International Journal of Pediatric Otorhinolaryngology Extra 6 (2011) 285–287

Case report

A laryngeal Rosai-Dorfman disease

Mireille Folia a,*, Laurent Martin b, Christian Duvillard a, Philippe Romanet a

a Otorhinolaryngology General Hospital, DijonFranceb Laboratory of Histopathology Bocage Hospital, DijonFrance

A R T I C L E I N F O

Article history:

Received 11 October 2010

Received in revised form 13 January 2011

Accepted 15 January 2011

Available online 12 February 2011

Keywords:

Rosai-Dorfman disease

Dyspnoea

Sub-glottal mass

Laryngoscopy

A B S T R A C T

Rosai-Dorfman disease is a sinus histiocytosis with massive lymphadenopathy, most often in the cervical

region. While extra-ganglion involvement is frequent (40% of cases), involvement of the larynx is rare.

We present the case of a 9-year-old boy presented a laryngeal localisation of Rosai-Dorfman disease. We

describe the management of diagnosis and therapeutics that location. If treatment of the disease is not

well codified, its prognosis is usually good, but requires prolonged follow-up.

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International Journal of Pediatric OtorhinolaryngologyExtra

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1. Introduction

Destombes [1] in 1965 then Rosai-Dorfmann in 1969 describeda new pathological entity and defined as sinus histiocytosis withmassive lymphadenopathy. It was distinct from other forms ofhistiocytoses reported until then [2]. This disease is usuallycharacterized by often painless massive cervical lymphadenopathyin which the lymph node sinuses appear dilated and are colonisedby large numbers of histiocytes. This lymphadenopathy usuallyregresses progressively [1–3]. Though extra-nodal areas arefrequently affected [3–5] involvement of the larynx and tracheaare rare [6]. The aim of this article is to present the diagnosis andtherapeutic management of Rosai-Dorfman disease, the first signof which was a mass in the larynx.

2. Clinical case

A 9-year-old boy, with no particular medical history, wasreferred to the ENT department in December 97 because ofprogressive-onset dyspnoea, which gradually worsened. The boyhad suffered from this dyspnoea for 10 months, and had benefitedfrom a medical examination 3 months earlier. The examinationcomprised chest radiography and pulmonary function tests, butdid not lead to a conclusive diagnosis.

During the consultation, we found dyspnoea at rest on bothinhalation and exhalation. According to the mother, the dyspnoea

* Corresponding author at: 3 rue Faubourg Raines, 21000 Dijon, France.

Tel.: +33 0380293758; fax: +33 0380293598.

E-mail address: mireille.folia@chu-dijon.fr (M. Folia).

1871-4048/$ – see front matter � 2011 Elsevier Ireland Ltd. All rights reserved.

doi:10.1016/j.pedex.2011.01.005

often led to acute episodes of asphyxia. The clinical examinationrevealed a swollen, mobile, painless left subdigastric lymph nodethat measured 3 cm along the longest axis. Laryngoscopy revealeda subglottic mass. The patient was referred for cervicothoracic CTscan and endoscopy. The CT scan (Fig. 1) revealed a highlycontrast-enhanced mass attached to the left side of the trachea,obstructing approximately 60% of the tracheal lumen, suggesting ahighly vascularised tumour. The swollen lymph node was,however, isolated.

Suspension laryngoscopy revealed a sub-glottal sessile mass,all along and slightly posterior to the left side of the trachea, whichreduced the tracheal lumen. This mass was yellowish and coveredwith a number of vessels (Fig. 2). The mass was excised usinglaser.

The post-operative period was without incident and thedyspnoea disappeared.

Pathological examination of the surgical piece revealed ahistiocytic reaction and a large number of macrophage foam cellswith intracytoplasmic lymphocytes. Immunohistochemistryshowed the presence of S-100 protein and CD68 leucocyteantigens that indicated a diagnosis of Rosai-Dorfman disease(RD) (Fig. 3).

One month later, the patient presented new painless bilateralcervical lymphadenopathy accompanied by bilateral nasal ob-struction with purulent rhinorrhea.

Rhinoscopy and laryngoscopy were performed. The laryngos-copy revealed the persistence of the tumour base, but there hadbeen no new growth. Rhinoscopy showed the presence ofyellowish lesions that extended from the inferior nasal conchato the septum and occupied the cavum and the posterior wall of thesoft palate. Biopsy confirmed the presence of sinus histiocytosis.

Fig. 1. Cervical CT scan: sub-glottal mass reducing the lumen.

Fig. 2. Sub-glottal mass by laryngoscopy.

M. Folia et al. / International Journal of Pediatric Otorhinolaryngology Extra 6 (2011) 285–287286

Local corticotherapy associated with a short course of systemiccorticotherapy was implemented.

One year later, the sub-glottal lesion was no longer visible, andthe nasal lesions had partially disappeared, but the cervicallymphadenopathy had hardly regressed.

Fig. 3. Pathological aspect: (a) standard staining, (b) s

At two years there were no visible lesions and the cervicallymph nodes were not palpable.

At 10 years follow-up there was no recurrence.

3. Discussion

Rosai-Dorfman disease or sinus histiocytosis was first describedin 1969 as a disease of unknown etiology. The prognosis isgenerally good. The disease is most often encountered during thefirst twenty years of life (mean age 20 years) [5,6] and is differentfrom other forms of childhood histiocytoses.

Rosai-Dorfman disease usually presents as painless, bilateral,massive cervical lymphadenopathy; involvement of the axillary,inguinal or mediastinal lymph nodes is far more rare. Cervicalinvolvement is found in 80% of cases [5].

When the lymph nodes are diseased, extra-nodal involve-ment is found in 30–40% of cases. The head and neck are oftenaffected by this disease, in particular the nasal cavity andsinuses (20% of patients) [6,7]. After the lymph nodes, it is thesecond most frequent site of this disease. The salivary glands,the skin, the orbit and the central nervous system are alsofrequently affected [8].

Only nine cases of involvement of the larynx have beendescribed in the literature [3,7–13], and in seven of these therewas associated cervical lymphadenopathy. For five, the vocalcords were affected by the histiocytic process emanating fromthe sub-glottal region. Eight of the nine patients presentedinvolvement of another territory in particular the nasal cavity orsinuses.

In our case, other sites were also affected, but involvement ofthe larynx was the first sign of the disease and was life-threatening.

From a macroscopic point of view, the lesions in Rosai-Dorfmandisease are polyploid, exophytic and yellowish [3], as was the casein our study.

With regard to histology [3,5,10], the disease presentsa number of specific characteristics, in particular the

taining for S-100 protein, (c) staining for CD 68.

M. Folia et al. / International Journal of Pediatric Otorhinolaryngology Extra 6 (2011) 285–287 287

presence of lymphocytes, cellular components from the plasmawith a predominance of histiocytes characterized by a volumi-nous nucleus and abundant clear cytoplasm. The normalarchitecture of the lymph node is replaced by large numbersof histiocytes.

Lysosomal activity of alpha 1 antichymotrypsin is a majorattribute of this disease.

Emperipolesis or lymphagocytosis are typical features of thisdisease and allow diagnosis. Fibrosis is often found and is also seenin extra-nodal Rosai-Dorfman disease.

Immunohistochemistry [10,14] shows the presence of S-100protein.

The prognosis for RD is generally good with completerecovery from the first episode in 25% of cases, but there areoften long periods of remission interspersed with nodal or extra-nodal recurrences. These recurrences may occur long afterthe first episode [4–6]; this is why our patient is followedregularly.

The evolution of RD varies according to the degree ofimmunodeficiency and the spread of the disease [4].

The presence of widespread tissue involvement affecting inparticular the lungs, kidneys or brain is a predictor of a poorprognosis. In such cases the disease may be fatal [5,7].

The treatment of Rosai-Dorfman disease has not been codified.It seems however that corticotherapy can be used in severe cases.Other treatments (chemotherapy, radiotherapy) have not beenshown to be effective [5,15]

For laryngeal involvement, surgery is the first-line treatment[8–12], except in cases where simple surveillance is appropriate[13]. A number of authors performed preoperative tracheotomy[3,9]. Unlike certain authors [12], we decided to free the upperairways by removing the lesion using laser rather than wait for thespontaneous regression. This appeared particularly appropriate asthe glottis was healthy.

4. Conclusion

Laryngeal involvement is rare, but potentially life-threateningas it can obstruct the upper airways. In such cases surgery can be

used to open them up. Long-term surveillance is necessary toensure that cure has been achieved.

References

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