1/5. Cytologic finding of chyloascites in lymphangioleiomyomatosis. A case report.BACKGROUND: lymphangioleiomyomatosis is a rare disease, histologically characterized by an abnormal proliferation of smooth muscle around the lymphatics. lung is the most common site of involvement, and patients usually present with dyspnea, chest pain, and cough. Chylous pleural effusion and ascites occasionally appear during the course of the disease. There are only a few reports on the cytologic findings in this disease. To our knowledge, the cytologic findings of chylous pleural effusion and chyloascites have not been reported before. CASE: A 23-year-old female presented with chylothorax, chyloascites and a retroperitoneal mass. Cytologic examination of chylous pleural effusion and chyloascites revealed numerous cohesive and thick clusters of cells with a high nuclear/cytoplasmic ratio, oval nuclei and slightly increased chromatin content. mitosis and necrosis were not observed. Exploratory laparotomy and transbronchial lung biopsy were performed, and the histologic diagnosis was lymphangioleiomyomatosis involving the retroperitoneal lymph nodes, uterine fundus and lungs. immunohistochemistry showed that the characteristic clusters in chylous fluids were positive for alpha-smooth muscle actin. CONCLUSION: A diagnosis of lymphangioleiomyomatosis is possible from cytologic findings of effusions with the aid of clinical findings.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
2/5. Human herpes virus 8-negative primary effusion lymphoma (PEL) in a patient after repeated chylous ascites and chylothorax.We describe a case of malignant lymphoma which presented in the body cavities without identifiable tumor masses. Malignant lymphoma cells showed strong atypia with prominent nuclei and basophilic cytoplasm containing vacuoles. The chromosomes showed diploidy and complex abnormalities including translocations and deletions. We diagnosed this patient with primary effusion lymphoma (PEL), even though she tested negative for human herpes virus-8 (HHV-8) which has been suggested to be causally related to PEL. Interestingly, the patient also showed complicated protein-losing enteropathy, and PEL occurred after repeated chylous ascites and chylothorax. The possible pathogenesis of this rare disease is discussed here.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
3/5. Thoracoscopic treatment for primary chylopericardium: report of a case.We used video-assisted thoracoscopic surgery to successfully treat primary chylopericardium, a very rare disease. A 27-year-old woman was admitted to our hospital for investigation of cardiomegaly and dyspnea on exertion. echocardiography showed severe peri-cardial effusion. A milky fluid was extracted by pericardiocentesis and diagnosed as being chylous. A computed tomography (CT) scan taken after lymphangiography showed the leakage of contrast solution into the pericardial cavity. Initially, conservative therapy was used to treat the condition, but this proved ineffective and we decided to perform video-assisted thoracoscopic surgery. The thoracic duct was ligated and excised, and partial pericardiectomy was carried out under thoracoscopy. The patient has been in good health with no sign of recurrence since her operation. Thus, video-assisted thoracoscopic thoracic duct ligation and partial pericardiectomy can be effectively used to treat primary chylopericardium.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
4/5. Very unusual case of post-traumatic chylothorax.chylothorax is a rare disease caused by both traumatic and nontraumatic events. chylothorax can cause cardiopulmonary abnormalities and significant nutritional, metabolic, and immunologic consequences. We present an exceptional case of chylothorax due to penetrating chest trauma. The diagnosis was made by thoracentesis. Conservative management with nothing by mouth and total parenteral nutrition failed; therefore the patient needed surgical closure of the duct leak.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
5/5. chylothorax in Gorham's syndrome. A common complication of a rare disease.One hundred forty-six cases of Gorham's syndrome are documented in the literature. Twenty-five (17 percent) patients have been reported to have chylothorax as a complication. To our knowledge, this high incidence of chylothorax has not been reported previously. We describe two such patients treated by thoracic duct ligation and compared our results with others to support early surgical intervention via a low right-sided thoracotomy in these cases.- - - - - - - - - - ranking = 4keywords = rare disease (Clic here for more details about this article) |