Zusammenfassung
Das CUP-Syndrom („carcinoma of unknown primary“) ist definiert als Metastasierung eines durch klinische Routineuntersuchungen (inklusive Histologie) nicht lokalisierbaren Primärtumors. CUP-Patienten stellen den behandelten Arzt daher vor ein schwieriges diagnostisches und therapeutisches Problem. Patienten mit CUP machen an spezialisierten Zentren bis zu 10% aller Tumorpatienten aus. Die Besonderheiten dieser Entität bestehen im atypischen Metastasierungsmuster und einer ungewöhnlichen Wachstumskinetik. 60% der CUP-Patienten haben Metastasen eines Adenokarzinoms. Wichtig ist, dass möglichst schnell und eindeutig potenziell kurative (maligne Lymphome oder extragonadale Keinzelltumoren) oder zumindest gut behandelbare Tumormanifestationen (z. B. neuroendokrine Tumoren) identifiziert und damit einer adäquaten Therapie zugeführt werden. Ansonsten stehen je nach Ausdehnung und Lokalisation chirurgische, strahlentherapeutische und systemische Therapiemaßnahmen zur Verfügung. Für die große Mehrheit der Patienten mit CUP-Syndrom ist die Prognose mit einer Überlebenszeit von 4–11 Monaten sehr ungünstig. Populationsbasierte Analysen zeigen, dass nur 15% der Patienten ein Jahr nach der Diagnose eines CUP leben.
Abstract
Despite the growing array of sophisticated diagnostic tools for establishing a diagnosis of human neoplasia, 2–6% of all cancer patients still present metastatic cancer of which detailed investigations fail to identify the primary anatomic site. The site is found in less than 25% of patients before death and frequently goes undiscovered at post-mortem examination. At the time of first diagnosis with carcinoma of unknown primary site, usually more than 80% of patients present with dissemination. Prognosis depends on the site(s) involved and is unaffected by whether the primary site is ever found. Node dissection may be curative for patients with metastases to peripheral lymph nodes. Objective long-term response is possible in combination with chemotherapy in patients with small-cell malignancies, peritoneal carcinomatosis (in women), or poorly differentiated carcinomas involving external lymph nodes, mediastinum, or retroperitoneum but without metastases to viscera or bone. Toxic therapies are recommended only for palliation of symptoms and maintaining quality of life support in patients with good functional status. Patients should be encouraged to participate in clinical trials for novel therapies.
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Sendler, A., Strumberg, D. & Tannapfel, A. Metastasen eines unbekannten Primärtumors. Chirurg 79, 689–696 (2008). https://doi.org/10.1007/s00104-008-1553-3
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DOI: https://doi.org/10.1007/s00104-008-1553-3