ABSTRACT
A limited number of sclerosing Angiomatoid Nodular Transformation (SANT) have been reported in pediatric age. We describe the first case of SANT occurring in a nine-week-old female infant that was admitted to our unit for severe abdominal distension and rectal bleeding. Enlarged spleen was detected on physical examination. Laboratory investigations revealed severe anemia and coagulation abnormalities. Abdominal ultrasound and computed tomography revealed ascites and splenomegaly with a large mass at the lower medial splenic pole. A diagnosis of intraabdominal hemorrhage was presumed and an exploratory laparotomy was performed. A complete transformation of the giant splenomegaly to bossellated masses and multiple bleeding capsular ruptures without subcapsular hematoma were found and an urgent splenectomy was performed. At histology, a SANT was diagnosed (CD34, CD31, CD8 positivity). The postoperative follow up was uneventful. SANT may also occur in infancy with a potentially lifethreatening presentation. Splenectomy may represent the only treatment in severe cases.
ABSTRACT
From 1987 to 2000 108 patients were operated on for thymic tumours. Two of these underwent replacement of the superior vena cava with polytetrafluoroethylene prostheses because the tumours invaded the superior vena cava. One of these was affected by myasthenia gravis, and was treated preoperatively with concurrent radio-chemotherapy and lymphocytopheresis. The other received preoperative chemotherapy and post-operative radio-chemotherapy because of minimal residual disease. The clinical courses of the two patients are reported here and we conclude that eradication of thymic malignancies is suitable even when superior vena cava replacement is required, and that neither antimyasthenic therapy nor adjuvant and/or neoadjuvant treatment interfere with aggressive surgical management or vice versa. Moreover, the long-term survival of one of these patients despite relapse of disease shows that extended surgery is indicated in these cases.