Chirurgische Therapie gastroduodenaler neuroendokriner Neoplasien

Gastroduodenal neuroendocrine tumors are rare but an increase in incidence has been recognized worldwide over the past 35 years. At the same time the prognosis of patients has substantially improved because the majority of these tumors can now be detected at an early stage. Neuroendocrine neoplasms (NENs) of the stomach are the most frequent neoplasms of neuroendocrine origin in the gastrointestinal tract. The therapeutic management of these tumors is complicated by the fact that they must be classified not only by staging and grading but also according to their pathophysiological background (types). These types differ in biological behavior and therefore have an influence on the therapeutic concept. Because more than 90?% of duodenal NENs are often asymptomatic and are as a rule identified at a curable stage, resection of the tumor should always be the first line of therapy. The therapeutic strategies vary from local endoscopic resection (duodenotomy with excision) up to pancreas retaining duodenectomy and pylorus retaining or classical Whipple procedures. This article presents the various surgical approaches to gastric and duodenal NENs.     

Endoskopische Therapie gastroduodenaler neuroendokriner Neoplasien

Neuroendocrine neoplasms (NEN) represent a group of potentially malignant tumors, which can be located in every section of the gastrointestinal tract, the pancreas and the bronchopulmonary system. Gastroduodenal NENs have a relatively good prognosis in comparison to other subentities, e.g. pancreatic or ileojejunal NENs. In the stomach there are four different types of NENs, while in the duodenum there are five types and all vary in the malignant potential and the therapeutic approach. Due to the simple access endoscopic methods not only have diagnostic but also important therapeutic relevance in this subgroup. Lesions smaller than 1 cm can easily be resected with forceps or snare resection and for larger lesions up to 2 cm more invasive strategies, such as endoscopic mucosal resection (EMR) or endoscopic submucosal dissection (ESD) are available. Important criteria in gastric NEN for the risk evaluation of endoscopic treatment alone are the size of the lesion, depth of invasion and the tumor biology, e.g. neuroendocrine tumor (NET) G1/G2 versus neuroendocrine carcinoma (NEC) G3. In duodenal NEN the aforementioned risk factors also apply and in addition only lesions outside the ampulla of Vater should be endoscopically resected whereas periampullary lesions need to be addressed surgically. As an individualized therapeutic approach the possibility of a combined endoscopic and laparoscopic resection technique exists. Follow-up endoscopic investigations are necessary, especially in gastric type 1 NENs, which have a tendency to relapse.     

Benigne und maligne Neoplasien der Nebenniere aus der Sicht der Pathologie

Die Chirurgie - Bei bildgebend und klinisch unklaren Neoplasien der Nebenniere fallen die Indikationsstellung zur Operation, aber auch die nachfolgende Klärung von Entität und...     

Minimal-invasive Resektion neuroendokriner Pankreastumoren

Pancreatic endocrine tumors (PET) are a heterogeneous group of lesions with an annual incidence of 0.1 to 0.4 per 100,000. They account for 2–4% of pancreatic neoplasms. Due to their mostly small size, some are suited to a laparoscopic approach. Preoperative localization, intraoperative laparoscopic ultrasonography, and considerable experience in pancreatic endocrine surgery and sophisticated laparoscopic techniques are essential for successful laparoscopic treatment of these tumors. If definite or highly suspicious signs of malignancy occur, a conventional open approach should be preferred. Insulinomas and small nonfunctioning PET in the pancreatic body or tail or near the surface of the pancreatic head and not in contact with the portal vein or the main pancreatic duct are suited to a laparoscopic approach. Patients with MEN1 who have insulinomas or small nonfunctioning PET may also benefit from a laparoscopic spleen-preserving distal pancreatic resection. Neither sporadic and MEN1-associated gastrinomas nor the very rare glucagonomas and vasoactive intestinal peptide-producing tumors (vipomas), which are often large and malignant, should also be tackled laparoscopically.     

Metastasierte neuroendokrine Neoplasien

The clinical presentation of neuroendocrine neoplasms (NEN) varies depending on the site of origin, functional activity, histological differentiation and tumor growth behavior. Therapeutic strategies aim to improve the symptoms of clinical syndromes related to the secretion of amines and peptides, inhibit tumor growth and prevent complications. Novel molecular targeted drug therapies offer new treatment options in addition to established therapies, such as somatostatin analogs and chemotherapy. The precise positioning of these drugs and of peptide receptor radionuclide therapy still needs to be defined. In general, the different medical treatments are used in a sequential way and are in part combined with surgical and loco-regional/ablative procedures in a highly individualized approach. In certain clinical conditions, however, established treatment strategies are followed. This review presents the management of patients with small intestinal and pancreatic NEN with respect to the functionality and tumor grading. The use of novel targeted drug therapy is included in a therapeutic algorithm.     

Maligne Neoplasien und Nierentransplantation

Together with cardiovascular disorders and metabolic changes, malignant diseases are considered as great challenges in clinical transplantation. As far as long-term function of transplanted organs is concerned, an impact of malignancies is obvious. However, it is important to distinguish between neoplastic disease originating from preexisting lesions in the transplanted organs and de novo graft tumors. Further, there is also a high risk of developing malignant disease during the dialysis, likely due to potential harmful metabolic changes associated with this procedure. After curative management of tumors in such patients, an interval of 2 years for surveillance should be adhered to before patients are put back on the waiting list. The overall risk of transmission of a malignant disease with the transplanted graft has been considered to be as low as <0.2%. In this context, and considering the continual shortage of donated organs, there is an international consensus about the use of kidney grafts with a history of small tumors (<2 cm in diameter und low-grade, i.e., G1). However, the lesions should have been removed with subsequent histopathologic characterization before the acceptance of the organ for transplantation. Early diagnosis and management of de novo malignant disease in transplant patients is crucial for the prognosis of graft function and patient survival. Genitourinary malignancies are frequent among de novo malignancies in transplanted patients. Thus, there is a need for clearly structured concepts for screening of transplant patients in order to detect early malignancies. The incidence of malignant disease correlates directly with the extent of immunosuppression in patients with end-stage renal disease (ESRD) on dialysis, as well as after transplantation with life-long immunosuppressant therapy. In addition, also geographic factors seem to play a role in the differential incidence of tumors among different populations. For instance, the highest incidence of malignancies among immunosuppressed patients has been observed in Australia followed by the USA and Europe. This might be due to the high incidence of de novo skin cancer, which has been linked to the extent of UV exposure.     

Diagnostik und Therapie von Lebermetastasen neuroendokriner Tumoren

Zusammenfassung. Neuroendokrine Tumoren entstehen vorwiegend im Gastrointestinaltrakt und sind vergleichsweise selten. Sie zeichnen sich durch langsames Wachstum aus und werden oft erst durch ihre spezifischen hormonellen Stoffwechselprodukte symptomatisch, wenn bei entsprechend gro?er hormonproduzierender Tumormasse aus dem portalen Stromgebiet anflutende stoffwechselaktive Metaboliten in der Leber nicht mehr ausreichend abgebaut werden k?nnen. Das gleiche gilt bei der sehr h?ufigen Metastasierung in die Leber selbst, wenn hormonell aktive Substanzen den systemischen Kreislauf erreichen bevor sie abgebaut werden k?nnen. Die pr?operative artspezifische Diagnostik beruht auf dem Nachweis der jeweiligen Hormone in Serum oder Urin und weniger auf der histologischen Sicherung. Die Lokalisationsdiagnostik erreicht durch spezielle Untersuchungsverfahren wie CT-AP, Somatostatinrezeptorszintigraphie, SPECT und PET eine sehr hohe Sensitivit?t. über 60 % der Patienten haben bei Erstdiagnose bereits Lebermetastasen. Bei potentiell kurativer Resektion der Lebermetastasen werden 5-Jahres-überlebensraten von über 60 % erreicht. Wegen der starken Beeintr?chtigung des Allgemeinbefindens durch die – m?glicherweise sogar lebensbedrohliche – hormonelle überproduktion kommt auch palliativen Ma?nahmen eine sehr gro?e Bedeutung zu. Nach palliativer Leberresektion betr?gt die 5-Jahres-überlebensrate fast 40 %. Damit kann die palliative Leberresektion als Alternative zur Transplantation mit einer 5-Jahres-überlebensrate von 34 % in einer Sammelstatistik erwogen werden. Falls eine Leberresektion nicht m?glich ist, kann eine wirksame Palliation der Symptome und eine Wachstumsverlangsamung durch Somatostatinanaloga zumindest vorübergehend erreicht werden.      

Pathologie der Appendizitis

Acute appendicitis represents one of the most common inflammatory diseases of the gastrointestinal tract. Acute appendicitis is most frequently based on the coincidence of obstruction, reduced blood supply, ischemic damage of the mucosa, and bacterial infection. Inflammation at other abdominal sites may also involve the appendix in 7% of cases causing periappendicitis. In 15-20% there are no pathologic findings of acute inflammation but rather post-inflammatory residues and a neuroma-like proliferation of Schwann cells, typically found in neurogenic appendicopathy. This finding may in part explain the clinical symptoms.     

Pathologie der Appendizitis

Die akute Appendizitis repr?sentiert eine der h?ufigsten entzündlichen Erkrankungen des Gastrointestinaltraktes. Die akute Entzündung der Appendix basiert zumeist auf dem Zusammentreffen von Obstruktion, eingeschr?nkter Blutversorgung, isch?mischem Schleimhautschaden und bakterieller Infektion. In ca. 7% liegt eine von au?en auf die Appendix übergreifende Entzündung im Sinne einer Periappendizitis vor, in 15-25% findet sich trotz Symptomatik eine makroskopisch blande Appendix mit postentzündlichen Residuen und einer neuromartigen Proliferation von Schwann-Zellen im Sinne einer neurogenen Appendikopathie, die in einem Teil der F?lle eine Erkl?rung für die Beschwerden liefert.     

Zur Pathologie der Thymus

Ohne Zusammenfassung (Mit 27 Abbildungen.) Siehe I. Teil: Schweizer Arch. f. Neurol., Bd. 8. II. Teil: Deutsche Zeitschr. f. Chir. Bd. 176.