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Background: After the first case publication using the term "autoimmune pancreatitis" in 1995 and the successful treatment with steroids we now can distinguish between two clinical und histopathological forms of autoimmune pancreatitis. Type 1 autoimmune pancreatitis (AIP) is usually part of an IgG4-related systemic disease. AIP Typ 2 is an IgG4-independent pancreatic disease. For both entities pancreas cancer is the most important differential diagnosis., Case Report: We report the case of an 82-year-old male patient who primarily presented with obstructive jaundice. Computed tomography (CT) revealed the typical image of a small cancer of the head of the pancreas with pulmonary metastases. After endoscopic drainage of the bile duct a CT-guided biopsy of a pulmonary nodule was performed in which cancer was ruled out. Next the patient was treated with steroids because of "tumour-associated cachexia". In the follow-up the mass in the head of the pancreas like the lung nodules had surprisingly disappeared. In the complete work-up the immune histochemical staining of the lung biopsy revealed subsequently a typical IgG4-associated inflammation. After termination of the therapy the disease relapsed as sclerosing cholangitis., Conclusion: The IgG4-related systemic disease with AIP can present as cancer of the pancreas with lung metastases. Extrapancreatic IgG4-positive histopathology and response to therapy with steroids can help to diagnose the disease in complex clinical presentations., (© Georg Thieme Verlag KG Stuttgart · New York.)

Neuroendocrine tumors (NETs) of the small bowels are on the rise: in the US they have increased by 300-500% in the last 35 years. At the same time their prognosis has been much improved. Most NETs of the duodenum are nowadays detected "incidentally" and therefore recognized at an early stage. Duodenal NETs that are well differentiated, not larger than 10 mm in greatest dimension and limited to the mucosa/submucosa can be endoscopically resected. In NETs with a size between 10 mm and 20 mm the therapeutic strategy has to be individually discussed. Endoscopic ultrasound is the method of choice to determine tumor size and depth of infiltration. Surgery is indicated for well differentiated duodenal NETs greater than 20 mm, for localized sporadic gastrinomas and for localized poorly differentiated NE cancers. Surgery is also indicated for localized/regional ileal NETs. Advanced ileal NETs with a carcinoid syndrome are treated with stable somatostatin analogs. This treatment also significantly improves the (progression-free) survival in patients with metastatic NETs of the ileum. For optimal NET management tumor biology, type, localization and stage of the neoplasm as well as the individual situation of the patient have to be taken into account., (Georg Thieme Verlag KG Stuttgart, New York.)

Rectal carcinoids are becoming more common: in the USA they have increased in frequency by 800 - 1000 % in the past 35 years. This dramatic increase is probably related to the introduction of colonoscopic screening which also results in the "incidentally" detected neuroendocrine rectal tumours/carcinomas being smaller than in the pre-screening era. Endosonography is the method of choice for determining the size and depth of penetration of the tumours and for detecting lymph node metastases. Tumours of < 10 mm in size that have not infiltrated the muscularis propria can usually be removed endoscopically. When invasion of lymph or blood vessels or lymph node metastases are found, surgical resection of the lymph nodes is indicated. Neuroendocrine rectal neoplasms (rectal carcinoids) of 10.1 - 20 mm in diameter have a metastasis risk of 17 - 42 (81) % for neuroendocrine rectal neoplasms > 20 mm in size this risk increases to 60 - 80 %. A carcinoid syndrome is rarely observed, even in cases of distant metastases of neuroendocrine rectal carcinomas. Stable somatostatin analogues and interferon-alpha constitute the drug therapies of choice for carcinoid syndrome. As a result of the increasing early detection of rectal carcinoids/carcinomas the prognosis for the patients has improved considerably in the last 30 years. In addition to the early detection of colorectal adenoma and adenocarcinoma, screening colonoscopy also makes possible the early detection and early therapy for neuroendocrine rectal tumours/carcinomas.

A 40-year-old female patient was admitted with bleeding from esophageal varices. The histology of liver and spleen showed granulomatous infiltrations that had developed without symptoms so far. The portal hypertension and a thrombopenic bleeding diathesis gave rise to the establishment of a splenorenal shunt with exstirpation of the spleen. By this the patient could be cured symptomatically, as three years after the acute episode liver functions are still normal. In the light of this case, pathogenesis, the variety of etiologies and the clinical courses of granulomatous liver diseases are discussed in detail with regard to the differential diagnosis.