Thyroid Rosai-Dorfman disease with infiltration of IgG4-bearing plasma cells associated with multiple small pulmonary cysts

BMC Pulm Med. 2019 May 3;19(1):83. doi: 10.1186/s12890-019-0847-1.


Background: Rosai-Dorfman disease (RDD) is a rare histiocytosis which involves principally lymph nodes. Thyroid involvement in RDD is a very rare situation, and lung involvement is even rarer.

Case presentation: We report the case of a 46-year-old woman presenting a painless mass in the right side of the neck and subacute dyspnoea. Computerised tomography (CT) scans of the neck and thorax showed a large thyroid mass causing tracheal stenosis and multiple cystic lesions in both lungs. Subtotal thyroidectomy with a tracheal segment resection and histological analysis confirmed the diagnosis of nodal and extranodal (thyroid, tracheal and probably lung) Rosai-Dorfman disease (RDD) with the presence of increased numbers of IgG4-bearing plasma cells. Clinical, functional and radiological follow up 4 years after surgery without medical treatment did not show any disease progression.

Conclusions: This case report indicates a benign course of nodal RDD with thyroid and tracheal infiltration following surgical resection, association of typical histological signs of RDD (emperipolesis) with IgG4-related disease features, and that lung cysts might be a manifestation of RDD.

Keywords: Lungs cystic lesions; Non-Langerhans cell histiocytosis; Rosai-Dorfman disease.

Publication types

  • Case Reports

MeSH terms

  • Cysts / pathology
  • Diagnosis, Differential
  • Female
  • Histiocytosis, Sinus / pathology*
  • Histiocytosis, Sinus / surgery*
  • Humans
  • Immunoglobulin G / blood
  • Immunohistochemistry
  • Lung / pathology
  • Lymph Nodes / pathology*
  • Middle Aged
  • Plasma Cells / pathology
  • Thyroid Gland / growth & development
  • Thyroid Gland / pathology*
  • Thyroidectomy
  • Tomography, X-Ray Computed
  • Tracheal Stenosis / etiology


  • Immunoglobulin G