Clinical Presentation and Outcome of Sinonasal Extraosseous Plasmacytoma in Denmark: A Nationwide Cohort From 1980 to 2017

Patrick René Gerhard Eriksen*, Elham Khakbaz, Erik Clasen-Linde, Peter de Nully Brown, Laura Haunstrup, Mette Christoffersen, Peter Asdahl, Troels Møller Thomsen, Jonas Gerner-Rasmussen, Steffen Heegaard, Christian von Buchwald

*Corresponding author for this work


OBJECTIVES: Extraosseous plasmacytoma (EOP) is a rare plasma cell neoplasm that tends to convert to plasma cell myeloma (PCM) in about 11% to 35% of cases. It has a predilection for the upper respiratory tract, prototypically affecting the nasal cavity and paranasal sinuses. Contemporary first-line treatment is radiotherapy, with more recent studies showing an added benefit of combining radiation with surgery. In this cohort study, we aimed to examine clinical presentation, treatment, and prognosis for all patients nationwide from 1980 through 2017. Furthermore, we determined the size and extension of tumors, investigating the rate at which minimally invasive surgery would have been possible.

METHODS: Patients were found in the national pathology registry, and all biopsies were collected for pathology review by a hematopathologist. We performed survival statistics for overall survival (OS), progression-free survival (PFS), and the cumulative incidence of conversion to PCM.

RESULTS: Twenty-three patients were included. The median age was 65, and patients were primarily men (78%). Tumors were located in either the nasal cavity (57%), maxillary sinus (39%), or sphenoid sinus (4%). In most cases, the tumor was <5 cm (65%) without extension to adjacent structures (60%). The national incidence was 0.02/100,000 person-years, the median symptom duration until diagnosis was 5 months, and none of the patients presented with contiguous spread to regional lymph nodes. Stand-alone radiotherapy was the predominant treatment (61%). In the entire cohort, one patient died from the initial disease, and six patients died from either relapse of EOP or PCM. The 5-year OS, PFS, and conversion rate to PCM were 78%, 56%, and 23%, respectively.

CONCLUSION: SN-EOP responds well to radiotherapy, but relapse and conversion to PCM were not uncommon and entailed a poor prognosis. Most tumors were endoscopically resectable and non-invasive, making the majority of tumors suitable for surgery as an addition to radiation.

Original languageEnglish
JournalAnnals of Otology, Rhinology and Laryngology
Issue number10
Pages (from-to)1186-1193
Number of pages8
Publication statusPublished - Oct 2023


  • endoscopic sinus surgery
  • extraosseous plasmacytoma
  • radiotherapy
  • sinonasal
  • Prognosis
  • Humans
  • Neoplasm Recurrence, Local
  • Male
  • Denmark/epidemiology
  • Maxillary Sinus/pathology
  • Paranasal Sinus Neoplasms/epidemiology
  • Aged
  • Nose Neoplasms/epidemiology
  • Retrospective Studies
  • Plasmacytoma/therapy
  • Cohort Studies
  • Multiple Myeloma


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