Mucoepidermoid carcinoma of the head and neck: CRTC1/3 MAML 2 translocation and its prognosticators

Eur Arch Otorhinolaryngol. 2022 May;279(5):2573-2581. doi: 10.1007/s00405-021-07039-2. Epub 2021 Aug 17.

Abstract

Purpose: Mucoepidermoid carcinoma (MEC) of the head and neck is a prevalent malignant salivary gland tumour with a reported good outcome. The aim of this study was to report the outcome in our centre.

Methods: A retrospective chart analysis with survival analyses was performed combined with fluorescence in situ hybridization (FISH) analysis to assess CRTC1/3 MAML 2 fusion gene presence.

Results: Sixty-four cases of MEC were identified. Median age at presentation was 51.4 years with a predominance for parotid gland involvement. Five, 10- and 20- year disease-free survival was 98%, 90% and 68%, respectively. Overall survival was 94%, 90% and 64%, respectively. Local recurrence was seen up to 14 years after primary diagnosis; distant metastases were diagnosed up to 17 years later. The overall recurrence rate was less than 20 per cent. CRTC1/3 MAML 2 fusion gene presence showed no survival benefit.

Conclusion: MEC of the head and neck has a favorable outcome with the exception of high-grade MEC. PNI and nodal involvement are not rare. CRTC1/3 MAML 2 fusion gene presence showed no survival benefit. The tendency for late onset of loco-regional and distant recurrence should not be underestimated.

Keywords: Mucoepidermoid carcinoma; Parotid gland; Prognosticators; Salivary glands; Translocation.

MeSH terms

  • Carcinoma, Mucoepidermoid* / genetics
  • Carcinoma, Mucoepidermoid* / pathology
  • DNA-Binding Proteins / genetics
  • Humans
  • In Situ Hybridization, Fluorescence
  • Nuclear Proteins / genetics
  • Retrospective Studies
  • Salivary Gland Neoplasms* / pathology
  • Trans-Activators / genetics
  • Transcription Factors / genetics
  • Translocation, Genetic

Substances

  • CRTC1 protein, human
  • DNA-Binding Proteins
  • Nuclear Proteins
  • Trans-Activators
  • Transcription Factors