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Significance of Multiple Carcinoid Tumors and Tumorlets in Surgical Lung Specimens: Analysis of 28 Patients

¹Departments of Laboratory Medicine and Pathology ²Pulmonary and Critical Care Medicine ³Radiology, Mayo Clinic, Rochester, MN, Department of Pathology, University of Michigan, Ann Arbor, MI

aubry.mariechristine{at}mayo.edu

Abstract

BackgroundThe clinical significance of multiple carcinoid tumorlets in surgical lung specimens has not been systematically analyzed. We reviewed our experience to determine the range of clinical circumstances associated with this finding.

MethodsWe reviewed clinical records, available imaging, and pathology materials from patients evaluated at Mayo Clinic Rochester (1987-2000) with 2 or more carcinoid tumors or tumorlets in lung specimens.

ResultsTwenty-eight of 294 patients with diagnoses of carcinoid tumor or tumorlet had 2 lesions. Twenty-six (93%) were women; mean age was 65 years. Patients fell into three groups: multiple nodules (17), solitary lung nodules on pre-operative imaging (7), and airflow limitation (4). Approximately half of patients with multiple nodules had respiratory complaints; two had Cushing syndrome. Ten (58.8%) were suspected of having pulmonary metastases, including seven with previously diagnosed malignancies. Intrathoracic lymph node metastases were present in three patients, none of whom experienced recurrent disease. One patient had a carcinoid tumor resected 8 years later. Another developed extrathoracic metastases three years after presentation and was alive with disease two years later. Only one patient with airflow limitation had a syndrome resembling diffuse idiopathic pulmonary neuroendocrine cell hyperplasia.

ConclusionsOur series represents the largest compilation of multiple carcinoid tumors or tumorlets. Our analysis reveals that multiple carcinoid tumors or tumorlets occur most commonly in patients with multiple nodules resembling metastatic disease. Significant airflow limitation is rare. Long term survival is excellent although patients experience persistent disease.

Key Words: lung neoplasm • carcinoid tumor • tumorlets • neuroendocrine cell hyperplasia