Unusual gastric mucosal infiltration by a medullary thyroid carcinoma: a case report.

BACKGROUND: Medullary thyroid carcinoma accounts for approximately 1 to 2 % of all thyroid carcinoma cases. The most common route of dissemination is to locoregional lymph nodes. Distant metastases commonly affect bones, lungs, and liver. We present a case of a white woman with a 25-year history of medullary thyroid carcinoma on multiple medications including tyrosine kinase inhibitor therapy for the last 11 months, who exhibited unusual diffuse infiltration of advanced stage medullary thyroid carcinoma to her gastric mucosa. CASE PRESENTATION: A 53-year-old white woman presented with increasing fatigue, loss of appetite, and severe epigastric pain radiating to her back. She had a history of medullary thyroid carcinoma (pT2pN1b), diagnosed 25 years ago and treated by complete thyroidectomy and repeated bilateral cervical lymph node dissection. Medical therapy included octreotide 20 mg every 4 weeks, which was switched to the tyrosine kinase inhibitor vandetanib 300 mg/day 11 months ago when computed tomography scanning revealed progressive mediastinal lymph node and diffuse and symptomatic pulmonary metastases. Of note, she demonstrated macroscopically stable pulmonary and mediastinal lymph node metastases; however, her calcitonin serum levels dramatically increased. Computed tomography scanning revealed a single new intrahepatic lesion (4 mm) as well as multiple (>10) new supraclavicular lesions suggestive of medullary thyroid carcinoma progress. As proven by gastric biopsy and immunohistochemical evaluation, her epigastric pain was explained by a diffuse infiltration of her gastric mucosa by metastatic medullary thyroid carcinoma. Subsequently, she rapidly deteriorated and died. CONCLUSIONS: The current case report shows for the first time an unusual metastatic infiltration of the gastric mucosa by medullary thyroid carcinoma. When treating these patients, it is important to include this differential diagnosis during follow-up.

Whipple's disease - current status of diagnostics and therapy.

Whipple's disease is a multisystemic infectious disease whose pathogen, a gram-unstable actinomycete, has been characterized in the meantime by molecular-biological techniques (polymerase chain reaction). This infectious disease which was firstly described in 1907 by G.H. Whipple as intestinal lipodystrophy appears rarely and sporadically and can affect nearly every organ, in the course of which the small intestine is also concerned in the majority of patients. The symptoms and signs are polymorphous and depend on organic involvement and stage. This leads to significant difficulties concerning differential diagnosis and to a delay in diagnosis. Misjudging the syndromes provokes invalidism and death whereas correct therapy leads to a cure in most of the cases.

Evaluation of ERCP- and endoscopic sphincterotomy-induced pancreatic damage: a prospective study on the time course and the significance of serum levels of pancreatic secretory enzymes.

In the present study the time courses of serum lipase, serum amylase and serum elastase 1 after ERCP/ES as indicators for pancreatic damage were prospectively analysed in 46 cases. The elevations of pancreatic enzymes after ERCP/ES scattered in a wide range and elevations occured which were greater than one hundred times the upper limit of normal. A moderate increment was seen as early as 5 minutes after intubation of the papilla. Elevations above the upper limit of normal were still seen at 24 hours after the procedure. The maxima occurred about 6 hours after the procedure. Lipase was the most sensitive among the parameters tested, nearly 50% of the cases with previously normal values revealed elevated lipase after the procedure. For daily clinical routine a single lipase measurement at 2 hours after the beginning of the ERCP/ES provides valuable information for planning further surveillance. Younger age and high calcium levels seem to be risk factors for ERCP/ES-induced pancreatic damage. The time course of serum lipase seems to be a more reliable criterion for ERCP/ES-induced pancreatic damage than the poorly defined complication of post-ERCP pancreatitis . The high incidence of a measurable pancreatic injury after ERCP/ES provides a sensitive tool for the testing of drugs claimed to be protective for the pancreas and for evaluating new ERCP/ES techniques. Measurement of the serum lipase before, 8 and 24 hours after the procedure, and a detailed description of degree and duration of pain, are necessary for such studies.

Octreotide in the prevention of pancreatic damage induced by endoscopic sphincterotomy.

Somatostatin and the long acting analogue octreotide have been proposed as a therapeutic agent in acute pancreatitis and for the prophylaxis of pancreatic damage by ERCP and EST for their ability to reduce exocrine pancreatic secretion. However, clinical trials could not show significant beneficial effects in acute pancreatitis and ERCP. In patients undergoing EST, data remained controversial, most authors describing positive effects of prophylaxis. In this study we investigated the use of octreotide prophylaxis to reduce EST-induced pancreatic damage in a randomised, double blind trial. 94 consecutive ERCP/EST-patients were randomised to receive either octreotide 200 microgram s.c. or placebo 3 times daily, starting the night before endoscopic procedures. In 59 patients EST was performed. Blood samples were collected before and 40 min, 2 hrs, 6 hrs, 24 hrs, 48 hrs and 72 hrs after the endoscopic procedures. Samples were analysed for pancreatic serum enzymes, acute phase proteins and blood counts. A clinical pain score was investigated. Post-EST-pancreatitis (amylase > 3x upper limit and persistent abdominal pain) was diagnosed in 3 patients in the treatment group, in 4 patients in the placebo group. There were no significant differences in the time-courses of serum enzymes or acute phase proteins in-between the groups, nor in the pain-score. According to these data, prophylactic octreotide application does not prevent acute pancreatic damage induced by endoscopic sphincterotomy.

[Purulent pericarditis as an initial manifestation of esophageal carcinoma]. / Eitrige Perikarditis als Erstmanifestation eines Osophaguskarzinoms.

HISTORY AND ADMISSION FINDINGS: A 71-year old heavy smoker was admitted because of chest pain unrelated to physical activity, radiating into the left arm and neck as well as exertional dyspnoea and dizziness. Physical examination was unremarkable except for mild venous congestion over the upper part of the body. Myocardial infarction was excluded. A haemodynamically significant pericardial effusion developed a few days later and required emergency pericardiocentesis. INVESTIGATIONS: Laboratory tests indicated marked inflammatory disease. Echocardiography demonstrated the pericardial effusion. Needle aspiration revealed coagulase-negative staphylococcus and plant cells. Chest X-ray showed a pneumopericardium. Computed thoracic tomography suggested malignant tumour of the oesophagus with spread to the surrounding lymph nodes and pericardial fistula. Proximal endoscopy showed a highly malignant looking ulcer, 30 cm in diameter, in the anterior wall of the oesophagus with a central fistula. The endoscopic biopsy indicated a poorly differentiated non-cornified squamous cell carcinoma. TREATMENT AND COURSE: An uncovered self-expanding metal stent was placed into the fistula, whereupon the perimyocarditis quickly healed. When the patient was discharged he was able to take food by mouth and the signs of inflammation subsided. He died at home 6 weeks later. CONCLUSION: An oesophageal carcinoma with fistula should be included in the differential diagnosis of purulent pericardial effusion even in the absence of dysphagia. Implantation of a self-expanding metal stent into the fistula is the treatment of choice for palliation.

[Acinar cell tumor of the pancreas]. / Azinuszelltumor des Pankreas.

A case of a solid and cystic acinar cell tumor or pancreas is reported. The macroscopic and microscopic features, the ultrastructure, the immunocytochemical characteristics and the typical epidemiology of this extremely rare and, since a short time, new entity are depicted. The differential diagnosis to other tumors of the pancreas is discussed.

Evidence for a hepatocellular lineage in a combined hepatocellular-cholangiocarcinoma of transitional type.

A combined hepatocellular-cholangiocarcinoma (CHC) of transitional subtype and the surrounding cirrhotic liver tissue were investigated immunocytochemically by monoclonal antibodies specific for each of the keratin polypeptides 7, 8, 18 and 19. Different keratin subsets were found in different parts of the tumour. The hepatocellular component reveals keratins 8 and 18, with the bordering cells of trabecular formations additionally expressing keratins 7 and 19. The same keratins i.e. 7, 8, 18, 19 were found in normal bile duct epithelium as well as in cholangiocarcinomatous and transitional areas of hepatocellular and cholangiocellular differentiation. Normal hepatocytes express only keratin 8 and 18. In cirrhotic liver some modified hepatocytes additionally express keratin 7. When ductal transformation is observed in the marginal parts of portal tracts and fibrous septa the keratin polypeptide pattern mimics that of bile duct epithelium. The cholangiocellular metaplasia of hepatocytes observed here correlates well with findings in hepato-organogenesis and hepatocarcinogenesis and suggests that the transitional subtype of combined hepatocellular-cholangiocarcinoma is a variant of hepatocellular carcinoma.

Frequency of pancreatitis after endoscopic retrograde cholangiopancreatography with iopromid or iotrolan: a randomized trial.

Blood isotone contrast media is considered to be less toxic to vascular and pancreatic duct endothelium than high-osmolar contrast media. In this study we assessed the impact of a low-osmolar contrast agent compared with a blood isotone product on pancreatic damage induced by endoscopic retrograde cholangiopancreatography (ERCP) and endoscopic retrograde sphincterotomy (EST). In a prospective trial 42 consecutive ERCP/EST patients were randomized to receive either iopromid, a low-osmolar non-ionic contrast agent (770 mosmol/kg H2O), or iotrolan, a blood-isotone non-ionic product (320 mosmol/kg H2O). The endoscopies were performed by two experienced endoscopists. Forty patients were included in the study. Blood samples were collected before and 40 min, 2, 4, 6 and 24 h after the endoscopic procedure. Samples were analysed for pancreatic serum enzymes, acute-phase proteins and blood counts. A clinical pain score was investigated. Post-ERCP pancreatitis was diagnosed in 2 patients in the iopromid group and in 5 patients in the iotrolan group. There was no significant difference between groups in the time course of pancreatic serum enzymes, acute-phase proteins or in the pain score. Due to the small number of patients in this study, only stronger differences caused by the two contrast media could have led to statistically significant results. We did not observe statistically significant differences in comparing iotrolan and iopromid concerning ERCP/EST-induced pancreatic damage.

[Hemodynamic and lung function analytic findings in so-called collagen diseases]. / Hämodynamische und lungenfunktionsanalytische Befunde bei sogenannten Kollagenosen.

Twenty-four patients with connective tissue disease as defined by ARA criteria were submitted to a thorough cardiological and pulmonary diagnostic evaluation. Mean pulmonary arterial pressure was elevated in 54 per cent of the patients, a left-ventricular functional disorder taking the form of elevated pulmonary capillary occlusive pressure was almost equally as frequent. Interindividual comparisons suggest only a low progressivity of the cardiac involvement, while pulmonary involvement progresses rapidly, to become the prognostically predominating factor. This suspicion must be checked by performing follow-up examinations with repeated cath. examinations of the right heart in individual patients. In common with ACE determinations, haemodynamically effective pericardial disorders are of no significance in connective tissue diseases. Patients with "collagenosis" should be submitted to right-heart catheterisation early on. Attempts at therapy taking the form of aggressive treatment of the underlying disease or administration of nitrates or calcium antagonists, would appear meaningful.