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Reu, Simone und Huber, Rudolf M. (2017): Kleinzelliges Lungenkarzinom. Pathologie und Biologie. In: Onkologe, Bd. 23, Nr. 5: S. 340-346

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Abstract

Small cell lung cancer is an aggressive malignant neoplasm with a strong tendency to early and widespread metastastic lesions and is associated with a dismal prognosis. It has the strongest association with a smoking history of all subtypes of pulmonary carcinomas. Together with large cell neuroendocrine carcinoma of the lungs and pulmonary carcinoid tumors it comprises the pulmonary neuroendocrine tumors as a histogenetic group. It is defined by its characteristic morphology consisting of small cells with scant cytoplasm, neuroendocrine differentiation, high proliferative activity and tumor cell necrosis that is typically extensive. On morphological features it is indistinguishable from small cell carcinomas originating in other organs than the lungs. Inactivating alterations of the tumor suppressor TP53 and RB1 genes constitute the main and ubiquitous drivers of tumor development and growth. They generate a genomic instability of the tumor cells that together with abundant numbers of mutations induced by carcinogens of tobacco smoke add to the remarkable mutational load of this tumor type. The large numbers of genetic and epigenetic alterations hinder the identification of molecular targets for targeted therapy but may provide a basis for immune checkpoint inhibition. Small cell lung cancer is an undifferentiated tumor that can be clearly separated from other histological types of lung cancer by specific clinical, prognostic, biological and morphological features and for which there are still a restricted number of therapeutic options.

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