Multifokale neuroendokrine Lungentumoren: Entstehung, Diagnostik und Behandlung

Zusammenfassung

Selten werden multifokale neuroendokrine Lungentumoren diagnostiziert. Im Thorax-CT zeigen sich meist multiple Lungenherde unterschiedlicher Größe. Es ist anzunehmen, dass sich multifokale neuroendokrine Lungentumoren ursprünglich aus diffusen idiopathischen pulmonalen neuroendokrinen Zellhyperplasien (DIPNECH) entwickeln. Darunter versteht man Zellverbände von physiologisch im Bronchialsystem vorhandenen neuroendokrinen Zellen, die wachsen. Durchbrechen die Zellvermehrungen die bronchiale Basalmembran, so liegt bei einer Größe ≤ 5 mm ein Tumorlet, ab einer Größe von 5 mm ein Karzinoidtumor vor. Die Proliferationsgeschwindigkeit der Zellhyperplasien scheint unterschiedlich zu sein. Da ein nicht unerheblicher Teil der Patienten völlig asymptomatisch ist, werden die multifokalen neuroendokrinen Lungentumoren zufällig diagnostiziert. Daneben gibt es jedoch Patienten, die über Luftnot, Einschränkung der Leistungsfähigkeit und Husten klagen. Die Lungenfunktion kann dabei schon eingeschränkt sein. In diesen Fällen zeigt eine HR-CT-Thorax-Untersuchung häufig außer den Lungenherden zusätzlich noch eine peribronchiale Fibrose oder Bronchiektasen. Eine Bronchoskopie ist in der Regel nicht wegweisend. Die chirurgische thorakoskopische Lungenbiopsie gilt als diagnostischer Goldstandard. Typischerweise zeigt sich histologisch eine Mischung aus Zellhyperplasien, Tumorlets und Karzinoidtumoren. Über die Behandlung multifokaler neuroendokriner Tumoren gibt es keinen Konsens. Ausgehend von der Klinik und den Befunden im HR-CT des Brustkorbes entwickelten wir ein stufenweises Vorgehen, das sich an dem Erfolg der einzelnen therapeutischen Maßnahmen orientiert. Die günstigste Prognose haben Betroffene ohne klinische Beschwerden, bei denen alle Lungenherde unter 5 mm sind. In diesen Fällen liegt das 5-Jahres-Überleben über 90 %.

Abstract

Multifocal neuroendocrine lung tumour is a rare diagnosis. Multiple lung foci of different sizes are usually apparent on chest CT scans. It is assumed that multifocal neuroendocrine lung tumours originally develop from diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH). This results in cell aggregations formed by proliferation of neuroendocrine cells that are already physiologically present in the bronchial system. If these cell proliferations break through the bronchial basement membrane, they are considered to constitute tumourlets if they measure ≤ 5 mm and carcinoid tumours if they are larger than 5 mm. The speed of proliferation of the cell hyperplasias appears to vary. Many of the patients are completely asymptomatic, the multifocal neuroendocrine lung tumours being diagnosed by chance. However, other patients complain of breathlessness, reduced physical capacity and cough. There may also be reduction of lung function. In these cases, chest HRCT often reveals peribronchial fibrosis or bronchiectasis in addition to the lung foci. Bronchoscopy is usually not helpful. Surgical lung biopsy is considered to be the diagnostic gold standard. Histological examination typically shows a mixture of cell hyperplasias, tumourlets and carcinoid tumours. There is no consensus on the treatment of multifocal neuroendocrine tumours. Taking the clinical situation and the chest HRCT findings as our starting point, we developed a stepwise approach that is guided by the success of the individual therapeutic procedures. The most favourable prognosis is found in affected people without clinical symptoms whose lung foci all measure less than 5 mm. In these cases the 5-year survival rate is over 90 %.

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