Résumé
Biliary cystadenomas are benign but potentially malignant cystic neoplasm. The preferred treatment is radical resection because it is difficult to differentiate a benign from a malignant biliary cystadenoma. A 40 year-old woman presented with moderate abdominal discomfort. Esophageal varix was found up to mid-esophagus on endoscopy. She has no prior history of liver disease or chronic alcohol ingestion. About 15cm sized biliary cystadenoma was diagnosed by ultrasonography, computed tomography and magnetic resonance imaging. Serum level of bilirubin, alanine aminotransferase, alkaline phosphatase, gamma-glutamyl transpeptidase and tumor marker were elevated. The patient underwent US-guided aspiration. Tumor markers from the aspirated fluid are increased. Left hepatectomy was performed to completely remove the cyst. Histology of the resected specimen confirmed a biliary cystadenoma of the liver with ovary-like stroma. Without prior history of liver disease or chronic alcoholic ingestion, incidental finding of esophageal varix could show an important clue for diagnosis of biliary cystadenoma.
Sujets)
Femelle , Humains , Alanine transaminase , Alcooliques , Phosphatase alcaline , Bilirubine , Marqueurs biologiques tumoraux , Cystadénome , Diagnostic , Consommation alimentaire , Endoscopie , Varices oesophagiennes et gastriques , gamma-Glutamyltransferase , Hépatectomie , Résultats fortuits , Foie , Maladies du foie , Imagerie par résonance magnétique , ÉchographieRésumé
Multiple endocrine neoplasia type 2B (MEN 2B) is an autosomal dominant disorder characterized by medullary thyroid cancer, pheochromocytoma, neuroma and Marfanoid feature. Medullary thyroid cancer occurs in more than 95% patients of MEN 2B and increases mortality. So, the early diagnosis of multiple endocrine neoplasia is very important, because in the early diagnosed and treated medullary thyroid cancer, the prognosis is excellent. This is a case of multiple endocrine neoplasia type 2B that diagnosed early by conjunctival neuroma. A 15-year-old female patient was presented with both conjunctival masses that occurred 6 months ago. The excisional biopsy revealed conjunctival neuroma. The multiple endocrine tumor was suspected, further evaluation was performed. Medullary thyroid cancer was confirmed by thyroid ultrasound and fine needle aspiration. Finally, MEN type 2B was confirmed by a RET mutation genetic testing.