Manifestations of Pediatric Extranodal Rosai Dorfman Disease in the head and neck

Alwani, Mohamedkazim; Elghouche, Alhasan N.; Schueth, Elizabeth; Campiti, Vincent J.; Matt, Bruce H.; Yekinni, Abideen O.

doi:10.1016/j.ijporl.2019.109851

Cited by 9 publications

(14 citation statements)

References 43 publications

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“…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 . Progressive laryngotracheal involvement, as seen in the current patient, is otherwise extremely rare and, to our knowledge, was previously reported only once …”

Section: Discussionmentioning

confidence: 57%

Cervical Lymphadenopathy and Airway Masses in a Child

Moore

Astle

Shay

2023

JAMA Otolaryngol Head Neck Surg

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Section: Discussionmentioning

confidence: 57%

Cervical Lymphadenopathy and Airway Masses in a Child

Moore

Astle

Shay

2023

JAMA Otolaryngol Head Neck Surg

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“…Sinonasal involvement is present in 11% of RDD patients, of whom 70% also have multisite disease. [16][17][18][19] Sinonasal involvement does not drive overall prognosis, although it can be associated with aggressive behaviour in systemic disease. [16][17][18]20 However, localised RDD also can arise in the sinonasal tract and generally has excellent outcomes after surgical excision alone.…”

Section: Introductionmentioning

confidence: 99%

“…[16][17][18]20 However, localised RDD also can arise in the sinonasal tract and generally has excellent outcomes after surgical excision alone. [16][17][18][19][20][21][22] Recently, understanding of sinonasal RDD became more complicated with description of RDD-like histiocytesa phenomenon defined by the presence of histiocyte aggregates with histological features of RDD in patients with chronic sinusitis symptoms. 23 It is currently unclear whether these histiocytes truly represent a separate process or a localised manifestation of RDD.…”

Section: Introductionmentioning

confidence: 99%

“…The sinonasal tract is one of the most common sites for extranodal RDD. Sinonasal involvement is present in 11% of RDD patients, of whom 70% also have multisite disease 16–19 . Sinonasal involvement does not drive overall prognosis, although it can be associated with aggressive behaviour in systemic disease 16–18,20 .…”

Section: Introductionmentioning

confidence: 99%

“…Sinonasal involvement does not drive overall prognosis, although it can be associated with aggressive behaviour in systemic disease 16–18,20 . However, localised RDD also can arise in the sinonasal tract and generally has excellent outcomes after surgical excision alone 16–22 . Recently, understanding of sinonasal RDD became more complicated with description of RDD‐like histiocytes – a phenomenon defined by the presence of histiocyte aggregates with histological features of RDD in patients with chronic sinusitis symptoms 23 .…”

Section: Introductionmentioning

confidence: 99%

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Limited sinonasal Rosai–Dorfman disease presenting as chronic sinusitis

et al. 2022

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Aims The sinonasal tract is a common extranodal site for Rosai–Dorfman disease (RDD). Recently, histiocytes with features of RDD were identified in the clinical setting of chronic sinusitis. This study evaluates whether this phenomenon should be considered part of the RDD spectrum or classified separately as RDD‐like histiocytes. Methods and results We prospectively collected 13 cases showing histological features of RDD in chronic sinusitis patients and identified 14 with similar findings (3.5%) via retrospective review of 403 sinus contents over 2 years. All 27 cases displayed nodular aggregates of eosinophilic histiocytes with intermixed lymphoplasmacytic inflammation, prominent eosinophils and emperipolesis. The histiocytes were positive for S100 protein and cyclin D1 and negative for CD1a and CD207. All patients presented with severe chronic sinusitis without tumour formation or systemic symptoms. Twelve patients with follow‐up (55%) required repeat sinus surgery compared with just 43 other sinusitis patients evaluated (11%); features of RDD were present in their additional specimens. Two cases that underwent targeted next‐generation sequencing (20%) had oncogenic mutations in NF1 and KEAP1. Conclusions Overall, these findings confirm diagnostic histological and immunohistochemical features of RDD in a subset of chronic sinusitis specimens. While patients uniformly lack systemic involvement or tumefactive growth, they have a high risk of recurrent sinus disease. Although the relatively subtle nature of the findings raises consideration of separate classification, the presence of occasional oncogenic mutations and evidence of consistent MAPK/ERK pathway activation via cyclin D1 positivity suggests that this phenomenon represents a unique limited manifestation of RDD.

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Pediatric Lymphoid and Histiocytic Lesions in the Head and Neck

Auerbach¹,

Schmieg²,

Aguilera

2021

Head and Neck Pathol

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Lymphoid and histiocytic lesions of the head and neck in pediatric patients is a fascinating topic as most of these lesions are benign, but that the neoplastic cases are essential to diagnose accurately for appropriate treatment. It is thought that 90% of children will have palpable lymph nodes between the ages of 4 to 8; most, but not all, are non-malignant and some resolve spontaneously without treatment. This paper will look at many of the benign and malignant lesions of both lymphocytic and histiocytic origin that present in the head and neck of children focusing on their diagnostic criteria. There is a very pertinent discussion of nonmalignant lymphoid proliferations, as infections and other reactive conditions dominate the pathology of pediatric lymphohistiocytic head and neck lesions. Discussion of those lymphomas which arise more frequently in the head and neck focuses on those seen in children and young adults such as classic Hodgkin lymphoma and Burkitt lymphoma, as well as new more controversial entities such as pediatric-type follicular lymphoma. Histiocytic lesions, both benign and malignant, are described and may be challenging to diagnose.

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Manifestations of Pediatric Extranodal Rosai Dorfman Disease in the head and neck

Cited by 9 publications

References 43 publications

Cervical Lymphadenopathy and Airway Masses in a Child

Cervical Lymphadenopathy and Airway Masses in a Child

Limited sinonasal Rosai–Dorfman disease presenting as chronic sinusitis

Pediatric Lymphoid and Histiocytic Lesions in the Head and Neck

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