Introduction <p>Salivary gland intraductal papillary mucinous neoplasm (SG IPMN) is a recently described entity arising predominately from intraoral minor salivary glands, characterised by a low-grade papillary cystic proliferation of mucinous columnar cells, so-named due to its resemblance to the pancreatic duct counterpart [<CitationRef CitationID="CR1">1</CitationRef>]. It is currently mentioned in the World Health Organization (WHO) Classification of Tumours (5th edition) salivary gland tumours introduction as a lesion with low-grade mucinous morphology that shares frequent <i>AKT1</i> mutations with mucinous adenocarcinoma, hence it is thought to represent a low-grade salivary gland mucinous adenocarcinoma and is currently subsumed into this entity [<CitationRef AdditionalCitationIDS="CR2" CitationID="CR1">1</CitationRef>–<CitationRef CitationID="CR3">3</CitationRef>]. However, the classification of the neoplasm remains a controversial issue, with the WHO noting that it is still not established whether SG IPMN should be classified separately or within the mucinous adenocarcinoma spectrum as a potential precursor [<CitationRef CitationID="CR2">2</CitationRef>].</p> Methods <p>We describe an unusual case of a minor salivary gland papillary mucinous neoplasm presenting in a novel location (subglottic larynx) with a novel mutational driver for this entity (<i>GNAS</i> R844H mutation), and briefly review the literature surrounding SG IPMN, its classification and related tumours.</p>

错误:搜索内容不能为空,请输入英文关键词
错误:关键词超出字数限制,请精简
高级检索

Subglottic Laryngeal Salivary Gland Intraductal Papillary Mucinous Neoplasm with GNAS Mutation: A Case Report and Review of the Literature

  • Benjamin Champion,
  • Benjamin Ryan,
  • Chady Sader,
  • Connull Leslie,
  • Chris Van Vliet

摘要

Introduction

Salivary gland intraductal papillary mucinous neoplasm (SG IPMN) is a recently described entity arising predominately from intraoral minor salivary glands, characterised by a low-grade papillary cystic proliferation of mucinous columnar cells, so-named due to its resemblance to the pancreatic duct counterpart [1]. It is currently mentioned in the World Health Organization (WHO) Classification of Tumours (5th edition) salivary gland tumours introduction as a lesion with low-grade mucinous morphology that shares frequent AKT1 mutations with mucinous adenocarcinoma, hence it is thought to represent a low-grade salivary gland mucinous adenocarcinoma and is currently subsumed into this entity [13]. However, the classification of the neoplasm remains a controversial issue, with the WHO noting that it is still not established whether SG IPMN should be classified separately or within the mucinous adenocarcinoma spectrum as a potential precursor [2].

Methods

We describe an unusual case of a minor salivary gland papillary mucinous neoplasm presenting in a novel location (subglottic larynx) with a novel mutational driver for this entity (GNAS R844H mutation), and briefly review the literature surrounding SG IPMN, its classification and related tumours.