[Benign mature teratomas of the mediastinum]. / Tératomes matures bénins du médiastin.

INTRODUCTION: Mature teratomas of the mediastinum are rare. However, they represent the most common mediastinal germ cell tumours. The aim of this study is to describe their clinical and pathological characteristics. PATIENTS AND METHODS: Fourteen cases of mediastinal mature teratoma, diagnosed between January 1992 and December 2006, were reviewed retrospectively, noting the clinical, radiological, surgical, and pathological findings. RESULTS: The patient population consisted of 10 females and 4 males with mean age of 29 years (5-56 years). Chest pain was the main symptom. Imaging features comprised a heterogeneous anterior mediastinal mass containing soft-tissue, fluid, fat, or calcium attenuation, or any combination of the four. Macroscopically all the tumours were cystic, or predominantly cystic, containing mostly grumous material. The mean size was 9 cm (5-12 cm). Histological examination constantly revealed skin with or without appendages. Other components identified were bronchial mucosa (12 cases), fat (12 cases), muscle (10 cases), cartilage (8 cases), bone (7 cases), gastrointestinal mucosa (7 cases), pancreas (5 cases), urothelial epithelium (3 cases), nervous and prostatic tissues (one case each). All the teratomas were mature, and 7 of them were intrathymic. CONCLUSION: Every form of teratoma occurs in the mediastinum (mature, immature and with malignant transformation) but, in our study, they were exclusively mature. The histology is essentially similar to that of teratomas of gonads. However, pancreatic tissue occurs frequently in mediastinum (54% in literature, 25% in our study) but not in the gonads. On the other hand, thyroid follicles have not yet been seen in mediastinal teratomas.

[Fibrous dysplasia of the rib. Ten case reports]. / Dysplasie fibreuse costale. A propos de dix cas.

PURPOSE OF THE STUDY: Fibrous dyplasia is a rare sporadic disease accounting for 0.8% of primary bone tumors. This benign pseudotumor results from proliferation of fibrous tissue in bone and the production of immature bone tissue without an osteoblastic crown. The disease can involve one or more bones, ribs are rarely involved. We report a series of 10 cases of costal fibrous dysplasia. CASE REPORTS: This series of 10 cases was collected over a period of 10 years (1996-2005). There were five men and five women, mean age 38.4 years, range 27-52 years. One rib was involved in eight patients, two ribs in two. Pain was the most frequent symptom. Plain X-rays showed signs suggestive of fibrous dysplasia. To confirm the diagnosis, rib resection was performed in all ten patients. Pathology examination provided the diagnosis. The postoperative period was uneventful in all patients and all are recurrence free at mean 50 months follow-up. DISCUSSION: Management of fibrous dysplasia can be simple surveillance in the majority of patients. Nevertheless, in the event of a single focus, particularly in a rib, fibrous dysplasia can raise a difficult problem of differential diagnosis with malignant tumors. Surgical resection is therefore required in selected cases.

[Congenital cystic adenomatoid malformation of the lung. Report of 3 cases with late presentation]. / Malformation adénomatoïde kystique congénitale du poumon: à propos de trois cas de révélation tardive.

Congenital cystic adenomatoid malformation (CCAM) of the lung is a rare congenital developmental abnormality, representing about 25% of all congenital lung lesions. In many cases, respiratory distress occurs during the neonatal period, and in about 80- 85% of patients, CCAM is diagnosed before the age of two years due to respiratory infection. It is very rare that presentation is delayed until adulthood. We report three cases of CCAM presenting in adults. The diagnosis was based on clinical and radiological findings in one case and two patients were not diagnosed until surgery. The lesion was present in the right lung in two and in left lung in one patient. All patients underwent surgical resection. The result of histopathological examination confirmed CCAM Stocker type 1, without malignancy. The post operative follow up showed an excellent recovery. Clinicians and pathologists need to be aware of the fact that CCAM can be present for the first time in adolescents or in adults. The clinical diagnosis is suggested by radiographic findings and is confirmed at pathology as surgery is generally indicated.

[Desmoid fibromatosis of the posterior mediastinum]. / Fibromatose de type desmoïde du médiastin postérieur.

Fibromatosis are uncommon connective tissue tumours arising from musculo-aponeurotic tissue and characterised by spindle cell fibroblastic and myofibroblastic proliferation. The exact aetiology is unknown but several factors are considered to be positively correlated with their development and growth (genetic and hormonal factors and trauma). Although they are considered histologically benign they behave aggressively locally and relapse repeatedly after surgical excision. Mediastinal localisation is very rare. We describe a case of de novo fibromatosis of the posterior mediastinum in a 61 year old man with no history of thoracotomy or trauma. Although mediastinal fibromatosis is very uncommon physicians should be aware of this disease in order to ensure appropriate surgical treatment.

[Hydatid disease of the first rib treated surgically by the Roos approach]. / Hydatidose de la première côte opérée par voie de Roos.

Hydatid disease is a parasitic infection due to Echinococcus granulosus. Since involvement of the ribs and in particular the first rib is rare, diagnosis and treatment in these locations can pose special problems. The purpose of this report is to describe the case of a 27-year-old man in whom an apical left opacity was discovered by chance. Clinical examination was normal. Thoracic ultrasound demonstrated multilocular cyst in the left apical region of the thorax. Thoracic computed tomography (CT) demonstrated a hydatid cyst originating from the first rib. The patient was operated using the technique known as the Roos approach. The procedure consisted of cystectomy with resection of the first rib. The multiloculated appearance of the lesion and erosion at the first rib was consistent with hydatid cyst. The Roos approach is the technique of choice for the treatment of the first rib.

[Costal chondromyxoid fibroma]. / Fibrome chondromyxoïde costal.

Chondromyxoid fibroma is a rare benign bone tumor observed in less than 1% of all bone tumors. Pathological diagnosis remains difficult. All bone sites may be involved with a predominance of the metaphysis of long tubular bones, particularly the proximal tibia. The tumor has been rarely reported in ribs. We present of case of chondromyxoid fibroma observed in this unusual location in a 31-year-old woman. Routine chest x-ray and computed tomography revealed an expansive destructive mass with a lobulated contour which arose in the fourth left rib. Local resection was performed and the histological examination showed benign chondromyxoid fibroma. The clinical outcome was favorable without recurrence after 15 months follow-up.

[Computed tomography of complicated pulmonary hydatid cyst by rupture in the bronchi]. / Kyste hydatique du poumon ouvert dans les bronches: apport de la tomodensitometrie.

PURPOSE: To describe the CT findings of ruptured pulmonary hydatid cyst (HC) and to establish a progressive CT staging. PATIENTS AND METHODS: CT scans of 41 patients with pulmonary hydatid cyst complicated by rupture in the bronchi were retrospectively reviewed. We also reviewed the findings in the literature on the rupture of pulmonary hydatid cysts and the Lewall and McCorkell classification and proposed a more detailed staging related to the progression of HC. The Lewall and McCorkell communicating rupture is manifested by a tear of the endocyst with a discharge of the cyst's contents via the bronchioles that were incorporated in the pericyst: -Stage I: signet ring sign; -State II: crescent sign and inverse crescent sign; -Stage III: air bubble sign and honeycomb; Stage IV: air-water level, double arch sin, water lily sign, serpiginous aspect, regular air-water level; -Stage V: dry cyst sign, ball of wool aspect, small bell image, pseudotumoral aspect; -Stage VI: sequela image, residual cavity, and cicatricial image. RESULTS: Seventy-three complicated pulmonary hydatid cysts were included in the analysis and were distributed as follows: stage I (16%), stage II (12.7%), stage III (42.8%), stage IV, double arch sign (2.7%), water lily sign (10%), serpiginous aspect (8.2%), regular air-water level (5.4%), stage V, ball of wool aspect (6.8%), small bell image (15%), pseudotumoral aspect (4%), stage VI, residual cavity (4%) and cicatricial image (2.7%). CONCLUSION: Our staging offers more details than the Lewall and McCorkell general classification. Moreover, this staging takes into consideration both the natural evolution of the HC and the particularities of the pulmonary location.

[Management of postoperative chylothorax]. / Prise en charge des chylothorax post opératoires.

INTRODUCTION: A chylothorax can occur following any intrathoracic procedure. It is generally straightforward to make the diagnosis but optimal management can be problematic. METHODS: Between 1995 and 2002, three women and one man aged from 13 to 58 years were treated for chylothorax after thoracic surgery. Their initial illnesses were a right pulmonary hydatid cyst associated with hepatic disease, a tumour of the posterior mediastinum, an oesophageal carcinoma and metastases in the left lung. RESULTS: These patients had: a pulmonary and hepatic cystectomies, a resection of the mediastinal tumor, an Akyama oesophagectomy and a resection of four left pulmonary metastases. Chylothorax became apparent post operatively between the 1st and the 4th day. All patients were treated with a medium-chain triglyceride diet. Two patients were re-explored with ligation of lymphatic vessels. One woman who did not have further surgery was treated with etilefrine. In the patient who had had an oesophagectomy, chylothorax persisted after re-operation. He was successfully treated by talc pleurodesis via a chest drain, which prevented further recurrence. CONCLUSIONS: In the management of postoperative chylothorax, medical treatment must be started early but surgery should not be delayed as operative risk is increased by the development of malnutrition and immune deficiency.

[Hydatid cyst of the mediastinum: 14 case reports]. / Kyste hydatique primitif du médiastin: 14 cas.

PURPOSE: The objective of this study was to describe the different radiological features of the hydatid cyst of the mediastinum. MATERIALS AND METHODS: We conducted a retrospective study on 14 patients (seven women, seven men), diagnosed with mediastinal hydatid cyst, aged 13-67 years (mean, 48 years) over a period of 16 years in our hospital where 71 cases of hydatid disease are annually diagnosed. The patients were explored by chest radiography (n=14), chest ultrasonography (n=10), abdomen ultrasonography (n=14), computed tomography (CT scan) (n=13), and magnetic resonance imaging (MRI) (n=8). RESULTS: The chest x-ray showed an opacity of the anterior (n=11), middle (n=1), and posterior (n=1) mediastinum. The thoracic ultrasound confirmed the liquid nature of the mass with a multivesicular aspect (n=9). CT and thoracic MRI made it possible to precisely locate the mediastinal compartment involved and study the relationship to adjacent structures. A purely mediastinal location was found in only six cases. In the remaining cases, it was associated with hepatic (n=7) and pleural (n=1) locations. CONCLUSION: Hydatid cysts of the mediastinum are very uncommon. Diagnosis can be made by chest sonography. CT scan is the main tool for diagnosis and the study of possible extension. Our study showed a more frequent location to the anterior mediastinum.

[Clear cell tumor of the lung. A case report with review of the literature]. / La tumeur à cellules claires du poumon. A propos d'un cas avec revue de la littérature.

Clear-cell tumor of the lung is a rare entity of unknown etiology and histogenesis. This neoplasm typically presents as an asymptomatic, peripheral, sharply rounded mass in the lung, and histologically composed of large cells with a clear cytoplasm rich in glycogen, blended with an abundant network of sinusoid-type vessels. Immunohistochemical and ultrastructural procedures lead to diagnosis. We describe a primary pulmonary clear cell "sugar" tumor observed in a 28-year-old woman, and give a review of the literature. Clinical aspects, differential diagnosis, therapy and histogenetic aspects are discussed.

[Clinical aspects and diagnosis of pleural fibromas]. / Aspects cliniques et approche diagnostique des fibromes pleuraux.

Pleural fibromas are rare malignant or benign tumors requiring pathology study for certain diagnosis. From January 1985 to January 2001, 7 patients underwent surgery in our unit for pleural fibroma: 4 females and 3 males, mean age 60 years. The inaugural symptoms were chest pain (3 patients), dyspnea (2 patients), joint pain in a patient with Pierre-Marie pneumonic hypertrophic osteo-arthropathy, and acute hypoglycemia. Radiological investigations were decisive in orienting the diagnosis (chest X-ray, ultrasound, computed tomography and MRI). Surgical resection and pathological study of the surgical specimen is required to confirm the diagnosis. Patients should be carefully followed due to the risk of malignant recurrence.

[Huge benign lung tumor in a female smoker]. / Tumeur de 5 cm chez une femme fumeuse.

UNLABELLED: Pulmonary sclerosing hemangioma is a rare, slow-growing, benign tumor. Its potential for progression and its histiogenesis remains controversial. CASE REPORT: A routine chest X-ray revealed a right abdominal mass in 41-year-old woman. Search for a cause was negative. The patient underwent posterolateral thoracotomy for tumorectomy. Intraoperative pathology analysis revealed the benign nature of the tumor. No complication was observed postoperatively. The final pathological conclusion was sclerosing hemangioma of the lung. Pulmonary sclerosing hemangioma is a parenchymal tumor of the lung. The latest immunohistochemical studies of this lesion suggest a pneumocyte origin. Prognosis is good, but extension to lymph nodes may occur. Surgery is always required for cure, and must be associated with lymph node dissection for large tumors.