification of primary lung adenocarcinomas idendified not only new subtypes but also new prognostic groups. Solid and micropapillary adenocarcinomas are considered of bad prognosis, lepidici adenocarcinomas are considered of good prognosis and acinary and papillary ones are considered of intermediate prognosis. We aimed to assess the prognostic relevance of this classification through the experience of a specialized Department in Thoracic Oncology. METHODS: We described a survival study about 101 patients presenting primary adenocarcinomas diagnosed between 2008 and 2011. Survival curves were compared using the Log-Rank test. In order to assess the prognostic impact of the classification, 3 groups were formes: group 1 included lepidic adenocarcinomas, le group 2 included acinary and papillary subtypes and group 3 included solid subtype. A univariate analysis was performed to assess the age, sex, size, vascular emboli, lymph nodes, pTNM, histologic subtypes, neoadjuvant treatment and adjuvant treatment. Multivariate analysis using COX model was performed. RESULTS: There was no significant difference between the different subtypes. Besides, the multivariate analysis revealed the pTNM (p=0,02)  stage and the adjuvant treatment (p=0,007) as relevant prognostic factors. CONCLUSION: Our results are discordant with those reported in the literature. It seems that including large series is necessary in order to assess this prognostic classification.

Interstitial pneumonia in a glassblower: think to chronic beryllium disease!

Chronic beryllium disease (CBD) is an occupational illness with varying severity. In this report, we describe a 27 year old man, glassblower, who developed a fatal CBD after six months of unknown Beryllium's exposure. The diagnosis was suspected on histological examination and then consolidated by confirmation of Beryllium's exposure at the working area. Physicians should be aware of the potential risk to develop CBD in glassblowers. These workers should benefit from early medical surveillance using the Beryllium lymphocyte proliferation test (BeLPT) and therefore from suitable management.

Second lung malignancy and Richter syndrome in chronic lymphocytic leukemia: case report and literature review.

BACKGROUND: Chronic lymphocytic leukemia (CLL) is the most frequent lymphoproliferative disease. Transformation into Richter disease and occurrence of second malignancies involving the lungs are rare complications. The hallmarks of any thoracic involvement are still unknown. CASE PRESENTATION: We report a case of a 56-year-old male patient, with history of tobacco smoking, who presented with recurrent hemoptysis, fatigue and weight loss. Physical examination was normal except a slightly enlarged supraclavicular lymph node. Chest x-ray revealed a mediastinal widening due to enlarged paratracheal nodes and a left parahilar infiltrate. Blood tests showed a hyperlymphocytosis and a biological inflammatory syndrome. CT scan showed bilateral mediastinal and axillary lymphadenopathy, as well as left supraclavicular lymphadenopathy, with a left upper lobe alveolar attenuation and a solitary contralateral pulmonary nodule. Examination of Virchow's node and bone marrow biopsies confirmed metastasis of a pulmonary adenocarcinoma, as well as chronic lymphocytic leukemia with Richter's transformation. The clinical course was unfavorable since the first days of therapy as the patient passed away in a matter of a few days. CONCLUSIONS: Steady surveillance of CLL patients and systematic screening for second solid tumors, particularly lung cancer, and Richter's transformation seem to be relevant more than ever. Early diagnosis might help us understand the pathways leading to these complications and adapt therapy.

The diagnostic importance of the bronchoalveolar lavage in lymphocytic alveolitis.

Multidisciplinary concertation is mandatory in order to assess interstitial pneumonias. The study of the bronchoalveolar lavage helps evoking a diagnosis according to the lavage profile. In lymphocytic alveolitis, immunocytochemistry, or in flux cytometry are necessary in order to identify the different clusters of lymphocytes implicated. Our objective was to evaluate the profile of 31 lymphocytic alveolitis using 2 different techniques which are the immunocytochemistry and the in flow cytometry in order to evaluate the efficacy of each technique and to compare the different results to the final diagnoses. We describe a retrospective study about 31 patients admitted to our hospital in order to explore an interstitial pneumonia between January and July 2014. Bronchial endoscopy and bronchoalveolar lavage were performed in all cases. The sensitivity of the in flow cytometry was estimated to 53% and its specificity reached 33%. On the other hand, the immunocytochemistry presented a specificity of 42.8% and a sensitivity of 42.8%. The final diagnoses retained consisted in sarcoidosis in 12 cases, infectious pneumonia in 10 cases, hypersensitivity pneumonia in 3 cases, cryptogenic pneumonia in 3 cases, idiopathic fibrosis in 2 cases, and adenocarcinoma in 1 case. The relevance of both techniques depends on many factors. They necessitate an available material, well-trained technicians, and experimented pathologists.

Synchronous mantle cell lymph node lymphoma and pulmonary adenocarcinoma: a case report with literature review.

BACKGROUND AND AIMS: Coexistence of mantle cell lymphoma and lung adenocarcinoma is extremely rare. The aim of this study is to present the first case of incidental discovery of primary lung adenocarcinoma associated to lymph node mantle cell lymphoma. METHODS: A 45 year-old man, admitted for inguinal mass appeared since three months. Physical examination showed a 1 cm axillary and 3 cm inguinal adenopathies which were hard and fixed. A biopsy of inguinal mass revealed mantle cell lymphoma. Assessment of disease extension found a right superior lobe tumor with mediastinal lymphadenopathies. Fibreoptic bronchoscopy was normal. A fine needle biopsy of lung tumor was performed to determinate the nature of the pulmonary lesion. Histopathological findings were consistent with primary poor differentiated pulmonary adenocarcinoma with TTF-1 expression. A first mediastinoscopy was realized to verify mediastinal lymphadenopathies; frozen section showed a mantle cell lymphoma. A right lobectomy was then performed confirming the diagnosis of lung adenocarcinoma. Then, the patient was treated for the mantle cell lymphoma by 6 cures of alternating RCHOP and RDHAP (dexamethasone, high-dose Ara-Cytarabine and cisplatin) regimens followed by autologous stem cell transplantation. RESULTS: The patient died because of treatment complications 3 years after diagnosis. CONCLUSION: Progresses are necessary to understand the pathogenesis of of synchronous occurrence of both diseases.

Clinicopathologic and prognostic factors of thymoma in Tunisia.

BACKGROUND: although rare, thymomas are the most common tumors of the thymus in adults. They represent about 20% of all mediastinal tumors. AIM: the aim of this study is to present clinicopathological features of thymomas in Tunisia and analyse the prognostic factors. METHODS: From 1993 to 2004, clinical data of 40 cases of thymomas were compiled retrospectively. Microscopic slides were reviewed and reclassified according to the WHO classification of thymic tumors 2004. Clinical staging adopted was Masaoka system. Analysis of survival was determined by Kaplan-Meier method and log-rank test was used to compare survival curves. These statistical analyses were performed by SPSS. RESULTS: they were 23 women and 17 males of ages ranging from 14 to 76 years (mean age 51 years). The distribution of histological WHO types was: 1 type A, 7 type AB, 6 type B1, 17 type B2, 6 type B3, 2 cases of micronodular thymoma with lymphoid stroma and 1 case of metaplastic thymoma. According to Masaoka stage, 10 patients were in stage I, 11 stage II, 9 stage IIIa, 4 stage IIIb, 5 stage Iva and 1 stage IVb. The average overall survival was 56 months. Univariate analyses showed that Masaoka stage, completeness surgical resection and age were prognostic factors whereas in multivariate analysis, age was the only prognostic factor. Neither myasthenia gravis nor histological WHO subtypes had effect in survival. CONCLUSION: masaoka stage, completeness surgical resection and age are the prognostic factors predicting survival in our series.

The inter-connection between fibrosis and microvascular remodeling in idiopathic pulmonary fibrosis: Reality or just a phenomenon.

BACKGROUND: Idiopathic pulmonary fibrosis is the most frequent interstitial disease with the worst prognosis. It is characterized by an uncontrolled fibrosis which is difficult to manage. The pathogenesis of this disease remains unclear with many theories resulting in multiple target therapies. The relation between fibrosis and vascular remodeling has been debated in the literature with different results that may seem contradictory. AIM: We target to evaluate the connection between fibrosis and vascular remodeling in usual interstitial pneumonia. MATERIAL AND METHODS: 26 cases of idiopathic pulmonary fibrosis were reviewed by 2 pathologists and the diagnosis of UIP was retained according to the American Thoracic Society's criteria. Fibrotic changes and vascular remodeling were evaluated blindly. The fibrotic changes were classified as severe, intermediate and mild. Vascular occlusion was graded in 4 grades extending from medial hypertrophy (grade 1) to plexiform lesions of the vascular wall (grade 4). RESULTS: We noticed that severe degrees of fibrosis were correlated with severe grades of vascular obstruction. In fact, our 26 cases were classified as severe fibrosis in 11 cases with grade IV vascular lesions in 6 cases, intermediate fibrosis in 12 cases with grade II vascular lesions in 8 cases and mild fibrosis in 3 cases with grade I vascular lesions in all cases. CONCLUSION: Many theories have been reported concerning the UIP's pathogenesis. Recently, many authors reported that the primum movens of these lesions was an epithelial/endothelial injury which induces uncontrolled fibrosis and microvascular remodeling using different pathways. This puts emphasis on the necessity of multi-target therapies in order to improve the management of this fatal disease.

About thymic carcinomas: challenges in diagnosis and management.

BACKGROUND: Thymic carcinomas are rare tumors with a challenging diagnosis. Our aim was to report our 17-year experience of these tumors and to highlight the challenges encountered and the main differential diagnoses ruled out. METHODS: We studied 12 (92%) men and 1 (7.7%) woman with a mean age of 37 years (range 15-60 years). All patients were symptomatic, with chest pain representing the most frequent symptom. Radiology revealed anterior mediastinal masses in all cases, with either infiltration of the adjacent organs or pulmonary parenchymal metastases. RESULTS: The diagnosis was made on surgical biopsies in 12 cases and a lymph node biopsy in one. Microscopic examination revealed squamous carcinoma in 3 cases, synovial sarcoma in 1, mucinous adenocarcinoma in 1, undifferentiated carcinoma in 2, clear cell carcinoma in 1, lymphoepithelioma-like carcinoma in 2, atypical carcinoid tumor in 2, and sarcomatoid carcinoma in 1. Total surgical resection was possible in one patient after neoadjuvant chemotherapy and radiotherapy. Follow-up was possible in only 6 patients, and the mean survival reached 13 months. CONCLUSION: In spite of the lack of follow-up information, this study demonstrates the poor outcome associated with these tumors and the need for standardized treatment.

Malignant glomus tumor of trachea: a case report with literature review.

Glomus tumors of the trachea are extremely rare and generally benign, with very few cases of malignant transformation reported in literature. We present the case of a 74-year-old man explored for cough and dyspnea. Bronchoscopy showed a polypoid mass arising from the posterolateral tracheal wall. Computed tomography demonstrated a mid-tracheal tissular mass obliterating the tracheal lumen. Resection and anastomosis of the trachea were performed. The histological and immunohistochemical findings were consistent with malignant glomus tumor.

Genomic classification of lung cancer: toward a personalized treatment.

Lung cancer is the first cause of death by cancer worldwide. In Tunisia, its incidence has increased from 17.6 cases per 100.000 persons in 1997 to 27.6 cases per 100.000 persons in 2003. Its prognosis has been improving thanks to the emergence of molecular targets. The first one is represented by EGFR (Epidermal growth factor receptor), which marks this (2014) its tenth anniversary. many other targets have been identified. the most famous and useful of them the fusion gene ALK-EML4 but other oncogenic pathways have been implicated and under investigations including HER2, BRAF, MET, RET... The relevant challenges encountered are represented by the difficulty to achieve a consensual decisional and therapeutic algorithm, the absence of standardized diagnostic techniques and unavoidable occurrence of secondary resistance due to the activation of other oncogenic pathways that must be explored and targeted. In this update, we tried to present the major pathways implicated and the most relevant practice routine strategies.

[Multicentric tubulopapillary carcinoma of the kidney associated with renal adenomatosis]. / Carcinome tubulopapillaire multicentrique du rein associé à une adénomatose rénale.

Tubulopapillary tumors of the kidney represent a particular group of the renal tumors. They cover a continuous spectrum of tumors ranging from the adenoma to the renal cells papillary carcinoma. The histological and immunohistochemical similarities, as well as the high recurrence of the association of these two entities suggest a continuity of the same biological process. Although rare, the association between adenoma and papillary carcinoma remains still subject to controversy and plead in favor of a narrow relation between them. We report the case of a multicentric tubulopapillary carcinoma of the kidney associated with multifocal adenomas, discovered by fortuity in a right nephrectomy at a 57-year-old patient operated for an obstructive chronic pyelonephritis.

A unilocular thymic cyst associated with true thymic hyperplasia: a challenging diagnosis especially in a child.

We report a new case of a mediastinal mass in a 19-year-old patient corresponding microscopically to an association of unilocular thymic cyst and true thymic hyperplasia. Our aim is to highlight the absence of specificity of clinical and radiological findings and the necessity of a thorough sampling of the tumor in order to establish the diagnosis.

[Fatal pulmonary mycosis in a diabetic and cirrhotic patient]. / Une mycose pulmonaire d'issue fatale chez une patiente diabétique et cirrhotique.

Pulmonary mucormycosis is a rare, devastating, opportunistic fungal infection, caused by the ubiquitous filamentous fungi of the Mucorales order of the class of Zygomycetes. This infection occurs principally in some particular conditions, specially in diabetic patients and immunocompromised host, and rarely in cirrhotic patients. The diagnosis of mucormycosis can only be confirmed by pathological and mycological examination of biopsy specimens. We report a case of pulmonary mucormycosis in a 68-year-old woman with underlying liver cirrhosis and diabetes mellitus. Endoscopic and radiologic findings supported the diagnosis of hydatid cyst of the lung. The patient underwent surgical resection and was started on amphotericin B, after pathological examination. Unfortunately, she succumbed to the infection within one month of surgery.