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Case Reports
. 2019 May 3;19(1):83.
doi: 10.1186/s12890-019-0847-1.

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

Pietro Gianella  1 , Nicolas Dulguerov  2 , Grégoire Arnoux  3 , Marc Pusztaszeri  3 , Jörg D Seebach  4
Affiliations
Case Reports

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

Pietro Gianella et al. BMC Pulm Med. .

Abstract

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.

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Conflict of interest statement

Ethics approval and consent to participate

Not applicable.

Consent for publication

a written consent from patient was obtained to publish this case report.

Competing interests

P. G. has nothing to disclose, N. D. has nothing to disclose, G.A. has nothing to disclose, M.P. has nothing to disclose, J.D.S. has nothing to disclose.

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Figures

Fig. 1
Fig. 1
Neck CT scan (axial reconstruction) showing a large right sided thyroid mass (asterisk) which caused marked tracheal deviation and stenosis (a). Thorax CT scan (coronal reconstruction) showing multiple cystic lesions with thin wall in both lungs (arrows) (b)
Fig. 2
Fig. 2
Surgical specimen showing a poorly delimited infiltrative whitish thyroid mass with extension to the adjacent tracheal structure. Star indicates the luminal surface of the trachea
Fig. 3
Fig. 3
a Overview of one representative fragment showing a dense fibro-inflammatory process involving the thyroid and the tracheal wall (boxed area). The star indicates residual thyroid tissue. b Detail view of the boxed area in (A) showing predominant histiocytic infiltrates along with numerous emperipolesis figures, i.e. histiocytes engulfing neutrophil granulocytes and lymphocytes (arrow head). This combination is a prominent feature of Rosai-Dorfman disease. H&E staining; original magnification: A × 20; B × 400
Fig. 4
Fig. 4
Detailed view of the immunostaining results showing histiocytes strongly positive for CD68 (a) and S100 (b), negative staining for CD1a (c), and increased numbers of IgG4-bearing plasma cells (d). Original magnification A-D × 400
See this image and copyright information in PMC

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