Background Neurogenic tumors of the mediastinum are rare tumors located mainly in the posterior mediastinum. The aim of this study was to report a single-institution experience of these tumors. Methods This study was conducted between 2005 and 2017. Bivariate analysis was performed to assess correlations between the cellularity, mitotic index, presence of a capsule, and potential for malignancy of peripheral nerve sheath tumors. Results There were 86 patients enrolled in the study. The mean age was 42.51 years. Nineteen patients were asymptomatic and their tumors were discovered incidentally. Microscopic diagnosis confirmed a schwannoma in 45 cases, neurofibroma in 12, malignant peripheral nerve sheath tumor in 9, ganglioneuroma in 14, ganglioneuroblastoma in 4, and neuroblastoma in 2. Bivariate analysis showed a significant correlation between the absence of a capsule, the degree of atypia, a high mitotic index, and high cellularity with the potential for malignancy ( p < 0.05). Surgical resection was the main treatment modality in 84 cases. The mean survival was estimated to be 51.309 months. The log-rank test showed a significant difference in survival according to histologic subtype and between benign and malignant tumors ( p < 0.0001). Conclusion Even if radiologic means are helpful in suggesting the diagnosis, a positive diagnosis of neurogenic tumors is based on microscopic features. High mitotic activity and cellularity, and severe cytonuclear atypia are the features most suggestive of malignancy.
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.
BackgroundIdiopathic 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.AimWe target to evaluate the connection between fibrosis and vascular remodeling in usual interstitial pneumonia.Material and methods26 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).ResultsWe 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.ConclusionMany 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.
Objectives:
Primary cardiac tumors are rare and account for 0.001 to 0.03% of cardiac
lesions. The authors aim to describe the clinical, the microscopic and the therapeutic characteristics
of these tumors through a 13-year-experience in order to highlight the diagnostic challenges faced.
Methods:
We report 10 primary cardiac tumors diagnosed in the Departments of Pathology and
Thoracic Surgery of the same hospital through a 13-year-period.
Results:
Our study was conducted on 7 women and 3 men. The mean age of the patients was 54.22
years (average, 12 to 79 years). Dyspnea represented the most frequent symptom. Physical
examination was normal in all patients. Trans-thoracic ultra-sound examination was performed in all
patients. Cardiac MRI allowed localizing the tumors in 2 patients. They were located into the left
auricle (6 cases), the right auricle (1 case) and the pericardium (3 cases). The microscopic
examination was concluded to myxoma (7 cases), haemangioma (2 cases) and
hemangioendothelioma (1 case). Surgical resection was possible in 9 patients. It was impossible in
the case of hemangioendothelioma because of the adherence. One death was recorded secondary to
postoperative arrhythmia. The other patients presented no complications after a follow-up period
ranging from 2 months to 5 years.
Conclusion:
Cardiac tumors are challenging in their diagnosis and management. A positive
diagnosis is based on microscopic examination. Surgical treatment plays a key role and is possible in
the majority of benign tumors. The prognosis of malignant tumors remains poor even if a complete
surgical resection is possible.
Paragangliomas of the mediastinum are rare neoplasms, representing less than 0.3% of mediastinal tumours and less than 2% of all paragangliomas. To date, no definitive morphologic criteria exist that correlate with the clinical outcome of these tumours. This report describes three patients with peculiar primary paragangliomas of the posterior mediastinum: gangliocytic, dumbbell and functional paraganglioma. The clinicopathological characteristics of these patients are presented and the literature reviewed.
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