1/6. Pulmonary lymphangiomyomatosis (LAM) developing chylothorax.We describe a case of pulmonary lymphangiomyomatosis (LAM) with chylothorax that developed in a 46-year-old Japanese woman. This patient exhibited clinical symptoms of dyspnea and chest X-ray showed right pleural effusion. Thoracocentesis demonstrated chylous effusion. Chest computed tomography (CT) scan revealed multiple cystic lesions. Subsequent thoracoscopy revealed the chylorrhea from swelled vessels on the diaphragm. The clinical diagnosis, based on histological examinations with biopsy specimens obtained by thoracoscopy, was pulmonary LAM. Although the hormone therapy was not effective, chylous effusion was improved by the pleurodesis. Pulmonary LAM developing chylothorax is rare in japan.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
2/6. An unusual renal angiomyolipoma with morphological lymphangioleiomyomatosis features and coexpression of oestrogen and progesterone receptors.angiomyolipoma is the most common mesenchymal renal tumour, the clonal origin of which has recently been demonstrated. It is composed of varying amounts of blood vessels, smooth muscle and fat. In this report, we describe a renal angiomyolipoma, which is unusual owing to the presence of a lymphangioleiomyomatosis-like component, occurring in a 41-year-old woman suffering from sporadic lymphangioleiomyomatosis. The diagnosis was based on histopathological and immunohistochemical findings. The tumour consisted of an intimate admixture of two components: one was typical of a classical angiomyolipoma and the other was reminiscent of lymphangioleiomyomatosis. HMB45 positivity was found on 5% of the cells of the angiomyolipoma component. Ten percent of the nuclei of the lymphangioleiomyomatosis and angiomyolipoma components expressed oestrogen receptors and 5% progesterone receptors. This case illustrates a very unusual pattern of a renal angiomyolipoma containing a lymphangioleiomyomatosis-like component. The oestrogen and progesterone immunoreactivity suggests that angiomyolipoma could be hormonally dependent. Therefore, we have emphasised the morphological and immunohistochemical similarities between angiomyolipoma and lymphangioleiomyomatosis.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
3/6. lymphangioleiomyomatosis initially presenting with abdominal pain: a case report.lymphangioleiomyomatosis (LAM) is a rare idiopathic disease characterized by hamartomatous smooth muscle proliferation of the lymph node, lymphatics, blood vessels as well as airways within the lungs, mediastinum and abdomen. It exclusively affects women during the reproductive period. Though extrapulmonary manifestations have been reported, the initial presentation of LAM as abdominal pain is extremely rare. To our knowledge, there are only three cases with LAM presenting first with abdominal symptoms so far [Chest 106 (1994) 267; Eur J Radiol 14 (1992) 192; Eur J Surg 157 (1991) 36]. We describe a case of LAM suffering from abdominal pain followed by pulmonary symptoms and the diagnosis was not made until pathohistological examination.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
4/6. Conservative treatment of a mesenteric lymphangiomyomatosis in an 11-year-old girl with a long follow-up period.The authors report the case of a 11-year-old girl admitted for a mesenteric tumor discovered during an appendicectomy. The abdominal Doppler echography and computed tomography (CT) scan showed a 8 x 6 x 7-cm multinodular mass involved in the mesenterium. biological markers and iodine 131 meta-iodobenzylguanidine (MIBG) scintigraphy results were normal. Percutaneous biopsies were performed. pathology results showed perilymphatic proliferation of smooth muscle cells leading to cystic lesions. No sign of malignancy was seen. laparotomy showed a multinodular fixed mass involved from the root of the mesenterium. The superior mesenteric vessels were encircled by the tumor, which could not be resected completely. Partial resection was performed. The pathology and the immunohistochemical tests confirmed the diagnosis of lymphangiomyomatosis. At 21 years of age, she remains asymptomatic. An echography and CT scan showed the mass remaining unchanged in size and echopattern. Lymphangiomyomatosis (LMA) is thought to affect exclusively women in their reproductive life, and the steroid hormones are supposed to play an essential role in the pathology and in the behavior of this condition. Infantile LMA is exceptional. The patient reported is remarkable for her young age, the location of the tumor, her long history, and her benign course. Because the tumor is supposed to be hormone dependent, the surge of oestrogen in this 21-year-old woman may initiate the extensive form of lymphangiomyomatosis with pulmonary involvement, the prognosis of which is extremely poor.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
5/6. Extrapulmonary lymphangioleiomyomatosis and lymphangiomatous cysts in tuberous sclerosis complex.OBJECTIVE: To describe the clinical manifestations, imaging findings, and histologic features of extrapulmonary lymphangioleiomyomatosis (LAM) in the tuberous sclerosis complex (TSC). DESIGN: We retrospectively reviewed institutional medical records since 1940 to identify patients with TSC and extrapulmonary LAM. MATERIAL AND methods: Of 403 patients with TSC, 3 had pulmonary and extrapulmonary LAM and retroperitoneal lymphangiomatous cysts. The clinical, imaging, and histologic features of these three patients were summarized, including analysis of biopsy specimens by conventional histology, immunohistology, radiolabeled ligand-binding assays, and tissue culture. RESULTS: The three young women had characteristic dermatologic findings of TSC and pulmonary LAM. Two patients were of normal intelligence, and one had a recent history of contraceptive use. All three patients had intra-abdominal lymphangiomatous cysts, uterine LAM, and renal angiomyolipomas. Renal and uterine biopsy specimens demonstrated positive immunostaining for melanoma-related antigens and expression of estrogen and progesterone receptors by ligand-binding assay and immunohistology. Cells cultured from LAM tissue of one of the patients exhibited a mitogenic response to estradiol. CONCLUSION: Clinically significant extrapulmonary LAM is a rare manifestation of TSC and may occur in women with this disease who also have pulmonary LAM. The clinical features of these patients confirm the importance of sex steroids in the development of these lesions. Immunohistochemical findings suggest that LAM and angiomyolipomas have a neuroectodermal origin. The development of lymphangiomatous cysts in these patients is probably due to smooth muscle proliferation in lymph vessels, which can result in lymphatic obstruction.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
6/6. lymphangioleiomyomatosis with chylous ascites.A 41-year-old Malay housewife presented with recurrent chylous ascites and progressive cachexia over 17 years. A diagnosis of lymphangioleiomyomatosis (LAM) was established by laparotomy where biopsy of the liver, peritoneum and adhesions from previous surgery showed smooth muscle proliferation in the blood vessels and lymphatics. Clinically and radiologically, there was no evidence of pulmonary involvement. She was treated with dietary fat restriction and medium-chain triglycerides. This is the first case of LAM reported in malaysia.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |