ABSTRACT
INTRODUCTION: We report the first case of immunophenotypically confirmed primary hepatic Burkitt lymphoma presenting with acute liver failure. The patient survived following an aggressive diagnostic approach followed by emergent chemotherapy. DISCUSSION: Data from the primary hepatic non-Hodgkin lymphoma literature show a survival rate of 87% at 5 years with combination regimens of chemotherapy. However, mortality rate in this population is 85% in patients with acute liver failure. CONCLUSION: Primary hepatic Burkitt lymphoma may respond well to emergent chemotherapy even in the setting of acute liver failure.
Subject(s)
Burkitt Lymphoma/complications , Liver Failure, Acute/etiology , Liver Neoplasms/complications , Antibodies, Monoclonal, Murine-Derived/administration & dosage , Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Antiretroviral Therapy, Highly Active , Burkitt Lymphoma/drug therapy , Burkitt Lymphoma/pathology , Cytarabine/administration & dosage , Dexamethasone/administration & dosage , Female , HIV Infections/complications , HIV Infections/drug therapy , Humans , Liver Failure, Acute/physiopathology , Liver Neoplasms/drug therapy , Liver Neoplasms/pathology , Middle Aged , RituximabABSTRACT
BACKGROUND: Annular pancreas is rare; only 737 cases have been reported in the English literature. In addition, no large analysis has compared children and adults. Recently, prenatal diagnosis and advances in imaging have led to increased experience with this condition. STUDY DESIGN: Data from 103 patients (48 children, 55 adults) with annular pancreas, managed from 1992 to 2006, were reviewed. Patients with isolated duodenal atresia, stenosis, or webs were excluded. RESULTS: Median ages at diagnosis were 1 day in children and 47 years in adults. Annular pancreas was more common in girls and women (children, 58%; adults, 69%). Congenital anomalies were more frequent (p < 0.01) in children (71%) than in adults (16%); Down syndrome, cardiac, and intestinal anomalies were most common. Prenatal diagnosis was suspected in 56% of infants, and adults presented with pain (75%), vomiting (24%), pancreatitis (22%), or abnormal liver tests (11%). All children were managed with duodenal bypass. Children were more likely (p < 0.01) to require surgery for associated anomalies. In contrast, adults had fewer duodenal bypass procedures (24%) but more often required endoscopic pancreatobiliary procedures (67%), cholecystectomy (56%), and other pancreatobiliary surgery (20%; p < 0.01). Adults more commonly (p < 0.01) had pancreas divisum (29%) and pancreatobiliary neoplasia (11%). Five children (6%) with multiple anomalies died; all adults survived their operations. Late deaths occurred in 2 children (4%) with multiple anomalies and 3 adults (5%) with pancreatobiliary cancer. CONCLUSIONS: Annular pancreas is associated with a spectrum of disease that differs in children and adults. Congenital anomalies are more common in children with annular pancreas; complex pancreatobiliary disorders and malignancy are more frequent in adults.
Subject(s)
Digestive System Abnormalities/epidemiology , Pancreas/abnormalities , Adolescent , Adult , Age Factors , Aged , Aged, 80 and over , Child , Child, Preschool , Digestive System Abnormalities/diagnosis , Digestive System Abnormalities/surgery , Female , Humans , Infant , Infant, Newborn , Male , Middle AgedABSTRACT
Composite tumors containing pheochromocytoma and ganglioneuroma are rare tumors typically arising in the adrenal glands. Very few were reported outside of the adrenals. We present the case of a middle-aged woman with a retroperitoneal mass that was discovered when she complained of local signs of compression and systemic signs of catecholamine hypersecretion. On pathology the mass was composed of large polygonal and pleomorphic cells arranged in nests characteristic of pheochromocytoma that were mixed with clusters of mature ganglion cells and bundles of spindle cells characteristic of ganglioneuroma. The histological features were benign, but due to its invasion of the inferior vena cava and of the right renal artery, we had concerns about the malignant potential of the pheochromocytoma component, which is extremely rare in these composite tumors.
