1/4. Mediastinal fibromatosis presenting with superior vena cava syndrome.We encountered a fatal case of mediastinal fibromatosis in a 67-year-old female in whom there was aggressive infiltration into the large vessels, nerves and pericardium. She presented with the superior vena cava syndrome, Horner's syndrome, paralysis of bilateral vocal cords and diaphragm and heart failure. Mediastinoscopical examination revealed an extremely firm tumor adhering to the sternum, trachea and brachiocephalic artery. She died of severe heart failure due to the disturbed dilatation of the heart and ventilatory insufficiency. Although mediastinal fibromatosis is very uncommon and sometimes difficult to diagnose at an early stage, physicians should be aware of this disease for the differential diagnosis of mediastinal tumors.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
2/4. Successful treatment of recurrent pelvic desmoid tumour with tamoxifen: case report.The case report of a young woman with recurrent pelvic desmoid tumour successfully treated with tamoxifen is described. The desmoid tumour recurred within 6 months after the initial exploratory laparotomy. tamoxifen therapy led to complete relief of ascites within 2 months and complete tumour regression by the end of the fourth month, and the patient has remained stable for 6 years. Without sacrificing pelvic organs or major vessels and preserving reproductive ability, tamoxifen should be considered as the first drug of choice in such a recurrent condition.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
3/4. Aggressive intra-abdominal fibromatosis in children and response to chemotherapy.Intra-abdominal fibromatosis (IAF) is a rare benign neoplasm arising from the abdominal fibrous tissue, mostly in the mesentery. IAF is characterized by a tendency to infiltrate the surrounding vessels and vital structures and recurrence after usually incomplete surgical removal. Accordingly, IAF is associated with considerable morbidity and mortality. The authors report on a boy who presented with a large IAF at the age of 5 years. Within 6 months after initial presentation, he underwent 4 subsequent abdominal explorations for diagnosis, tumor reduction, and intestinal obstructions. IAF was confirmed by the presence of vimentin and absence of other biological cell markers. Due to accelerated tumor growth and deteriorated general condition, as a last resort, a chemotherapy trial with vincristin and methotrexate was carried out. This regimen proved to be effective in reducing the tumor burden and improving the patient's general condition. Outcome of IAF depends on early diagnosis and complete tumor resection, and, if indicated, timely employment of neo/adjuvant chemotherapy. radiotherapy must be considered in life-threatening conditions as the last resort in a growing child [2-4].- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
4/4. Giant mesenteric fibromatosis presenting as small bowel obstruction.Mesenteric fibromatosis is a proliferative fibroblastic neoplasia of the small intestine mesentery that may occur as a unique or multiple formation. Mesenteric fibromatosis is a rare, locally aggressive neoplasm and may present with abdominal discomfort, abdominal pain, weight loss, or symptoms of ureteral obstruction, mesenteric ischemia, or intestinal obstruction. It is of the utmost importance to distinguish mesenteric fibromatosis from gastrointestinal stromal tumors. Histopathology accurately differentiates between these two distinct entities. The preferred treatment is local surgical excision with a margin of uninvolved tissue. The involvement of important structures like the superior mesenteric artery and the superior mesenteric vein may pose a challenge during resection, but these tumors can be excised and the vessels repaired primarily.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |