To : ………………………….

ENT Department
Medical Faculty, Universitas Indonesia
RSUPN Dr. Cipto Mangunkusumo

Jakarta

Case Report

Surgical Management of Head and Neck Manifestation in Rosai-Dorfman-Destombes

Disease

Presenter : Devianty Octavia, MD

Day/Date : Tuesday, July 26th, 2022
Time : 07.30 AM
Place : ORL-HNS Department, Public Wing, 7th Floor, CMGH
Zoom Meeting
Opponent : Budiman Gumilang K., MD
Note person : Charlene Alia Shavana, MD
Moderator : Syahrial M. Hutauruk, MD, ORL-HNS
Tutor : Marlinda Adham, MD, ORL-HNS, PhD, FACS
Resource person : Sukamto, MD, PhD, Internist
Rahmat Cahyanur, MD, Internist
Reyhan Eddy Yunus, MD, M.Sc, Radiologist
Maria Francisca Ham, MD, PhD, Pathologist
 Surgical Management of Head and Neck Manifestation in Rosai-Dorfman-Destombes
Disease
Devianty Octavia
Otorhinolaryngology-Head and Neck Surgery Department, Faculty of Medicine, Universitas Indonesia, Indonesia –
Dr. Cipto Mangunkusumo National Hospital

ABSTRACT
Background: Rosai-Dorfman-Destombes disease (RDD) is a rare non-Langerhans cell
histiocytosis disease. Classical RDD features massive cervical lymphadenopathy, while extranodal
manifestation occurs in 43% of patients. Wide clinical spectrum of RDD made its diagnosis and
treatment challenging. Purpose: To report a case of relapsing right cervical lymphadenopathy due
to Rosai-Dorfman-Destombes disease (RDD) treated with combination of systemic steroid therapy
and surgical resection. Case: A nineteen-year-old male presented with relapsing unilateral cervical
lymphadenopathy. Physical and supporting examination supported the diagnosis of Classical
RDD. Combination of systemic steroid therapy and surgical resection were the treatment of choice
as proposed by a current consensus of RDD management. Conclusion: Multidisciplinary
collaboration plays a vital role in evaluation and management of RDD with many diagnostic and
therapeutic challenges. Surgical resection may relieve symptoms and restore function for unifocal
disease of RDD. Subsequent adjuvant treatment can be considered in cases with local recurrence
after surgical resection.
Keywords: Rosai Dorfman Destombes disease, cervical lymphadenopathy, surgical resection

1.4:1.0 ratio.1–3 Patients with RDD may be

INTRODUCTION
presented with nodal or extranodal disease.
Rosai-Dorfman-Destombes disease (RDD) is
Its etiology remains uncertain and may
a rare non-Langerhans cell histiocytosis
appear as an individual disorder or associated
disease, identified by the accumulation of
with autoimmune, genetic, or malignancies.
activated histiocytes in the affected tissues.
Treatment modalities consist of observation,
RDD was first reported in 1965 by Pierre
steroids, surgery, radiotherapy,
Paul Louis Lucien Destombes, a French
chemotherapy, however no modality has
pathologist, and later in 1969 by Juan Rosai
presented consistent outcomes. Morbidity
and Ronald Dorfman, who reported 34 cases
and mortality are encountered by patients
of sinus histiocytosis with massive
with multifocal or refractory disease. Owing
lymphadenopathy (SHML). The worldwide
to its wide clinical spectrum, to diagnose and
prevalence of RDD is 1:200 000, and it is
manage RDD is an enduring challenge, a
more often seen in children and adolescents,
thorough examination and interdisciplinary
also in males compared to females in a
collaboration are required.1,2
CASE REPORT
A nineteen-year-old male was consulted to
ENT-Oncology division by medical hemato-
oncology division, complaining of relapsing
painless lymph nodes enlargement in the
right side of neck in the last 3 years,
especially when the patient suffered from
common cold. There was no history of
difficulty in deglutition nor respiration,
chronic cough, loss of appetite or loss of
weight, and no other lump or swelling in
other region. Patient was diagnosed with
right neck lymphadenopathy dd/ RDD and
had been previously prescribed with
Prednisone 1 mg/kg/day, and was consulted
for surgical resection consideration.
Physical examination of the right colli region
showed multiple conglomerated cervical
lymph nodes enlargement in level IB-IV
sized 7x5x3cm, and level V sized 5x3x2cm,
mobile, sharp-bordered, tender to firm
consistency, with no tenderness. Flexible
nasopharyngoscopy showed no abnormality
finding. Laboratory examination showed
normal level of leucocyte, LDH, slightly
elevated procalcitonin level (0.19 ng/mL),
and reactive IgG serum of anti HSV1, anti
CMV, anti Rubella. Ultrasonography and
Neck MSCT scan with contrast showed
multiple conglomerated right cervical
lymphadenopathy with necrotic component
in level IB, II-V (greatest size 3x5x4 cm), and
non-contrast-enhancing multiple bilateral
thyroid nodules (greatest size 1 cm in
diameter). No visible mass nor other
radiological abnormality in
thoracoabdominal organs as shown in
thoracoabdominal MSCT scan with contrast.
Right modified radical neck dissection was
done, and specimens were sent for
histopathology examination, and the result
showed sinus histiocytosis with massive
lymphadenopathy, immunohistochemistry
showed positive result in CD 68, S 100,
CD163, CD 20, CD 3, accordant with Rosai-
Dorfman disease.
DISCUSSION
RDD is a rare cause of lymph node
enlargement in children and adolescents,
most commonly seen in second decade of
life, and in males.2–4 Concordant with the
literature, the case reported in this case,
patient was a nineteen-year-old male, who
experienced the complaint of relapsing
cervical lymphadenopathy since 3 years,
although in study by Goyal et al in 2019, in
64 cases of RDD, there was a female
preponderance, with 1,5:1 ratio.1
The etiology of RDD is indistinct, however
studies mentioned viral infections, such as
HSV, EBV, CMV, and HIV are associated
with RDD. In our patients, laboratory
examination showed reactive IgG serum of
anti HSV1, anti CMV, anti Rubella.1
Although further examination has not been
performed, patient was considered a healthy
individual without any tendency to other
medical condition associated with RDD, such
as inherited condition predisposing to RDD ,
Neoplasia-associated RDD, Immune-related
RDD, and IgG4-related RDD.2
Symptomatic right cervical
lymphadenopathy was the chief complaint of
the patient, also described as classic (nodal)
RDD, as found in most patients. On the other
hand, Goyal et al reported more extranodal
RDD compared to other subtypes (92%).1,2
Supporting examination showed
asymptomatic multiple bilateral thyroid
nodule and multiple mediastinal
lymphadenopathy with considered normal
morphology and size. One of the extranodal
forms of RDD is nasal cavity and paranasal
sinuses involvement, which comprises 11%
of head and neck manifestation in RDD. The
occurance of oral cavity, oropharynx,
salivary glands, larynx, pharynx, and thymus
involvement can cause mass effect.2
Histopathology and immunohistochemistry
play a vital role to distinguish RDD from
other disease entities presenting with massive
lymphadenopathy. Langerhans cell
histiocytosis (LCH), RDD, malignant
histiocytosis, and juvenile xanthogranuloma
all are depicted by generalized or localized
histiocytes proliferation, but their
morphology is different, in terms of
immunochemical and histochemical staining
pattern.5 The presence of emperipolesis, sinus
histiocytosis and the absence of acid fast
bacilli in our patient excluded possibility of
other disease such as malignant lymphoma,
granulomatous lesion, etc.4
To date, there is only one consensus guideline
for RDD by Abla et al, in which proposed a
management algorithm for patients with
RDD as presented in figure 3. In case of
symptomatic nodal RDD, biopsy or resection
is amenable.2 Patient may need subsequent
treatment after surgery, as one-third of
patients in study by Goyal et al experienced.2
Corticosteroids, sirolimus, radiotherapy,
chemotherapy, immunomodulatory can be
considered as subsequent therapy.
After initial treatment, assessment of first
response should be evaluated in 4 months,
and if the treatment showed good response,
surveillance period can be prolonged to 6
months to a year. Insufficient data to describe
RDD prognosis is not yet available, in case of
nodal or cutaneous disease, outcomes are
usually favorable. Patients with multiple
focal and extranodal RDD, with involvement
of liver, kidney, and lower respiratory tract Journal of Otorhinolaryngology.
seen to have bad prognosis.2 2008;74(4):632–5.
4. Singh A, Kanaujiya SK. The Rosai–
Dorfman Disease: A Differential
CONCLUSION Diagnosis in Cervical Swelling.
Multidisciplinary collaboration plays a vital Indian Journal of Otolaryngology and
role in evaluation and management of RDD Head and Neck Surgery. 2019 Oct
1;71:107–12.
with many diagnostic and therapeutic
5. Hengartner H, Oehlschlegel C. Sinus
challenges. Surgical resection may relieve Histiocytosis with massive
symptoms and restore the function for lymphadenopathy (Rosai-Dorfman-
disease) treated with Cladribine (2-
unifocal disease of RDD. Subsequent
CdA). Schweizerische Pädiatrische
adjuvant treatment can be considered in cases Onkologie Gruppe Groupe suisse
with local recurrence after surgical resection. d’oncologie pédiatrique. 2009;

REFERENCES
1. Goyal G, Ravindran A, Young JR,
Shah M v., Bennani NN, Patnaik
MM, et al. Clinicopathological
features, treatment approaches, and
outcomes in Rosai-Dorfman disease.
In: Haematologica. Ferrata Storti
Foundation; 2020. p. 348–57.
2. Abla O, Jacobsen E, Picarsic J,
Krenova Z, Jaffe R, Emile JF, et al.
Consensus recommendations for the
diagnosis and clinical management of
Rosai-Dorfman-Destombes disease
[Internet]. 2018. Available from:
http://ashpublications.org/blood/articl
e-
pdf/131/26/2877/1466369/blood8397
53.pdf
3. Pinto DCG, Vidigal TDA, de Castro
B, dos Santos BH, de Sousa NJA.
Doença de Rosai-Dorfman como
diagnóstico diferencial de
linfadenopatia cervical. Brazilian
FIGURES

(a) (b)
Figure 1. (a) Patient with multiple right cervical lymphadenopathy, with hypertrophic scar from
previous history of incisional biopsy. (b) After right modified radical neck dissection, intact
suture with no visible mass nor lump.

Figure 2. Neck MSCT scan with contrast showed multiple conglomerated right cervical
lymphadenopathy with necrotic component in level IB, II-V (greatest size 3x5x4 cm), and non-
contrast-enhancing multiple bilateral thyroid nodules (greatest size 1 cm in diameter).

Figure 3. Proposed algorithm for management of RDD.2