Giant idiopathic benign retroperitoneal cyst: a case report.

BACKGROUND: Primary retroperitoneal cyst formation without connection to adjacent anatomical structures is a rare and benign entity with the majority of these cysts being discovered incidentally. If symptoms develop, they are generally non-specific and related to compression of the adjacent retro-/or intraperitoneal structures. Complete resection of the cyst is curative and therefore the preferred treatment option. We report a case of giant retroperitoneal cyst for which a total surgical removal was performed. CASE REPORT: A 79-year-old female consulted our department with complaints of long lasting abdominal pain and progressive abdominal distension. CT abdomen revealed a giant retroperitoneal cyst (35 cm × 25 cm × 21 cm) without radiological features of malignancy but with severe mass effect on the surrounding intra-abdominal structures. A median laparotomy was performed with a complete excision of cyst. No signs of malignancy or atypia were observed on histological examination. CONCLUSION: Idiopathic retroperitoneal cysts can slowly grow to giant proportions and subsequently lead to chronic vague abdominal symptoms. Complete surgical excision is curative and should be pursued.

Small cell carcinoma of the gallbladder.

A 72-year-old patient is reported presenting with an acute cholocystitis syndrome, caused by an extended and metastasized small cell carcinoma of the gallbladder. He was treated with palliative cholecystectomy and Platinum-based chemotherapy with short-term excellent response. Literature on the subject is reviewed and discussed.

Beta-glucuronidase deficiency as a cause of prenatally diagnosed non-immune hydrops fetalis.

We describe a case of beta-glucuronidase deficiency presenting as a non-immune hydrops fetalis diagnosed at 26 weeks of gestation. The deficiency was disclosed on cultured amniotic fluid cells and in fetal plasma and was confirmed post-abortion. In a second pregnancy, a normal beta-glucuronidase activity was found in extracts of chorionic villi obtained at 10 weeks of gestation. The pregnancy is continuing uneventfully. We conclude that it is of great importance to verify the presence of metabolic disease whenever the major causes of hydrops fetalis have been excluded.

The bile duct system and its anatomical variations.

The embryology and anatomy as well as the variations and abnormalities of the biliary system are briefly reviewed. Emphasis is placed upon the intimate anatomical relationship between extrahepatic bile duct, gallbladder, head of the pancreas and its vascular supply. The variations and abnormalities of the biliary system relevant to the endoscopist and surgeon are stressed.

Osteoblastoma of the patella. Case report and review of the literature.

Benign osteoblastoma is a rare tumor; the majority of lesions occur in the spine, femur, and tibia. Osteoblastoma in the patella is so rare that it seems not to have been reported previously in more than one case. A 29-year-old man with a sports injury, who failed to respond to conservative treatment over a prolonged period of time, was later found to have an osteoblastoma of the patella.

The significance of alpha-fetoprotein and other tumour markers in differential immunocytochemistry of primary liver tumours.

One hundred benign and malignant primary liver tumours were screened immunocytochemically for alpha-fetoprotein (AFP), alpha 1-antitrypsin, alpha-human chorionic gonadotropin, carcinoembryonic antigen (CEA), keratin and vimentin. Alpha-fetoprotein was found in 16/63 (24%) hepatocellular carcinomas and in two hepatoblastomas. When comparing tissue positivity for AFP with tumour differentiation, grade 1 hepatocellular carcinomas were found to be negative, while 21% of grade 2, 36% of grade 3 and 16% of grade 4, respectively, stained positively. Alpha-fetoprotein positive cells were present in 9/10 hepatocellular carcinomas with serum levels exceeding 5000 ng/ml, but were absent in 17 tumours with serum AFP levels below 5000 ng/ml. All tumours other than hepatocellular carcinomas and hepatoblastomas were AFP negative. Carcinoembryonic antigen was present in 72% of cholangiocarcinomas, but was demonstrated in only one hepatocellular carcinoma. This exception was a combined hepatocellular-cholangiocarcinoma in which CEA expression was restricted to the cholangiocellular part. Alpha 1-antitrypsin was found in 4/63 hepatocellular carcinomas, in 2/2 fibrolamellar carcinomas and in 2/18 cholangiocarcinomas. Alpha-human chorionic gonadotropin was detected in one hepatocellular carcinoma and was strongly expressed in both fibrolamellar carcinomas. Weak staining for keratin was seen in most tumours with hepatocellular differentiation. All cholangiocarcinomas, in contrast, were strongly labelled with the keratin antibody. Co-expression of keratin and vimentin was observed in seven poorly differentiated hepatocellular carcinomas and three cholangiocarcinomas as well as in the two hepatoblastomas. The findings suggest that AFP is a diagnostic but rather insensitive immunocytochemical marker for hepatocellular differentiation in malignant liver tumours; CEA and keratin may help in discriminating cholangiocarcinomas from hepatocellular carcinomas.