Demographic features and changing incidence and presentation of Merkel cell carcinoma in a retrospective single-center c

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ORIGINAL PAPER

Demographic features and changing incidence and presentation of Merkel cell carcinoma in a retrospective single‑center case series Soha Ghanian1 · Michelle Koh1 · Joanna Walker1 Received: 11 June 2020 / Revised: 11 June 2020 / Accepted: 12 September 2020 © Springer-Verlag GmbH Germany, part of Springer Nature 2020

Abstract Merkel cell carcinoma (MCC) is an uncommon aggressive cutaneous neuroendocrine tumor with high mortality. The purpose of this study is to describe the clinical correlates of MCC and identify changing trends over time using a retrospective chart review of 113 cases in a single tertiary center pathology database from 1997 to 2015. MCC occurred mostly in the elderly (median age 77 years), more often in males (n = 60, 53%), was most commonly located on the head and neck region (n = 63, 56%), and most commonly presented on the left side of the body (n = 59, 52%). Overall, there was an increase in MCC diagnoses per year from 1997 to 2015, with 2.3 times more cases diagnosed in the last 3 years of the study period than in the first 3 years. Furthermore, cases diagnosed after 2008 were more likely to occur in younger patients, less than or equal to 77 years of age (OR 2.32, 95% CI 1.08, 4.97, p = 0.04). The median age of diagnosis among females was 76 years and among males was 74 years. There was no change in the overall age of diagnosis among men and a decrease in age of diagnosis among females during the study. There was an overall increase in the MCC cases diagnosed among women, and a slight increase in the proportion of females affected by MCC throughout the course of the study. This retrospective case series elucidates MCC epidemiologic characteristics and describes how incidence, sex, and age at diagnosis have changed over the course of the study. Keywords  Merkel cell carcinoma · Age · Laterality · Epidemiology

Introduction

Materials and methods

Merkel cell carcinoma (MCC) is a rare, aggressive cutaneous neuroendocrine malignancy with an estimated 1600 new cases yearly and an increased incidence with age [1]. MCC is more common on the head and neck, among elderly Caucasian males, and in those with a history of chronic sun exposure, skin cancer, or immunosuppression [2]. MCC is particularly aggressive with associated high risk of metastasis, recurrence and 33% mortality rate [3]. Metastasis typically presents in the draining lymph node with subsequent spread to distant lymph nodes, lungs, liver, and bones [4]. Prior studies have reported an increase in MCC incidence by age and patient sex over time [5, 6]. The aim of this study is to investigate changes in median age of diagnosis by patient sex and changes in laterality of tumors over time.

A retrospective chart review of 117 cases of histologically diagnosed MCC between 1997 and 2015 were identified using a pathology database for the Lifespan/Rhode Island Hospital system, which encompasses a tertiary care center and several sites across the state. The term “Merkel” was included in either the final diagnosis or in body the path