Neuroendokrine Neoplasien des gastroenteropankreatischen Systems

Neuroendocrine neoplasms (NEN) appear to be morphologically homogeneous, however, biologically and clinically they are a very heterogeneous tumor group. They can occur in every organ but are most common in the lungs and the gastroenteropancreatic system (GEP). In order to unify and improve the diagnosis and therapy of GEP-NEN the European Neuroendocrine Tumor Society (ENETS) has developed guidelines over the last 5 years. In consideration of these guidelines the TNM classification of the Union for International Cancer Control (UICC) was introduced in 2009 and only 1 year later the new GEP-NEN classification of the World Health Organization (WHO) was presented. This review summarizes the new classification and the morphological diagnosis of GEP-NEN. Based on these classifications an optimal prognostic stratification of GEP-NEN is possible which is the basis for imaging and treatment and allows a comparison of large tumor collectives.     

Neuroendokrine Tumoren des Kolons und Rektums

Colonic neuroendocrine tumours (NET), frequently locally advanced, are often misdiagnosed as undifferentiated adenocarcinoma. Their stage-related prognosis is similar or slightly worse than for adenocarcinomas, more often showing distant metastases. Surgical therapy is performed according to oncological guidelines in colonic tumour surgery. Highly differentiated, even metastasized tumours should be treated by radical surgery because of a rather good prognosis. Small cell cancer is frequently advanced and can be treated with chemotherapy such as SCLC, accompanied by radical or palliative surgery. Rectal NET are mainly small incidental findings. Their metastatic potential correlates with submucosal infiltration. Tumours up to 2 cm can be locally excised. Larger tumours and locoregional disease should be radically resected. Extensive metastatic disease and low grading warrant rather palliative local and systemic therapy. Prognosis is comparable to rectal adenocarcinoma.     

Neuroendokrine Tumoren

Neuroendokrine Tumoren kommen im Gastrointestinaltrakt und im Pankreas vor. Sie k?nnen benigne oder maligne und funktionell aktiv oder inaktiv sein. Benigne und maligne funktionell aktive Tumoren verur sachen durch eine ungebremste Hormonproduktion charakteristische Syndrome. Die biochemische Diagnostik basiert auf dem Nachweis dieser Hormone. Inaktive Tumore werden entweder zuf?llig entdeckt oder führen durch ihr verdr?ngendes Wachstum zu Beschwerden. Der wichtigste Marker metastasierter, funktionell inaktiver Tumoren ist Chromogranin A. Maligne neuroendokrine Tumoren wachsen meist langsam und haben daher eine bessere Prognose als andere Malignome. Als Therapieoption steht die chirurgische Resektion des Prim?rtumors und eine Reduktion der Metastasen im Vordergrund. Ebenfalls eine Rolle spielen Somastotatin- Analoga, ablative Verfahren wie Chemoembolisation und die Radioligandentherapie. Eine Chemotherapie ist eher selten indiziert.     

Neuroendokrine Tumoren

Zusammenfassung Neuroendokrine Tumoren des Gastrointestinaltrakts können benigne oder maligne, funktionell aktiv oder inaktiv sein. Funktionell aktive Tumoren führen über eine ungebremste Hormonproduktion zu charakteristischen klinischen Syndromen wie dem Karzinoidsyndrom. Funktionell inaktive Tumoren werden entweder zufällig im Rahmen Bild gebender Untersuchungen entdeckt oder führen durch ihr verdrängendes Wachstum zu Beschwerden. Die biochemische Diagnostik funktionell aktiver neuroendokriner Tumoren basiert auf dem Nachweis der ungebremsten Freisetzung des für ein bestimmtes Syndrom verantwortlichen Hormons durch den Tumor. Der wichtigste biochemische Marker metastasierter, funktionell inaktiver Tumoren ist das Chromogranin A. Der natürliche Krankheitsverlauf neuroendokriner Tumoren ist in der Regel günstiger als bei vielen anderen Malignomenn und wird durch deren langsames Wachstum bestimmt.     

Moderne molekulare und bildgebende Diagnostik bei neuroendokrinen Neoplasien des Pankreas

best practice onkologie - Neue molekulardiagnostische und bildgebende Verfahren bieten sich an für die Einschätzung der Ausdehnung, des Rezidivrisikos, der Prognose und des...     

HIV-assoziierte Neoplasien

best practice onkologie - Trotz der erheblichen Fortschritte in der Behandlung der Infektion mit dem humanen Immundefizienzvirus (HIV) treten neue Herausforderungen für Therapeuten zutage. In...     

BCR-ABL-negative myeloproliferative Neoplasien

BCR-ABL negative myeloproliferative neoplasms are a heterogeneous group of disorders characterized by a proliferation of one or more myeloid cell lineages. The most common entities are polycythemia vera, essential thrombocythemia and myelofibrosis in which Janus kinase (JAK) and myeloproliferative leukemia virus oncogene (MPL) mutations are found in 60?C70% of patients resulting in constitutive activation of the JAK-STAT signal transduction pathway. The clinical and morphological characteristics in peripheral blood and bone marrow are nowadays regularly complemented by cytogenetic and molecular analyses. Precise diagnostic criteria and prognostic markers allow a reliable evaluation of the therapeutic options. Effective targeted therapies have now been developed against pathogenetic signaling pathways, e.g. JAK inhibitors, which in the future will possibly be used as monotherapy or in combination with or without subsequent allogeneic stem cell transplantation.     

Chirurgische Therapie gastroenteropankreatischer neuroendokriner Neoplasien

Neuroendocrine neoplasms of the gastroenteropancreatic tract (GEP-NET/NEC) represent a heterogeneous group of tumors with variable biological behavior and frequently only unspecific symptoms. They are diagnosed with increasing frequency and also earlier with subsequent improvement in survival. The sophisticated assessment of the anticipated prognosis, dependent upon the manifold forms of appearance of GEP-NET/NEC, is based on recent actualizations of classification systems of the European Neuroendocrine Tumor Society (ENETS) from 2006/2007 and the UICC/AJCC classification of the WHO from 2010. The optimal, often multimodal, treatment strategy is the result of an interdisciplinary discussion in specialized tumor boards. Depending on the localization and type of the primary tumor as well as on the extent of the disease the surgical therapy ranges from minimally invasive organ-preserving resection to multivisceral resection and remains the only curative approach. In case of non-resectability or following incomplete resection, complementary therapies (e.g., TACE, RFA, SIRT, biotherapy, radiopeptide therapy) and chemotherapy seem to improve patient prognosis. This article characterizes the different stage-oriented surgical approaches for GEP-NET/NEC.     

Klinik und Diagnostik neuroendokriner Neoplasien

The incidence and prevalence of neuroendocrine neoplasia are still being underestimated. These neoplasia were considered to be benign in the past, however, the malignant potential is today evident and increasingly more recognized. The (early) diagnosis of these tumors requires a high degree of suspicion and knowledge of symptoms caused by the secretion of proteins and hormones. When symptoms have been recognized patients can be subjected to appropriate diagnostic procedures. Unfortunately even “typical??symptoms are often nonspecific. Excellent biochemical, endoscopic radiological options and nuclear medicine techniques are available for diagnostic purposes. A sensible combination allows early (or earlier) diagnosis, risk stratification, planning of individual therapeutic procedure and follow-up.     

H?matologische Neoplasien in der Schwangerschaft

The management of hematologic malignancies during pregnancy presents diagnostic and therapeutic challenges requiring an interdisciplinary approach. The most common hematologic neoplasias in pregnancy include Hodgkin’s disease, aggressive non-Hodgkin’s lymphoma and acute leukemia. A cure for the mother often depends on the consistent administration of intensive chemotherapy regimens. Whenever possible, treatment should be deferred until the second trimester after the completion of organogenesis. Chemotherapy during the first trimester can induce a spontaneous abortion or significantly increase the risk of congenital malformations. During the second and third trimester chemotherapy can be administered with reasonable safety although an increased fetal risk will remain. It is important to balance the potential fetal risk against the maternal risk of an untreated neoplasia.     

Leukämien – Modell für Neoplasien

Für die akuten und chronischen Leuk?mien wurde in den vergangenen Jahrzehnten eine erhebliche Prognoseverbesserung erreicht. Sie geh?ren nun zu den wenigen disseminierten malignen Erkrankungen, die durch Chemotherapie, z.T. in Kombination mit einer Knochenmark- (KMT) oder peripheren Stammzelltransplantation (PBSCT), heilbar sind. Die leichte Zug?nglichkeit der malignen Zellen aus Blut oder Knochenmark und die M?glichkeit zur ex vivo-Kultivierung und Charakterisierung bieten günstige Voraussetzungen für zellbiologische und molekulargenetische Grundlagenforschung zur Aufkl?rung der Pathogenese und zur Entwicklung neuer Ans?tze in Diagnostik und Therapie. Leuk?mien sind deshalb Erkrankungen, die als Modell für eine Vielzahl anderer Erkrankungen dienen k?nnen.