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Cervical Lymphadenopathy and Airway Masses 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 previously healthy 12-year-old boy presented with a 3-month history of nasal obstruction, progressive dysphonia, recurrent deep neck abscesses, and tender, bulky cervical lymphadenopathy. Computed tomography (CT) of the neck with contrast at his initial hospital admission was concerning for suppurative lymphadenitis. Despite incision and drainage, and intravenous ampicillin and sulbactam, his cervical lymphadenopathy persisted. Results of subsequent workup—including Haemophilus, tetanus, and pneumococcal antibody titers; QuantiFERON-TB Gold test; serum IgG, IgM, IgA, and IgE levels; absolute lymphocyte count; neutrophil phenotype and function; angiotensin-converting enzyme level; HLA-B27 test; and antinuclear antibody level—were within normal limits. Magnetic resonance imaging of the neck with gadolinium contrast demonstrated bilateral, bulky, enlarged cervical lymph nodes, as well as enhancing nodules in the left laryngeal ventricle (2.3 × 1.6 × 1.2 cm), right false vocal cord, and right tracheal wall (0.6 × 0.4 cm). Findings from nasal endoscopy, awake flexible laryngoscopy, and operative microlaryngoscopy revealed yellow submucosal masses in the left nasal cavity, left laryngeal ventricle, and right trachea, as well as left vocal cord paresis (Figure, A). Biopsies were taken of the tracheal, laryngeal, and nasal cavity masses, and an excisional biopsy was taken of a left cervical lymph node (Figure, B). Histopathologic examination revealed a prominent infiltrate of benign-appearing histiocytes with numerous forms showing emperipolesis and some nuclei showing distinct central nucleoli. Immunohistochemical staining in these cells was positive for S100 and negative for CD1a.

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A. Rosai-Dorfman disease

Rosai-Dorfman disease (RDD), also known as sinus histiocytosis with massive lymphadenopathy, was originally described by Rosai and Dorfman in 1969.1 It is a rare, benign, proliferative disease with an unknown cause but is thought to have an association with viral infections, immune-related disorders, and genetic mutations.2,3 It is most frequently seen in children and young adults (mean age, 20 years) with a male predominance of 1.4.4,5 The classic, nodal form most commonly manifests as painless cervical lymphadenopathy. Extranodal involvement occurs in up to 43% of cases and usually affects the skin, soft tissues, central nervous system, and head and neck regions. While symptoms largely depend on location and disease burden, many patients are asymptomatic. A recent study identified 31 known cases of pediatric RDD with head and neck involvement, of which 58% had unifocal disease with a predilection for the nasal cavity and paranasal sinuses.6 Progressive laryngotracheal involvement, as seen in the current patient, is otherwise extremely rare and, to our knowledge, was previously reported only once.7

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Article Information

Corresponding Author: Samantha M. Moore, BS, School of Medicine, Medical College of Wisconsin, 8701 W Watertown Plank Rd, Milwaukee, WI 53226 (smmoore@mcw.edu).

Published Online: November 10, 2022. doi:10.1001/jamaoto.2022.3576

Conflict of Interest Disclosures: None reported.

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

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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
Foucar  E , Rosai  J , Dorfman  R .  Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): review of the entity.   Semin Diagn Pathol. 1990;7(1):19-73.PubMedGoogle Scholar
Bruce-Brand  C , Schneider  JW , Schubert  P .  Rosai-Dorfman disease: an overview.   J Clin Pathol. 2020;73(11):697-705. doi:10.1136/jclinpath-2020-206733PubMedGoogle ScholarCrossref
Alwani  MM , Elghouche  AN , Schueth  EA , Campiti  VJ , Matt  BH , Yekinni  AO .  Manifestations of pediatric extranodal Rosai Dorfman disease in the head and neck.   Int J Pediatr Otorhinolaryngol. 2020;131:109851. doi:10.1016/j.ijporl.2019.109851PubMedGoogle ScholarCrossref
Unal  OF , Koçan  EG , Sungur  A , Kaya  S .  Rosai–Dorfman disease with multi-organ involvement in head and neck region.   Int J Pediatr Otorhinolaryngol. 2004;68(5):581-584. doi:10.1016/j.ijporl.2003.11.019PubMedGoogle ScholarCrossref
Dispenzieri  A , Fajgenbaum  DC .  Overview of Castleman disease.   Blood. 2020;135(16):1353-1364. doi:10.1182/blood.2019000931PubMedGoogle ScholarCrossref
Kutok  JL , Pinkus  GS , Dorfman  DM , Fletcher  CD .  Inflammatory pseudotumor of lymph node and spleen: an entity biologically distinct from inflammatory myofibroblastic tumor.   Hum Pathol. 2001;32(12):1382-1387. doi:10.1053/hupa.2001.29679PubMedGoogle ScholarCrossref
Tana  C , Donatiello  I , Caputo  A ,  et al.  Clinical features, histopathology and differential diagnosis of sarcoidosis.   Cells. 2021;11(1):59. doi:10.3390/cells11010059PubMedGoogle ScholarCrossref
AMA CME Accreditation Information

Credit Designation Statement: The American Medical Association designates this Journal-based CME activity activity for a maximum of 1.00  AMA PRA Category 1 Credit(s)™. Physicians should claim only the credit commensurate with the extent of their participation in the activity.

Successful completion of this CME activity, which includes participation in the evaluation component, enables the participant to earn up to:

  • 1.00 Medical Knowledge MOC points in the American Board of Internal Medicine's (ABIM) Maintenance of Certification (MOC) program;;
  • 1.00 Self-Assessment points in the American Board of Otolaryngology – Head and Neck Surgery’s (ABOHNS) Continuing Certification program;
  • 1.00 MOC points in the American Board of Pediatrics’ (ABP) Maintenance of Certification (MOC) program;
  • 1.00 Lifelong Learning points in the American Board of Pathology’s (ABPath) Continuing Certification program; and
  • 1.00 CME points in the American Board of Surgery’s (ABS) Continuing Certification program

It is the CME activity provider's responsibility to submit participant completion information to ACCME for the purpose of granting MOC credit.

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