Abstract

Signet ring cell adenocarcinomas (SRCCs) are a rare histological subtype of adenocarcinomas with a poor prognosis, typically due to advanced disease at diagnosis. A signet ring cell, mimicking its moniker, contains abundant intracytoplasmic mucin that pushes the nucleus to the periphery. In these cancers, this cell feature comprises more than 50% of the tumor. Despite predilection for the gastrointestinal tract, and in particular the stomach, primary SRCCs are also described in other sites, typically in case reports. This literature, however, lacks a standardized overview of the SRCC disease entity. Using a retrospective cohort approach, we summarize the clinicodemographic and mortality outcomes of SRCCs in thirteen primary sites, comprising 95% of all SRCCs in the Surveillance, Epidemiology, and End Results Program (SEER), a population-level cancer database covering nearly one-third of the United States population. SRCCs general trends compared to matching nonvariant adenocarcinomas are earlier age of onset, with initial presentation favoring higher rates of regional or distant disease presentation and poor tumor differentiation. After multivariable analysis, SRCCs typically have worse overall survivals, but substantial variances exist depending on tumor location. Identifying SRCCs at earlier disease stages is likely the single most important intervention to improving outcomes for these patients.

Highlights

  • IntroductionSignet ring cell adenocarcinomas (SRCCs) have been described in the literature since the 1950s, with most initial papers describing these cancers in the colon and urinary bladder [1,2]

  • Overview of Signet Ring Cell AdenocarcinomasSignet ring cell adenocarcinomas (SRCCs) have been described in the literature since the 1950s, with most initial papers describing these cancers in the colon and urinary bladder [1,2]

  • SRCCs are distinct from mucinous adenocarcinomas in that in the latter greater than 50% of the tumor consists of extracellular mucin [5]

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Summary

Introduction

Signet ring cell adenocarcinomas (SRCCs) have been described in the literature since the 1950s, with most initial papers describing these cancers in the colon and urinary bladder [1,2]. These cancers have the appearance of a signet ring. A signet ring has a flattened surface at the top of the ring with an engraved or raised symbol, historically used to stamp a seal into correspondence These cells acquire a signet cell morphology due to accumulation of abundant mucin in cytoplasm, leading to nuclei dislocation to the periphery [3] (Figure 1). SRCCs are distinct from mucinous adenocarcinomas in that in the latter greater than 50% of the tumor consists of extracellular mucin [5]

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