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Management of pulmonary neuroendocrine tumors

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Abstract

Neuroendocrine tumors (NETs) of the lung are divided into 4 major types: small cell lung cancer (SCLC), large cell neuroendocrine carcinoma (LCNEC), atypical carcinoid (AC) or typical carcinoid (TC). Each classification has distinctly different treatment paradigms, making an accurate initial diagnosis essential. The inconsistent clinical presentation of this disease, however, makes this difficult. The objective of this manuscript is to detail the diagnosis and management of the well differentiated pulmonary carcinoid (PC) tumors. A multidisciplinary approach to work up and treatment should be utilized for each patient. A multimodal radiological work-up is used for diagnosis, with contrast enhanced CT predominantly utilized and functional imaging techniques. A definitive diagnosis is based on tissue findings. Surgical management remains the mainstay of therapy and can be curative. In those with advanced disease, medical treatments consist of somatostatin analog (SSA) therapy, targeted therapy, chemotherapy or peptide receptor radionuclide therapy. SSAs are the standard of care in those with metastatic NETs, using either Octreotide long acting repeatable (LAR) or lanreotide as reasonable options, despite a scarcity of prospective data in PCs. Targeted therapies consist of everolimus which is approved for use in PCs, with various studies showing mixed results with other targeted agents. Additionally, radionuclide therapy may be used and has been shown to increase survival and to reduce symptoms in some studies. Prospective trials are needed to determine other strategies that may be beneficial in PCs as well as sequencing of therapy. Successful diagnosis and optimal treatment relies on a multidisciplinary approach in patients with lung NETs. Clinical trials should be used in appropriate patients.

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Authors and Affiliations

  1. Ochsner Medical Center – Kenner, 200 West Esplanade Ave, Suite 200, Kenner, LA, 70065, USA

    Robert A. Ramirez, Juan Gimenez & Katharine E. H. Thomas

  2. University of Kentucky Medical Center, Lexington, KY, 40536, USA

    Aman Chauhan

  3. Ochsner Clinical School, The University of Queensland School of Medicine, New Orleans, LA, 70121, USA

    Ioni Kokodis

  4. Louisiana State University Health Sciences Center, New Orleans, LA, 70112, USA

    Brianne A. Voros

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  1. Robert A. Ramirez
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Robert A. Ramirez, DO serves as a consultant for Ipsen Biophamaceuticals Inc. and Bio Theranostics Inc. as well as a speaker for Merck & Co. Inc., Genetech, Astra Zeneca and Ipsen Biopharmaceuticals. No other authors have conflicts of interest to disclose.

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Ramirez, R.A., Chauhan, A., Gimenez, J. et al. Management of pulmonary neuroendocrine tumors. Rev Endocr Metab Disord 18, 433–442 (2017). https://doi.org/10.1007/s11154-017-9429-9

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  • DOI: https://doi.org/10.1007/s11154-017-9429-9

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