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Bilateral Painless Cervical Lymphadenopathy in a Child

Educational Objective
Based on this clinical scenario and the accompanying image, understand how to arrive at a correct diagnosis.
1 Credit CME

A child younger than 10 years presented with an 8-week history of bilateral cervical lymphadenopathy unresponsive to a 10-day course of Augmentin (GlaxoSmithKline). The patient’s parents denied any associated fevers, chills, night sweats, sore throat, abdominal pain, or weight changes. Medical history was noncontributory.

Physical examination showed bilateral, mobile, nontender cervical lymphadenopathy without erythema or swelling. Tonsils were symmetric and not enlarged. There was no noticeable thyromegaly or nodules. Laboratory testing was significant for an elevated erythrocyte sedimentation rate (37 mmol/h). Ultrasonography demonstrated cervical lymphadenopathy bilaterally with the largest node measuring 3.8 cm in maximum dimension. Lymph node needle core biopsy demonstrated increased histiocytes and numerous plasma cells on tissue section (Figure, A). Scattered neutrophils and rare giant cells were also present. Findings of Grocott methenamine silver, acid-fast, and Warthin-Starry stains were negative. The CD-68 immunostain showed numerous histiocytes. The specimen stained positive for CD-30 and S-100 immunostains showing emperipolesis (Figure, B and C). Findings of a CD1a stain were negative. Lymphoid markers by flow cytometry showed a polyclonal mixed B-cell and T-cell population.

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D. Rosai-Dorfman disease

The causes of cervical lymphadenopathy in a child include infection, autoimmune, reactive processes, and cancer. This patient’s 8-week history of lymphadenopathy and lack of clinical symptoms make infectious mononucleosis unlikely. Furthermore, tuberculosis, cat-scratch disease, and fungal infections were ruled out with negative acid-fast, Warthin-Starry, and Grocott methenamine silver staining results, respectively. Lack of distinct elongated, folded, or grooved nuclei and eosinophilic infiltrate, and lack of CD1a staining makes Langerhans cell histiocytosis unlikely. Reactive histiocytosis shows S-100 negativity, unlike that found in the present patient.1

Rosai-Dorfman disease (RDD), also known as sinus histiocytosis with massive lymphadenopathy, was first described in 1969.1 Much of our current understanding of the disease comes from case studies and original histological descriptions by Drs Rosai and Dorfman. Rosai-Dorfman disease classically presents with lymph node enlargement. The most common sites for nodal involvement include cervical (87%), inguinal (26%), axillary (24%), and mediastinal (15%) lymph nodes. Extranodal involvement occurs in up to 43% of patients, most commonly involving the skin, nasal cavity, and paranasal sinuses.1 Most patients are otherwise asymptomatic, although some cases have reported fever. Erythrocyte sedimentation rate is frequently elevated, as seen in this patient.2

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Article Information

Corresponding Author: Sima Amin, BS, Texas A&M College of Medicine, 8447 Bryan Rd, Bryan, TX 77807 (samin@medicine.tamhsc.edu).

Published Online: July 19, 2018. doi:10.1001/jamaoto.2018.1579

Conflict of Interest Disclosures: All authors have completed and submitted the ICMJE Form for Disclosure of Potential Conflicts of Interest and none were reported.

Additional Contributions: We thank the patient’s parents for granting permission to publish this information.

References
1.
Kroft  SH.  Rosai-Dorfman disease: familiar yet enigmatic.  Semin Diagn Pathol. 2016;33(5):244-253. doi:10.1053/j.semdp.2016.05.008PubMedGoogle ScholarCrossref
2.
Rosai  J, Dorfman  RF.  Sinus histiocytosis with massive lymphadenopathy: a pseudolymphomatous benign disorder. Analysis of 34 cases.  Cancer. 1972;30(5):1174-1188. doi:10.1002/1097-0142(197211)30:5<1174::AID-CNCR2820300507>3.0.CO;2-SPubMedGoogle ScholarCrossref
3.
Pulsoni  A, Anghel  G, Falcucci  P,  et al.  Treatment of sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): report of a case and literature review.  Am J Hematol. 2002;69(1):67-71. doi:10.1002/ajh.10008PubMedGoogle ScholarCrossref
4.
Garces  S, Medeiros  LJ, Patel  KP,  et al.  Mutually exclusive recurrent KRAS and MAP2K1 mutations in Rosai-Dorfman disease.  Mod Pathol. 2017;30(10):1367-1377. doi:10.1038/modpathol.2017.55PubMedGoogle ScholarCrossref
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