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Treatment guidelines address rare pulmonary tumors

The European Neuroendocrine Tumor Society has released an expert consensus document with recommendations for best practice management of pulmonary neuroendocrine tumors including typical and atypical carcinoids.

Pulmonary carcinoids (PC) are rare tumors with an age-adjusted incidence rate ranging from 0.2 to 2 per 100,000 people annually in the United States and in Europe.

In compiling the guidelines, the panel systematically searched PubMed for terms such as pulmonary neuroendocrine tumors (NET), bronchial carcinoid tumors, pulmonary typical carcinoid, and pulmonary carcinoids and treatment. After excluding in vitro and animal studies, the group identified 178 articles with the strongest level of evidence to influence the guidelines.

The guidelines included pathological, diagnostic, surgical and medical therapy recommendations for best practice management.

Highlights from the recommendations include:

• Pathology reports on NETs should be shared by the multidisciplinary team of oncologists, radiologists, nuclear medicine physicians, surgeons, pneumologists and endocrinologists, where pathologists contribute to the clinical decision-making process;
• Separation of typical carcinoids (TC) and atypical carcinoids (AC) requires a surgical specimen. TC and AC cannot be reliably distinguished from each other in small biopsy and cytology;
• Bronchoscopy may be required for the staging and assessment of central airway tumors preoperatively;
• Flexible bronchoscopy is preferable, however in patients at high risk for bleeding, rigid bronchoscopy may be preferred for obtaining biopsy specimens;
• In the case of localized disease, the surgical techniques of choice are lobectomy or sleeve resection;
• There is no consensus on adjuvant therapy in PCs after complete resection. There might be consideration in patients with AC of high proliferative index; and
• After primary surgery, patients with AC and TC should undergo long-term follow-up.

Disclosure: Caplin reports advisory board and research funding from Ipsen, Lexicon and Novartis Please see the full study for a list of all other authors’ relevant financial disclosures.