The major prognostic factors of thymomas: about a Tunisian study of 100 cases.

AIM: Thymomas are characterised by their rarity, histologic variability and peculiar patterns of recurrence. Herein, we present the experience of a single institution and aim to highlight the major prognostic factors of these tumours. MATERIALS AND METHODS: We present a retrospective study on 100 thymomas diagnosed between 1994 and 2011. Statistical analyses were performed using version 18.0 SPSS. The Kaplan Meier method was used to estimate survival, and survival curves were compared using the Log-Rank test. A p < 0.05 was considered statistically significant. RESULTS: 50 men and 50 women underwent surgical resection for thymoma. Radiologic findings highlighted a diagnosis of thymoma in 51% of cases. The thymomas were classified as stage I in 25 cases, stage II in 47 cases, stage III in 25 cases and stage IV in 3 cases. According to the WHO classification, tumours were classified as type A in 14 cases, type AB in 24 cases, type B1 in 17 cases, type B2 in 20 cases, type B3 in 8 cases, B1/B2 in 8 cases and B2/B3 in 9 cases. The mean survival of patients was 136 months. Age, sex, tumour size, WHO classification and Masaoka stage were evaluated as prognostic factors. Univariate analysis showed that the major prognostic factors were WHO classification (p = 0.019) and Masaoka Stage (p = 0.0001). CONCLUSION: Our results place emphasis on the prognostic value of WHO classification and Masaoka stage in thymomas; in addition, the necessity of improving reproducibility of microscopic classification to avoid discrepancies among prognostic groups is highlighted.

[Bronchopulmonary carcinoid tumors: Tunisian experience with 115 surgically treated cases]. / 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.

[Pulmonary zygomycosis in a diabetic child complicated with thrombus of the left atrial auricle]. / Mucormycose pulmonaire chez une enfant diabétique, compliquée d'un thrombus intra-auriculaire gauche.

Mucormycosis is a rare but fatal, opportunistic fungal infection caused by fungi of the order of mucorales in the class of Zygomycetes. Isolated pulmonary mucormycosis is rare and occurs principally in particular conditions, especially in patients with uncontrolled diabetes. The fungi invades the blood vessels and causes distal ischemic necrosis. We report a case in a 13-year-old girl with diabetes mellitus who developed pulmonary zygomycosis complicated with thrombus of the left atrial auricle. The diagnosis of mucormycosis was confirmed by histologic examination of endobronchial biopsies. Combined treatment with systemic amphotericin B and surgery resulted in a favorable outcome. This difficult diagnosis must be raised in diabetic patients with unresponsive lung infections in order to apply early aggressive therapy. Successful management continues to be early diagnosis, followed by systemic antifungal therapy and surgical resection combined with control of the underlying disease.

[Tracheal tumor treated as asthma]. / 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.

Fibroadenoma in an ectopic vulvar breast gland: a common neoplasm in an uncommon site.

Ectopic breast tissue is defined as glands located outside of the breast. It can be found anywhere along the milk line extending from the axilla to the groin, and can occur in the vulva. Ectopic breast tissue should be excised because it may develop benign or malignant pathologic processes. Less than 40 cases of fibroadenoma in the vulva have been reported in the literature. We report a case of a 37-year-old woman presenting a solitary vulvar mass. The mass was excised completely, and histology demonstrated an ectopic breast fibroadenoma. This is one of the few reports on the benign pathologies of vulvar mammary glands.

[Case report of pulmonary biphasic tumor with unusual follow-up]. / À propos d'une tumeur biphasique du poumon d'évolution inhabituelle.

BACKGROUND: Pulmonary blastoma, a rare primary lung malignancy is subdivided in 3 categories: well-differentiated fetal adenocarcinoma (WDFA), classic biphasic pulmonary blastoma (CBPB) and pleuropulmonary blastoma (PPB). Classic pulmonary blastoma is composed of a mixture of immature epithelial and mesenchymal tissue resembling fetal lung tissue. CASE REPORT: We described a case of a 48-year-old male, cigarette smoker, who presented with left thoracic pain and hemoptysis for 2 months. Chest radiography showed a well-delimited, homogeneous 4cm mass in the left lung periphery. Bronchoscopic examination revealed left endobronchial bleeding. Computed tomography of the chest revealed a tumor shadow measuring 7cm in the left upper lobe and bilateral nodules with no lymphadenopathy. A systemic evaluation demonstrated no metastatic lesion. Patient underwent a left upper lobectomy. The diagnosis of CBPB was affirmed on anatomopathology of the tumor resection. Immunohistochemical studies showed that tumor cells were positive for vimentin, desmin, actin, Pan Cytokeratin and TTF-1. The final diagnosis was BPB classified as pathological T3N0M0 and no adjuvant treatment was associated. The patient showed good objective response with no evidence of disease recurrence still in 5 years surgery resection. CONCLUSION: This case reiterates the importance of pathomorphological or immunohistochemical features in diagnosis of BPB.

Primary mediastinal angiosarcoma: a rare observation in a patient with 8-year-survival.

BACKGROUND: Vascular tumours of the mediastinum are rare, accounting for 1-2% of all mediastinal tumours in this location. Angiosarcomas are most often encountered as sporadic lesions, typically in the scalp or face of elderly patients. However, they can occur in any anatomic site. Mediastinal angiosarcomas (MA) are very rare with less than 50 cases reported. CASE REPORT: The authors describe the case of a 38-year-old woman whose past medical history was consistent for a MA that was diagnosed in 2003. This tumour was treated by complete surgical resection followed by radiation therapy and chemotherapy. Diagnosis was based on histologic examination. In 2011, the patient presented a pleural localisation of the angiosarcoma and died one month after admission, 8 years after diagnosis of the MA. CONCLUSION: MA is a very rare tumour causing a diagnostic dilemma. Clinical and radiologic findings are non-specific, and final diagnosis is based on histologic examination. The case described is unusual considering the long period of survival, which may be explained by the treatment modalities associating complete surgical resection, chemotherapy and radiation therapy.

Diagnosis and clinical course of cardiac myxoma.

Cardiac myxomas are the most common benign tumours of the heart. In spite of their benign nature, these tumours may induce metastasis or recurrences. Their diagnosis is challenging because of the lack of specific signs, and positive diagnosis is based on microscopic findings. We report a case series of 6 patients documented by radiologic and microscopic findings. In addition, one case was unique due to its location in the right atrium. Tumours were detected by trans-oesophageal ultra-sound examination in all cases. They were located in the left atrium in five cases and in the right side in one case. All patients underwent a successful surgical excision with en-bloc removal of the tumour. The outcome was fatal in one patient because of atrial arrhythmia.

[A rare pulmonary lesion association]. / 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.

[A case of costal haemangioma]. / Un cas d'hémangiome costal.

UNLABELLED: Costal primary tumors are rare and dominated by malignant tumors. Haemangioma of the bone represents only 1% of bone tumors. Costal localization accounts only for 1% of the cases and only about fifty cases have been reported in the literature. AIM: The authors aim to describe a rare costal tumor, its histological features and the main differential diagnoses. OBSERVATION: The authors describe the case of a 46-year-old woman who presented with chest pain. Radiological findings did not permit a malignant tumor to be ruled out and the treatment consisted of a resection of the posterior arch of the rib. Microscopic examination concluded that the patient had a costal haemangioma and the patient didn't present any recurrence after a six-year follow-up. CONCLUSION: The costal haemangioma is a very rare tumor with a debated etiology. Some radiological features are specific such as the "soap bubble" or "honeycomb" aspect. However, the basis for diagnosis remains microscopic examination. These tumors have a good prognosis and no cases of recurrence have been reported following complete resection.

[Clinical characteristics of resected bronchial hamartoma. Study of seven cases]. / Particularités anatomocliniques des hamartochondromes endobronchiques opérés. Étude de sept cas.

INTRODUCTION: Endobronchial hamartoma is a benign tumour derived from peribronchial mesenchymal tissue. It is a form of intrapulmonary hamartoma and can cause irreversible pulmonary destruction due to bronchial obstruction. Early diagnosis and treatment is very important and endoscopic treatment is usually the first choice. In cases in which prolonged bronchial obstruction has produced irreversible lung destruction surgical, resection of the tumour is necessary. The aim of this study is to describe the clinicopathological characteristics of resected endobronchial hamartoma. PATIENTS AND METHODS: Seven cases of endobronchial hamartoma, diagnosed in our institution between January 1995 and December 2009, were reviewed retrospectively. RESULTS: The patient population consisted of four males and three females with mean age of 53.7 years (41-68 years). The most frequent clinical presentation was obstructive pneumonia. Endoscopy and imaging showed an endobronchial mass in most cases. Surgical treatment consisted of atypical resection in one case, lobectomy in five cases and pneumonectomy in one case. Tumour size ranged between 0.5 and 3.5 cm. The diagnosis was confirmed in all cases by histological examination. The outcome was favorable in all cases. CONCLUSION: Endoscopic techniques are effective for the diagnosis and treatment of endobronchial hamartochondroma. Treatment should be prompt to prevent irreversible lung damage due to chronic obstruction and suppuration, in which case, pulmonary resection may be necessary.

[Hypertrophic osteoarthropathy associated with pulmonary tuberculosis]. / Ostéoarthropathie hypertrophique pneumique associée à la tuberculose pulmonaire.

Hypertrophic osteoarthropathy is a syndrome frequently described in intrathoracic diseases, especially malignant ones. The association with lung tuberculosis is rarely reported. The authors describe the case of a 35-year-old patient, a smoker, hospitalised for lung cavitation associated with hypertrophic osteoarthropathy. The assessment of the aetiology was negative and the patient underwent lung surgery. The histopathological examination concluded as to chronic pulmonary tuberculosis. This report aims at alerting physicians about the possibility of hypertrophic osteoarthropathy in non malignant diseases, especially pulmonary tuberculosis which is still endemic in our country.

[Primary mediastinal sarcomas]. / 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.

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.

Cutaneous metastasis of lung carcinoma: a retrospective study of 12 cases.

BACKGROUND: The skin is a rare site of metastasis of the internal malignancies. On the contrary, lung cancer is a common primary malignancy of skin metastasis. The latter is either synchronous with the primary lung tumour or can reveal it. OBJECTIVE: To analyse the clinical findings and the outcome of cutaneous metastasis in patients with primary lung cancer. METHODS: Retrospective study including 12 cases of cutaneous metastasis of lung cancer diagnosed in our institution during 6years. RESULTS: Our patients included three women and nine men with a median age of 59.83years. The cutaneous metastases were unique in seven cases. They revealed the primary tumour in two cases, were discovered synchronously with the primary tumour in six cases and after the primary tumour in four cases. They appeared in patients presenting multiple metastases in 10 cases. Clinical appearance consisted on a firm nodule, sometimes ulcerated with a diameter ranging from 0.5 to 5.5cm. The tumour was sited in the thorax, the back, the abdomen; or the upper or lower limbs. Pathological examination of bronchial biopsy revealed an adenocarcinoma in eight cases, a squamous cell carcinoma in two cases and a small cell carcinoma in two cases. In spite of lung cancer therapies, the patients with metastasis to other organs with a median follow-up time of 4months died. CONCLUSION: Cutaneous metastases of lung cancer are of poor prognosis. They are most of the time associated with an advanced neoplastic process with metastasis to other organs. Median survival after skin involvement does not exceed months.

[Solitary malignant fibrous tumors of the pleura]. / Les tumeurs fibreuses solitaires malignes de la plèvre.

Solitary fibrous tumours of the pleura are usually benign. However, malignancy is observed in 13% of the cases. The authors report two cases of 27 and 69 year-old patients, presenting chest pain and dyspnoea. The roentgenograms and computed tomography scanning revealed a pleural tumour in both cases. The patients underwent complete resections of these tumours. The immunohistochemical study confirmed the diagnosis of malignant solitary pleural fibroma. The patients succumbed to their disease secondary to local recurrences. Histopathology is very helpful in the diagnosis of malignant pleural fibroma. Surgery and long-term follow-up are mandatory.

Multiple myeloma presenting with multiple thoracic manifestations.

Multiple myeloma is a malignant proliferation of plasma cells that affects mainly bone marrow but may also involve other organs as well. We report thoracic involvement in the form of left-sided pleural effusion, osseous lesions, bronchial infiltration, and mediastinal lymphadenopathy in a 61-year-old woman, non-smoker presented with chest pain, dyspnoea, cough and deterioration in general health over the preceding seven months. Immunoelectrophoresis and immunofixation showed raised kappa-light chain immunoglobulin G (IgG) in serum and pleural fluid. Bronchial and pleural biopsies documented myelomatous infiltration and bone marrow aspirate revealed extensive plasma cell infiltration. At eight months, following the fourth cycle of melphalan, endoxan and prednisone based chemotherapy, the patient died.

Benign metastasizing leiomyoma: report of 2 cases and review of the literature.

Benign metastasizing leiomyoma (BML) is a benign spindle cell lesion affecting women who have undergone hysterectomy for uterine leiomyomas in young adulthood, and subsequently present pulmonary metastases during the peri-menopausal period. We present 2 cases of BML in women with prior medical history of hysterectomy for multiple myomas. Both patients presented pulmonary metastases at 17 and 12 years after hysterectomy. The pulmonary nodules were totally excised in the both cases, and neither patient experienced complications or recurrences after 1 and 2 years of follow up, respectively. BML is a rare benign entity with a debated pathogenesis. We have developed different hypotheses about its pathogenesis, mechanisms of spread, histological characteristics and commonly employed treatment modalities.

[Primary thymic carcinomas. Three cases and a review of the literature]. / Carcinomes thymiques primitifs : à propos de trois cas avec revue de la littérature.

Thymic carcinoma is a very rare malignancy. In 1999, a World Health Organization committee published histologic criteria for distinct thymoma entities (labelled as type A, AB, B1, B2, B3 thymomas) and for the heterogeneous group of thymic carcinomas, collectively called type C tumour. Thymic carcinoma differs from thymoma in that it displays cytologically malignant features, extensive local invasion, and a substantial potential for metastasis. It constitutes a heterogeneous group of tumours that display different biological behaviours and prognoses. The majority of thymic carcinomas are either squamous carcinomas or lymphoepithelioma-like carcinomas. This study included three male patients aged 20, 46 and 19years respectively with histologically proven thymic carcinoma diagnosed at the author's institution. All of the patients presented a large mass of the anterior mediastinum. Histological examination of the different tumours revealed three distinct variants of thymic carcinoma, namely: epidermoid carcinoma, clear cell carcinoma and lymphoepithelioma-like carcinoma.