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Neuroendokrine Tumoren der Lunge

Diagnostik, therapeutische Möglichkeiten und aktuelle Registerdaten

Neuroendocrine tumors of the lung

Diagnosis, treatment options, current registry data

Zusammenfassung

Hintergrund

Die neuroendokrinen Tumoren der Lunge bilden in der aktuellen Klassifikation gemäß WHO (Weltgesundheitsorganisation) eine sehr heterogene Gruppe maligner pulmonaler Neoplasien mit entsprechend unterschiedlichen klinischen Verläufen. Das Spektrum reicht vom kleinzelligen Lungenkarzinom (SCLC) und großzelligen neuroendokrinen Karzinom (LCNEC) als sehr aggressiven, schnell metastasierenden Tumoren bis zu pulmonalen Karzinoiden, bei denen es sich, v. a. beim typischen Karzinoid, um gut differenzierte, langsam wachsende Tumoren handelt, die nur selten extrathorakal metastasieren. Diese Übersichtsarbeit beschäftigt sich mit der aktuellen Diagnostik, Klassifikation und Therapie der Untergruppe der pulmonalen Karzinoide.

Methoden

Neben einer selektiven Literaturrecherche in PubMed und Vergleich der Leitlinien werden ergänzend Daten von 137 Patienten aus dem von der Evangelischen Lungenklinik Berlin initiierten Register für pulmonale neuroendokrine Tumoren vorgestellt.

Ergebnisse

Die chirurgische Resektion ist die einzige kurative Therapieoption und wird in den aktuellen Leitlinien bei lokal und regional begrenzten Tumorstadien empfohlen. Bei der Behandlung von inoperablen oder metastasierten Tumorstadien sollte aufgrund von guten Überlebensraten dieser Patienten v. a. auf die Toxizität der verfügbaren Optionen geachtet werden.

Schlussfolgerung

Durch komplexe Resektionsverfahren kann häufig gesundes Lungenparenchym erhalten und eine Pneumonektomie vermieden werden, daher sollte die Therapie in spezialisierten Zentren erfolgen. Da es nur wenige hochwertige prospektive Studien gibt, stützen sich die Empfehlungen der Fachgesellschaften im Wesentlichen auf retrospektive Analysen und weisen in vielen Punkten keinen Konsens auf. Daher ist die Behandlung im Rahmen von Studien empfehlenswert.

Abstract

Background

In the current WHO (World Health Organization) classification, neuroendocrine tumors of the lung represent a very heterogeneous group of malignant pulmonary neoplasms with different clinical courses. At one end of the spectrum are very aggressive, rapidly metastasizing tumors (small cell lung carcinoma, large cell neuroendocrine carcinoma); at the other end are pulmonary carcinoids, which, especially the typical carcinoid, are well-differentiated, slow-growing tumors that rarely metastasize extrathoracically. This review focuses on the current diagnosis, classification, and therapy of the subgroup of pulmonary carcinoids.

Methods

In addition to a selective literature search in PubMed and comparison of current guidelines, data of 137 patients from the pulmonary neuroendocrine tumor registry initiated by the ELK Berlin Chest Hospital (Evangelische Lungenklinik Berlin) are presented.

Results

Surgical resection is the only curative treatment option and is recommended in current guidelines for locally and regionally limited tumor stages. In the treatment of inoperable or metastatic tumor stages, special attention should be paid to the toxicity of the available treatment options due to good survival rates of these patients.

Conclusion

Complex resection procedures can often preserve healthy lung parenchyma and avoid pneumonectomy; therefore, surgical treatment should be performed in specialized centers. Because only a few high-quality prospective studies are available, treatment recommendations of the scientific societies are mainly based on retrospective analyses and do not show a consensus on all aspects. Accordingly, we recommend participation in studies.

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Correspondence to F. Weigold.

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F. Weigold und G. Leschber geben an, dass kein Interessenkonflikt besteht.

Für diesen Beitrag wurden von den Autoren keine Studien an Menschen oder Tieren durchgeführt. Für die aufgeführten Studien gelten die jeweils dort angegebenen ethischen Richtlinien.

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Weigold, F., Leschber, G. Neuroendokrine Tumoren der Lunge. Onkologe 27, 900–910 (2021). https://doi.org/10.1007/s00761-021-00966-z

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Schlüsselwörter

  • Lungenchirurgische Verfahren
  • Karzinoid
  • Neuroendokrines Karzinom
  • Manschettenresektion
  • Tumormetastasierung

Keywords

  • Pulmonary surgical procedures
  • Carcinoid tumor
  • Neuroendocrine carcinoma
  • Sleeve resection
  • Neoplasm metastasis