1/7. Intraabdominal lymphangiomyoma in an infant with protein-losing enteropathy and hemihypertrophy.lymphangiomyoma is an extremely rare tumor occurring exclusively in women of reproductive age. The tumor is characterized by proliferation of immature smooth muscle along the lymphatic vessels of the abdomen, thorax and lung. Although lymphangiomyoma has been reported in a young girl and a girl infant, none has been reported in boys. We report herein a case of lymphangiomyoma in a two-year-old boy. The unusual presentation in this patient was that the tumor arose from the small bowel mesentery without any evidence of lung involvement. The tumor was extirpated and lymphangiomyomatosis was confirmed pathologically.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
2/7. Ultrasound findings in retroperitoneal lymphangiomyomatosis.A 44-year old female patient with pulmonary lymphangiomyomatosis was examined by standard abdominal sonography. A wide involvement of the retroperitoneal lymph nodes and vessels was found. The relevant sonographic features were enlarged lymph nodes, and multiple anechoic structures, irregular in shape, thin-walled, with intraluminal septa. The findings, which resembled active cysts or liquid-filled bowels, were diagnosed as multiple ectasic lymphatic vessels.- - - - - - - - - - ranking = 2keywords = vessel (Clic here for more details about this article) |
3/7. Pulmonary lymphangiomyomatosis. Report of a case with immunohistochemical and ultrastructural findings.Pulmonary lymphangiomyomatosis is a rare disease characterized by wide-spread proliferation of smooth muscle cells around lymphatic and blood vessels, smaller airways and in the interstitium. Young women in the fertile status are almost exclusively affected. Spontaneous recurrent pneumothorax is the most important symptom. Chylous effusions are reported in 37.5% of cases. Our observation deals with a woman aged 43. light- and electron-microscopic findings are described. Estrogen receptors have been detected on smooth muscle cells. Bilateral oophorectomy and treatment with progesterone were successful.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
4/7. Pulmonary and extrapulmonary lymphangioleiomyomatosis. Report of a case with bilateral renal angiomyolipomas, multifocal lymphangioleiomyomatosis, and a glial polyp of the endocervix.The authors report an extraordinary case of a 33-year-old female who had pulmonary and extrapulmonary lymphangioleiomyomatosis, bilateral renal angiomyolipomas, multifocal lymphangioleiomyomatosis involving the uterus, ovaries, periadrenal vessels, and liver. The patient also had a glial polyp of the endocervix which represented retained fetal parts from an abortion 15 years earlier. Both ovaries showed multiple follicle cysts which may have been a source of endogenous estrogen. The classical stigmata of the tuberous sclerosis complex were absent. The multifocal lymphangioleiomyomatosis of the various organs described presents an extremely rare manifestation of this disorder.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
5/7. Pulmonary lymphangiomyomatosis.Pulmonary lymphangiomyomatosis is a rare disease of smooth muscle proliferation in the walls of lymphatic vessels and in the interstitial areas of the lungs. It only affects women of reproductive age. The majority of patients die from respiratory failure within 10 years. A new observation of pulmonary lymphangiomyomatosis is reported in a woman of 36 years. The disease was discovered at an advanced stage of respiratory failure. Left-sided pleurectomy and treatment with medroxy-progesterone and tamoxifen did not improve the patient's condition.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
6/7. Retroperitoneal lymphangiomyoma in an infant.Lymphangiomyomatosis (LAM) is a benign tumor-like lesion of lymphatic vessels with unknown etiology. 80 cases of LAM are presently described. So far, it appears that only women in their reproductive years seem to be affected. Here, we describe the first LAM in an 11-months-old infant with a presumably local form of LAM. This leads us to consider the possibility that this tumor-like lesion may originate from a hamartomateous malformation. The diagnosis is based on pre-operative sonography and CT, as well as on histological and immunohistochemical examination of the tumor.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
7/7. Mediastinal lymphangiomyoma in a child: report of a case.Lymphangiomas and lymphangiomyomas are benign abnormal collections of lymphatic vessels which form a cystic mass. The mediastinum is an uncommon location for these hamartomas in children, but may be life-threatening. We report herein the case of a 23-month-old boy who presented with stridor and a history of recurrent respiratory tract infections. Soon after his admission he suddenly developed respiratory failure and was found to have a multiloculated, cystic mediastinal mass which was initially thought to be a lymphangioma. The mass was extensively infiltrative, and histological examination showed prominent smooth muscle proliferation in the walls of the cysts, confirming a diagnosis of lymphangiomyoma. The terminology and relevant literature on the topic are reviewed following the presentation of this case.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |