Kleinzelliges neuroendokrines Karzinom des Kopf-Hals-Bereichs: eine Übersichtsarbeit und Fallserie

Zusammenfassung

Einleitung Kleinzellige neuroendokrine Karzinome (KNK) des Larynx sind eine seltene Tumorentität mit schlechter Prognose bei einer 5-Jahres-Überlebensrate nach Standardtherapie mit primärer Radiochemotherapie (pRCT) von 5 %.

Methoden Es erfolgte eine systematische Literaturrecherche auf PubMed mit den Suchbegriffen „small cell neuroendocrine carcinoma“ und „head and neck“ sowie die Aufarbeitung von Patientenfällen aus unserer Klinik.

Ergebnisse Die Recherche ergab keine großen randomisierten kontrollierten Studien zur Standardtherapie. Bisherige Therapiestrategien basieren auf den Erfahrungen bei kleinzelligen Karzinomen der Lunge. 0,5 % aller KNK treten im Kopf-Hals-Bereich auf. In unserer Klinik wurden in 12 Jahren 9 KNK diagnostiziert, 2 davon mit laryngealer Manifestation. Wir berichten über einen 29-jährigen Patienten mit Erstdiagnose (ED) eines Larynx-KNK im Frühjahr 2018. Im Staging zeigten sich zervikale Lymphknotenmetastasen, eine Fernmetastasierung wurde ausgeschlossen. Es erfolgte eine pRCT mit Cisplatin/Etoposid mit darauffolgender Komplettremission. Im Re-Staging 6 Monate nach ED zeigten sich Metastasen-suspekte Lungenherde. Unter 6 Zyklen palliativer Systemtherapie mit Cyclophosphamid, Adriamycin und Vincristin kam es zu einer partiellen Remission. Nach 12 Monaten erfolgte bei Progress die Therapieumstellung auf den PD-1-Antikörper Nivolumab. Der Patient verstarb 22 Monate nach ED an einer tumorbedingten Massenblutung mit Verlegung der Atemwege.

Schlussfolgerung Bisher existieren keine Studienergebnisse über den Einsatz von Nivolumab als Third-Line-Therapie bei KNK. Die Analyse einer NTRK-Fusion (neurotrophe Tyrosin-Rezeptor-Kinase) oder einer Folatrezeptor-Expression sollte erwogen werden zur Evaluation einer Tropomyosin-Rezeptor-Kinase-Inhibitor- oder einer Radionuklidtherapie.

Abstract

Introduction Small cell neuroendocrine carcinoma (SCNC) of the larynx is a rare tumor entity with a 5-year overall survival (OS) of only 5 % after treatment with chemoradiotherapy.

Methods A systematic review of the literature was performed for “SCNC” and “SCNC in head and neck”. Our hospital’s own electronic patient file database was investigated for patients diagnosed with a SCNC over the last 12 years.

Results The effectiveness of chemoradiotherapy in SCNC is still unclear since randomized clinical trials are missing for the evaluation of standard of care treatment. Common therapy approaches are based on experiences with small cell lung cancer. 0.5 % of all SCNC occur in the head and neck region. In the last 12 years, we diagnosed 9 patients with SCNC, two of which were located in the larynx. Exemplarily, we report the case of a 29-year-old male with the initial diagnosis of a SCNC of the larynx with concurrent lymph node metastasis. This case is particularly interesting due to the young age at disease onset and the lack of major risk factors. Treatment was modified to nivolumab due to progressive disease after treatment with chemoradiotherapy. After an OS of 22 months, the patient deceased due to a tumor-associated major bleeding with airway obstruction.

Conclusion So far there are no clinical reports evaluating the use of nivolumab in third-line-therapy of SCNC. NTRK fusion (neurotrophic tyrosine receptor kinase gene fusion) or the folate receptor expression analysis should be considered to evaluate the potential use of a tropomyosin receptor kinase inhibitor or a folate receptor targeting therapy.

Schlüsselwörter

kleinzelliges neuroendokrines Karzinom - Larynx - HNO

Key words

neuroendocrine small cell carcinoma - head and neck - larynx

Publication History

Article published online:
26 January 2022

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