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

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 year [2014] its tenth anniversary. Many other targets have been identified. The most famous and useful of them is the fusion gene ALK-EML4 but other oncogenic pathways have been implicated and are under investigations including HER2, BRAF, MET, RET…. The most relevant challenges encountered are represented by the difficulty to achieve a consensual decisional and therapeutic algorithm, the absence of standardized diagnostic techniques and the 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]

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

Sub-cutaneous histiocytic sarcoma in a child: a case report

Background: Histiocytic sarcoma [HS] is a rare hematologic malignancy with morphologic and immunophenotypic evidence of histiocytic differentiation. This tumor follows an aggressive clinical course

Case presentation: We report the case of a 14 year-old white girl who presented with a nodular lesion in the thigh, involving the skin and soft tissue. The histologic diagnosis retained was a HS

Conclusion: HS is a rare neoplasm that may cause a diagnostic pitfall. Unfortunately, incomplete clinical data and histopathologic disparities in addition to the overall rarity of the neoplasms induced difficulties of management and of full appreciation of their clinical behavior

[Carcinogenesis of non small cell lung cancer and therapeutic implications]

The occurrence of a lung cancer is a consequence of a long-lasting process dealing with a transformation of a normal cell to a malignant one. The four steps of transformation reflect the genetic modifications of the cells. The molecular studies of pre-invasive lesions have already established a correlation between the lesion continuum and the multi-step carcinogenesis. Gradual genetic alterations are correlated with the increase of the cell's malignant potential. We tried to present the carcinogenesis of the lung non microcellular carcinomas and to highlight the main therapeutic targets

[ new classification of lung adenocarcinomas according to the American Thoracic society and the European respiratory society: new recommendations to improve management]

Lung cancer represents a major public health problem.It represents the first cause of mortality by cancer in Tunisia. Its incidence reaches 40% of lung cancers. Its clinical, radiologic and molecular aspects have been improved inducing the necessity of a new classification which will consider the necessity of a multidisciplinary management. To highlight the new classification of lung adenocarcinomas and to present the major recommendations. We tried to present the main recommendations of the American Thoracic Society and the European Respiratory Society of lung adenocarcinoma. This new classification identifies pre-invasive lesions represented by in-situ adenocarcinoma [the ancient bronchioloalveolar], the micro-invasive adenocarcinoma and invasive adenocarcinoma. The latter have been divided in sub-types according to the predominant architectural features. Thus, three groups of invasive adenocarcinoma with presumed different prognoses have been identified: the lepidic predominant adenocarcinoma which has a good prognosis, the micro-papillary and solid predominant adenocarcinomas which have a bad prognosis and the papillary and acinar adenocarcinomas which have an intermediate prognosis. All these entities have specific diagnostic features and criteria. These recommendations are available for biopsies and surgical resected specimen. The new classification of lung adenocarcinoma puts emphasis on the necessity of a multi-disciplinary management of these tumors in order to improve their prognosis. It identifies new entities with different prognoses that could justify specific modalities of treatment and follow up

[Primary malignant tumors of the sternum]

Primary tumors of the sternum are rare and account only 0.5% of all primary bone tumors. They are often malignant, osteolytic and aggressive. They often present difficulties in management. To determine clinical, pathological and therapeutic characteristics for primary malignant tumors of the sternum. We report a series of six cases of primary malignant tumors of the sternum, collected in our institution between 1993 and 2009. There were 4 men and 2 women with a mean age of 69, 5 years. Parietal swelling was the most frequent symptom. Imaging showed a sternal lytic lesion. Three tumors were treated surgically. Associated treatments were neoadjuvant chemotherapy [1 case] and postoperative radiotherapy [1 case]. A medical treatment [radiotherapy alone or chemotherapy] was performed in 3 cases. These tumors were divided as below: 3 plasmacytoma, 1 chondrosarcoma, 1 osteosarcoma and 1 large B cell lymphoma. The management of primary malignant tumors of the sternum is multidisciplinary. It depends on the histological type, the possibility of surgical treatment and the distant and local aggressiveness

[Adjuvant chemothgerapy in resected non small cell lung cancer: preliminary results about 8 cases]

Report the preliminary results of adjuvant chemotherapy in patients surgically treated for non small lung cancer. Its a prospective study about 12 patients surgically treated between January 2005 and December 2007.8 patients had benefit of adjuvant chemotherapy. The protocol had been based at 4 cycles of Cisplatine Our 8 men patients, aged for the mean of 59 years with a Performans Status at 1, had benefit of adjuvant chemotherapy after curative surgery. Six patients considered us II B stage, had benefit immediately for surgically treatment. Adjuvant chemotherapy protocol had been based of Cisplatin and Vinorelbine [5 patients] and Cisplatin and Gemcitabine [1 patient]. The 4 cycles can be administered without any limiting toxicity only for one patient who's received 2 cycles of Cisplatin and Gemcitabine in front of the severity of digestive side effects. Two patients considered us IIIB stage, had been surgically treated after neo adjuvant chemotherapy based at Cisplatin and Vinorelbine. Histological response was complete for twice of them. The same chemotherapy was stopped after 2 cycles us adjuvant, in front of haematological side effects. Two patients did at 4 and 15 months of neoplasic progression. The six other patients had been still on life with a move back of 33 months. Post operative adjuvant chemotherapy is the standard treatment for the II A and II B stages and probably for lB stage. For none immediately operative patients [IIIA and some III B], articulation of chemotherapy with surgery must be clarified

[Bronchoalveolar lavage impact in sarcoidosis: study of 40 cases]

Studies on bronchoalveolar lavage [BAL] fluid samples profile characteristics in sarcoidosis patients hitherto reported in literature give rise to conflicting data. The typical finding is lymphocytic alveolitis with a high CD4/CD8 ratio, although a broad range of values has been found. The aim of the study was to evaluate different parameters of BAL fluid in patients with sarcoidosis before and after corticosteroid treatment and to determine the reliability of BAL in evaluating prognosis of sarcoidosis. The study involved 40 patients with clinical and histological evidence of sarcoidosis. Before treatment, BAL fluid showed an increase in total cell count and a T lymphocytosis with an increase in the CD4/CD8 ratio. After treatment, we have noted a significantly increase in macrophage proportion, a significantly decrease in lymphocyte proportion and in CD4/CD8 ratio. A significant higher CD4/CD8 ratio was noted in patients with multiple extrathoracic lesions compared with patients with only intrathoracic involvement and in patients with unfavourable evolution compared with patients with improved evolution. When typical clinical and radiological findings definitely suggest sarcoidosis, BAL results may add a biological support. Our data suggests that a high CD4/CD8 ratio seems to be predictive of poor prognosis with a multiple extrathoracic organ involvement and an unfavourable outcome

[Pulmonary lymphangiomatosis revealed by an interstitial syndrome in a young adult]

Diffuse pulmonary lymphangiomatosis is an extremely rare congenital lesion affecting the lymphatic pulmonary system. It is charcterized by a multifocal proliferation and a pulmonary lymphatic vessels dilatation. It is essentially observed in newborns and infants. The diagnosis is based on histological findings. The purpose of this article is to describe the main clinical and pathological features of this rare entity difficult to diagnose. We report a case of a 19-year old girl who presented with a 2- month standing dyspnea. Radiographic findings consisted in a soft tissue infiltration and thickening in the two lobes. Based on histological and immunohistochemical findings, the diagnosis of diffuse pulmonary lymphangiomatosis was retained. Diffuse pulmonary lymphangiomatosis is a difficult diagnosis which can be confused with lymphangioleiomyomatosis. Its worse prognosis could be improved by an early diagnosis enabling the use of interferon alpha 2B at the proper time