Follicular dendritic sarcoma masquerading as fibrosing mediastinitis

BMJ Case Rep. 2017 Jun 13:2017:bcr2016218889. doi: 10.1136/bcr-2016-218889.

Abstract

Fibrosing mediastinitis (FM) is a rare disorder resulting from abnormal immunological-mediated fibro-proliferative reaction in the mediastinum. Here, we describe a case of a 46-year-old female with an incidentally found 11×9 cm posterior mediastinal mass. Multiple biopsies of this unresectable, 18-fluorodeoxyglucose avid mass revealed marked fibrosis without any evidence of malignancy, suggesting idiopathic fibrosing mediastinitis as our initial diagnosis. Multiple interventions including a trial of steroids, fluconazole, and azathioprine to target fibrosing mediastinitis were not successful. Repeat biopsy was consistent with primary mediastinal follicular dendritic cell sarcoma. The manuscript highlights the heightened need for suspecting occult malignancies in cases of FM presenting with an indeterminate cause.

Keywords: Cardiothoracic surgery; Pathology; Surgical oncology.

Publication types

  • Case Reports

MeSH terms

  • Dendritic Cell Sarcoma, Follicular / diagnosis*
  • Dendritic Cell Sarcoma, Follicular / diagnostic imaging
  • Dendritic Cell Sarcoma, Follicular / drug therapy
  • Dendritic Cell Sarcoma, Follicular / pathology
  • Diagnosis, Differential
  • Fatal Outcome
  • Female
  • Humans
  • Mediastinitis / diagnosis
  • Mediastinum*
  • Middle Aged
  • Positron Emission Tomography Computed Tomography
  • Sclerosis / diagnosis

Supplementary concepts

  • Mediastinal Fibrosis