1/9. Malignant peritoneal mesothelioma presenting with respiratory symptoms.Malignant peritoneal mesothelioma is a rare disease associated with mild, nonspecific abdominal symptoms and a wide spectrum of imaging findings, with thickened mesentery and peritoneum being the most common ones. A case of a malignant peritoneal mesothelioma presenting with manifestations of pulmonary disease is reported. Imaging evaluation revealed pleural, lung and pericardial involvement together with retroperitoneal lymphadenopathy, little ascites and extensive omental, but only subtle, mesenteric thickening.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
2/9. Five cases of the lymphangioma of the mediastinum in adult.Mediastinal lymphangioma is rare disease. Above all cavernous type of mediastinal lymphangioma is very rare. We report 5 cases of mediastinal lymphangioma including cavernous type. CT was performed in all and revealed that they were smoothly marginated and cystic. All were surgically resected and specimens were classified pathologically into cystic type (3 cases), cavernous type (1 case) and mixed type of the two (1 case). MRI was performed in the cavernous type and suggested that the mass was lymphangioma because of pathognomonic lesion. Despite preoperative diagnosis of mediastinal lymphangioma is difficult, MRI is able to useful examination. In the follow-ups there has been no recurrence in our series.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
3/9. Nodular amyloidosis: case report and literature review.BACKGROUND: amyloidosis refers to a group of depositional diseases that are classified into two main types: systemic and localized. Large nodules of localized cutaneous amyloidosis of the nasal ala and surrounding skin are rare and the treatment is often unsatisfactory. OBJECTIVE: We report a case of rapidly enlarging, localized, nodular cutaneous amyloidosis of the nose and the surrounding skin with a brief review of the current literature regarding treatment of this rare disease. CONCLUSION: Nodular amyloidosis can be treated successfully with cold steel excision in combination with carbon dioxide laser. Close followup of these patients is warranted, as nodular amyloidosis may be the precursor to systemic amyloidosis.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
4/9. Exacerbation of pulmonary lymphangioleiomyomatosis by exogenous oestrogen used for infertility treatment.Pulmonary lymphangioleiomyomatosis (LAM) is a rare disease that affects women in the reproductive years. It is occasionally associated with tuberous sclerosis, especially in the incomplete form. As it is likely that oestrogen plays a central role in disease progression, exogenous oestrogen will cause a deterioration in LAM. However, the early stage of this disease is easy to miss unless the physician is a specialist. Although there have been some reports in menopausal women given exogenous oestrogen for osteoporosis, this is the first report of pulmonary LAM caused by exogenous oestrogen used for the treatment of infertility.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
5/9. pulmonary alveolar proteinosis.A 23-year-old man was admitted to the hospital because of dyspnea. Chest X-ray showed reticulo-nodular opacities. The crazy paving appearance on high-resolution CT was highly suggestive of pulmonary alveolar proteinosis. Histologic examination confirmed the diagnosis. pulmonary alveolar proteinosis is a rare disease but an important diagnosis to make as treatment with pulmonary lavage is curative in a large proportion of patients.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
6/9. Laryngotracheal involvement of relapsing polychondritis in a Korean girl.We describe a young girl presenting with acute dyspnea because of laryngotracheal involvement of relapsing polychondritis (RP). RP, a multisystemic disorder of unknown etiology, is a very rare disease in children. It is characterized by inflammation and destruction of the cartilaginous structure of many organs, including the respiratory tract. Early respiratory tract involvement in younger patients is the greatest threat to life, and aggressive therapy, including tracheostomy and intravenous high-dose steroids, is advocated.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
7/9. A case of idiopathic tracheal stenosis.Idiopathic tracheal stenosis (ITS) is an extremely rare disease. We report the case of a 32-year-old woman with ITS. She had no history of previous surgery, endotracheal intubation, neck trauma, granulomatous disease, or any other severe respiratory tract infections. She presented with progressive dyspnea on effort and had been treated for bronchial asthma for 3 years. Chest radiography and laboratory examinations revealed no abnormalities. bronchoscopy demonstrated almost circumferential tracheal stenosis extending for 10 mm from about 20 mm below the vocal cords. Luminal diameter was about 4 mm at the narrowest. Bronchoscopic biopsy revealed increased fibrous tissue and chronic inflammatory cell infiltration (nonspecific inflammatory tissue). These finding are compatible with idiopathic stenosis as reported by Grillo et al. After tracheostomy, the patient was treated by tracheal segmental resection (two rings) with end-to-end anastomosis of the cartilaginous trachea. Symptoms of tracheal stenosis were completely relieved and no recurrence has been observed as of 3 years postoperatively.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
8/9. Diffuse pulmonary lymphangiomatosis: imaging findings.Diffuse pulmonary lymphangiomatosis is a rare pulmonary disorder affecting the lymphatic channels from the mediastinum to the pleura. The disease usually occurs in children and young adults and frequently ends with death due to progressive course. Imaging findings of the disease are based on lymphatic involvement which appear as mediastinal soft tissue infiltration and thickening of pulmonary peribroncovascular bundles and interlobular septae. In this report, spiral and high-resolution computed tomography, and ultrasonography findings of severe form of this rare disease are presented. Furthermore, some lymphatic disorders, which are called with similar name but different appearances on imaging, are discussed.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
9/9. Relapsing polychondritis with severe aortic insufficiency.Relapsing polychondritis (RP) is a rare disease characterized by auricular, nasal and respiratory tract chondritis, ocular inflammation, inflammatory polyarthritis and cardiovascular abnormalities. Here we describe a patient with a five-year history of mild nasal and auricular chondritis, which suddenly developed into a severe aortic insufficiency with rest dyspnea. The pathogenesis and the management of this rare connective tissue disease are discussed.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |