A. Marghli

Desmoid-type chest wall fibromatosis. A six cases series.

PURPOSE OF THE STUDY Desmoid-type fibromatosis is a fibroblastic proliferation that develops in the deep soft tissues and is characterized by infiltrative growth and a tendency towards local recurrence but does not metastasize. Fibromatosis of the chest wall is rare, representing only 10 to 20% of all deep fibromatoses. CASE REPORT We report a six cases series of fibromatosis of the chest wall treated at our institution between 1996 and 2009. There were five men and one woman with a mean age of 42.2. Swelling of the chest wall was the most frequent symptom. Imaging showed an expansive homogeneous mass, invading the rib (four cases) or the sternum (two cases). Surgical resection was performed in all cases. The outcome was favorable (five cases) or marked by local recurrence (one case). DISCUSSION Although it is considered to be a benign lesion, this tumor can be locally very aggressive, and has a high local recurrence rate depending upon the efficacy of surgical resection.

Les sarcomes primitifs du médiastin

INTRODUCTION Primary sarcomas of the mediastinum are rare and account for 2 to 8% of malignant mediastinal tumours. The aim of this study is to describe their clinical and pathological characteristics. PATIENTS AND METHODS Fifteen cases of primary mediastinal sarcoma, diagnosed between 1993 and 2009, were reviewed retrospectively, noting the clinical, radiological and pathological findings, and the treatment given. RESULTS The patient population consisted of eight females and seven males with mean age of 40 years (14 to 73 years). The symptomatology was predominately respiratory. Imaging showed a mediastinal mass invading adjacent organs in nine cases. The diagnosis was made in all cases by histological examination. These 15 mediastinal sarcoma comprised 12 malignant peripheral nerve tumours, two liposarcomas and one angiosarcoma. Three were grade III, six grade II and six grade I. Ten were treated surgically, of which seven had radical resections. Associated treatments were neoadjuvant (one case) or adjuvant (one case) chemotherapy and postoperative radiotherapy (five cases). Radiotherapy was undertaken alone in three inoperable tumours. Eight patients (53%) had died. CONCLUSION Management of primary mediastinal sarcoma needs a multidisciplinary approach, and is based mainly on radical resection. The prognosis is poor and depends mainly on surgical excision and histological grade.

Tumeurs carcinoïdes bronchopulmonaires : une série tunisienne de 115 cas opérés

INTRODUCTION Bronchial carcinoid tumors are rare well-differentiated, neuro-endocrine tumors, with low-grade malignancy. They are distinguished in two different groups: typical and atypical carcinoid. The purpose of this study was to review clinical, surgical and pathological characteristics in patients with primary bronchopulmonary carcinoid tumors. METHODS This retrospective study included 115 bronchial carcinoid tumors surgically treated at a single institution during a twenty-one-year period, between 1992 and 2012. RESULTS There were 56 men and 59 women, with a mean age of 43.73 years. Hundred patients had typical carcinoids and 15 atypical carcinoids. The majority of patients were symptomatic (96.52%). CT revealed a proximal mass in 51% which was obstructive leading to ventilator disorders in 80%. Bronchoscopy showed an endo-bronchial tumor in 83.48% of the cases. Anatomical resection had been achieved among 99 patients, and a conservative resection among 16 patients with typical carcinoid tumor. Lymph node metastases were present in 12.17% of the cases. Follow-up revealed recurrence in one patient and distant metastasis in 4 others. The 5-year survival rate was worse for atypical carcinoid with 45% than typical carcinoid with 95%. CONCLUSIONS Carcinoids are rare malignant tumors, and their outcome is usually favorable after surgery. However, local recurrence and/or metastases can occur with both typical and atypical carcinoid tumors, justifying the need of prompt diagnosis and long-term follow-up. The factors influencing the survival included the pathological type, distant metastasis and mediastinal lymph node involvement.

Article originalLes sarcomes primitifs du médiastinPrimary mediastinal sarcomas

INTRODUCTION Primary sarcomas of the mediastinum are rare and account for 2 to 8% of malignant mediastinal tumours. The aim of this study is to describe their clinical and pathological characteristics. PATIENTS AND METHODS Fifteen cases of primary mediastinal sarcoma, diagnosed between 1993 and 2009, were reviewed retrospectively, noting the clinical, radiological and pathological findings, and the treatment given. RESULTS The patient population consisted of eight females and seven males with mean age of 40 years (14 to 73 years). The symptomatology was predominately respiratory. Imaging showed a mediastinal mass invading adjacent organs in nine cases. The diagnosis was made in all cases by histological examination. These 15 mediastinal sarcoma comprised 12 malignant peripheral nerve tumours, two liposarcomas and one angiosarcoma. Three were grade III, six grade II and six grade I. Ten were treated surgically, of which seven had radical resections. Associated treatments were neoadjuvant (one case) or adjuvant (one case) chemotherapy and postoperative radiotherapy (five cases). Radiotherapy was undertaken alone in three inoperable tumours. Eight patients (53%) had died. CONCLUSION Management of primary mediastinal sarcoma needs a multidisciplinary approach, and is based mainly on radical resection. The prognosis is poor and depends mainly on surgical excision and histological grade.

Une tumeur trachéale traitée comme un asthme

Primary tumors of the trachea are very rare. In adults, the majority of them are malignant. Schwannomas are exceedingly rare benign tumors in the tracheobronchial tree. We report a case of a 37-year-old man who was hospitalized for increasing dyspnea. He had been treated for bronchial asthma for the last 4 years with no benefit. The CT scan of the chest and bronchoscopy identified a tracheal mass that was prolapsed in the left stem bronchus. The patient did not remain free of disease after endoscopic laser resection. So, surgical resection was made. The tumor was excised at its base. A segment of the left stem bronchus was removed and primary anastomosis was performed. The histopathologic diagnosis was of a benign schwannoma without malignant elements. There was no recurrence during the follow-up period. This case demonstrates that intratracheal masses should be considered in patients with dyspnea or in patients with asthma refractory to conventional therapy.

Une association lésionnelle pulmonaire rare

INTRODUCTION Pulmonary amyloidoma or nodular amyloidosis is a localized form of amyloidosis, which can mimic a bronchopulmonary carcinoma. This form of amyloidosis may be associated to an infectious, a systemic disease or a lymphoma. OBSERVATION We describe the case of a 36-year-old patient whose past medical history was consistent for a diabetes mellitus and a hypothyroidism treated by medical treatment. The patient presented a Gougerot-Sjögren syndrome and was explored for non-specific respiratory symptoms. Physical examination was normal. Laboratory tests revealed a monoclonal pic of immunoglobulin. Radiologic findings showed bilateral pulmonary nodules associated to mediastinal lymph nodes. A pulmonary biopsy was performed. Histologic examination revealed a tumoral nodule containing an abundant eosinophilic material, which was acellular and surrounded by a dense lymphomatous infiltrate destroying the pulmonary parenchyma. Histochemical and immunohistochemical study revealed an association of a nodular pulmonary amyloidosis with a MAT pulmonary lymphoma complicating a Gougerot-Sjögren syndrome. CONCLUSION The association of MALT pulmonary lymphoma and localized amyloidosis is rarely observed in case of Gougerot-Sjögren syndrome. The pathogenesis of this association remains unknown and the management non-consensual because of the rarity of the cases reported. Whereas, it appears that localized amyloidosis associated to a MALT lymphoma seems to have a better prognosis than a disseminated amyloidosis.

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.

A decisional diagram for the management of thymomas based on a Tunisian multidisciplinary team's experience and a review of the literature

Background: Thymomas are rare tumors with a challenging management. After a review of the literature, we noticed the absence of a simplified decisional diagram. We aimed to present a scheme for the management of these tumors based on the experience of a Tunisian multi-disciplinary team and a review of the literature. Patients and Methods: We report a retrospective study 100 thymomas diagnosed between 1994 and 2011. We also performed an exhaustive PubMed research using the keywords: Thymoma, prognosis, management. We noticed, that the most famous studies were reported in the 1990s with most of them being a review of the literature. Results: The proposed decisional diagram is quite similar to the National Comprehensive Cancer Network (NCCN) guidelines with some particularities. These particularities consist in the recommendation of an initial surgical resection even in advances disease (stages IIIb or IVa) and the association of a radiation therapy in R1 or R2 stages I, II, and IIIa thymomas. Conclusion: This study highlights the necessity of further research performed in the field of thymoma in order to answer the unresolved question. This work seems to be necessary and helpful in daily practice of thoracic surgeons and clinicians.

Un pneumothorax révélateur d’une hydatidose pleurale primitive hétérotopique

INTRODUCTION Hydatid cyst is a parasitic disease that is endemic in many countries. Pneumothorax may be a presentation of this disease that presents urgent problems of diagnosis and treatment. CASE REPORT We report the case of a 23-year-old woman, amenorrheic for 22 weeks, who presented with chest pain and dyspnoea. Chest x-ray revealed a right-sided tension pneumothorax. A check x-ray after drainage showed a homogeneous opacity of water density occupying the lower 2/3 of the right hemithorax. Thoracic ultrasound suggested an uncomplicated hydatid cyst at the right base. Surgical exploration revealed a hydatid cyst 14cm in diameter in the pleural space, and a cavity in the right lower lobe with two bronchial fistulae. Treatment consisted of removal of the cyst intact, closure of the bronchial fistulae and capitonnage of the residual cavity. The postoperative course was uncomplicated. CONCLUSION Primary heterotopic pleural hydatid cyst is an exceptional cause of pneumothorax that should considered in countries where hydatid disease is endemic. Treatment is surgical following drainage of the pneumothorax.

Costal chondrosarcoma. Report of five cases.

We report five cases of coastal chondrosarcoma (CS) in four women and one man between 28 and 49 years of age. In four cases, the tumor had spread and infiltrated the adjacent structures (soft tissues, thoracic vertebrae, mediastinum, etc.). CS was diagnosed based either on wide surgical resection specimens in three patients, or on tumor biopsies in two cases. The CS was grade I in one patient, grade II in three cases, and grade III in one case. Wide surgical resection which was performed in three patients was associated in one case with adjuvant radiotherapy and chemotherapy. In the three cases, the disease natural history appeared favorable after a follow-up duration ranging from 1 month to 4 years. Two patients died, one after radiotherapy and chemotherapy done immediately after diagnosis as a result of the large size and invasive nature of the tumor. Coastal CS is characterized by a distinct potential for locoregional and distant metastasis. Diagnosis remains anatomopathological. Wide surgical resection is the only demonstrated curative treatment, even for high-grade CS.