Simplified 2-tier histologic grading system accurately predicts outcomes in goblet cell carcinoid of the appendix

Summary Goblet cell carcinoid (GCC) is a rare appendiceal malignancy with both neuroendocrine and glandular features. Clinical outcomes of patients with GCC vary widely and a histology-based 3-tiered prognostic scheme has been previously suggested; however, this scheme is subjective and challenging...

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Bibliographic Details
Published in:Human pathology 2015-12, Vol.46 (12), p.1881-1889
Main Authors: Lee, Lawrence H., MD, McConnell, Yarrow J., MD, Tsang, Erica, MD, Zerhouni, Siham, MD, Speers, Caroline, BA, Kennecke, Hagen, MD, Schaeffer, David F., MD
Format: Article
Language:English
Subjects:
Adenocarcinoid
Adenoneuroendocrine
Adult
Aged
Appendiceal Neoplasms - mortality
Appendiceal Neoplasms - pathology
Appendicitis
Archives & records
Carcinoid Tumor - mortality
Carcinoid Tumor - pathology
Classification
Crypt cell carcinoma
Demographics
Female
Goblet cell carcinoid
Histopathologic grading
Humans
Kaplan-Meier Estimate
Lymphatic system
Male
Medical prognosis
Middle Aged
Mixed adenoneuroendocrine carcinoma
Neoplasm Grading - methods
Pathology
Patients
Prognosis
Proportional Hazards Models
Survival analysis
Tumors
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Summary:Summary Goblet cell carcinoid (GCC) is a rare appendiceal malignancy with both neuroendocrine and glandular features. Clinical outcomes of patients with GCC vary widely and a histology-based 3-tiered prognostic scheme has been previously suggested; however, this scheme is subjective and challenging to apply in day-to-day practice. We sought to construct a simplified and prognostic grading system based on objective histologic features with specific criteria. A continuous population-based cohort of GCC with clinical outcome data and archival tissue available for review was extracted from regional databases. For the 78 patients with confirmed appendiceal GCC, specific histologic features, including cytologic atypia, peritumoral stromal desmoplasia, and solid growth pattern, were recorded, and a scoring system was devised, which separates patients with GCC into low-grade (n = 55; 71%) or high-grade (n = 23; 29%) histology. Correspondingly, clinical follow-up data show good prognosis in those with low-grade histology with median and 10-year overall survival of 51.0 months and 80.5%, respectively, whereas those with high-grade histology have a poor prognosis with median and 10-year overall survival of 16.5 months ( P = .006) and 0% ( P < .001), respectively. Multivariate Cox proportional hazard modeling demonstrates that this 2-tier histologic system remains predictive of overall survival when controlled for TNM clinicopathological stage. These data show that a simple and objective histologic scoring system separates GCC into low- and high-grade histology with divergent clinical outcomes.
ISSN:0046-8177
1532-8392
DOI:10.1016/j.humpath.2015.08.005