We report a case of Rosai-Dorfman Disease, a rare non neoplastic proliferative disorder of the cells of macrophage-histiocyte family, in a case with cervical lymphadenopathy and nasal mass diagnosed by fine needle aspiration cytology

Jena, M

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Madhusmita Jena

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Diagnosis of Rosai-Dorfman Disease by Fine Needle Aspiration Cytology in a Case with Cervical Lymphadenopathy and Nasal Mass

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 Case Report:
Diagnosis of Rosai-Dorfman Disease by Fine Needle Aspiration Cytology in a Case with 
Cervical Lymphadenopathy and Nasal Mass.
Madhusmita Jena, Associate Professor, Department of Pathology, 
MVJ Medical College & Research Hospital, Bangalore, India.
Address for Correspondence:
Dr. Madhusmita Jena,
11C4, Krishna Nagar Apt,
Annasandrapalya,
HAL Post, Bangalore -560017,
India. 
E-mail: jena_madhusmita@hotmail.com
Citation:  Jena  M.  Diagnosis  of  Rosai-Dorfman  Disease  by  Fine  Needle  Aspiration  Cytology  in  a  Case  with  Cervical 
Lymphadenopathy and Nasal Mass. Online J Health Allied Scs. 2011;10(2):22
URL: http://www.ojhas.org/issue38/2011-2-22.htm
Open Access Archives: http://cogprints.org/view/subjects/OJHAS.html and http://openmed.nic.in/view/subjects/ojhas.html
Submitted: Apr 30, 2011; Accepted: Jul 16, 2011; Published: Jul 30, 2011
Abstract:  We report a case of Rosai-Dorfman Disease, a rare 
non  neoplastic  proliferative  disorder  of  the  cells  of  macro-
phage-histiocyte  family,  in a case with cervical lymphadeno-
pathy and nasal mass diagnosed by fine needle aspiration cyto-
logy .
Key Words:  Rosai-Dorfman Disease; Cervical Lymphadeno-
pathy; Nasal Mass; Fine needle aspiration cytology
Case Report:
A 46 years  old  male  presented with complaints  of stuffiness 
and blockade of nose on the left side since six months. On ex-
amination a mass measuring 2.5x1.5cms was seen occupying 
the  left  nasal  cavity.  Multiple  bilateral cervical  lymph nodes 
were enlarged. The largest one measured 1.0x0.5cms. Routine 
blood  counts  were  within  normal  limits.  The  ESR  was 
40mm/1st hour. Chest X-Ray showed no abnormality. A clinical 
diagnosis of lymphoma was suggested.
A fine needle aspiration was performed on cervical lymphnodes 
using a 22G needle. Smears were stained in Papanicoleau and 
Giemsa stains. This was followed by an excisonal biopsy of the 
cervical lymph node and nasal mass.
The  aspirated  smears  showed  a  polymorphous  population  of 
cells consisting of mature lymphocytes,  plasma cells,  neutro-
phils and histiocytes. A diagnosis of reactive lymph node was 
made  initially.  However  a  review of  the  Pap stained smears 
showed few histiocytes with vesicular nuclei and abundant pale 
cytoplasm. The cytoplasm of the histiocytes exhibited intact en-
gulfed lymphocytes (emperipolesis) within them (Fig 1 & 2). A 
cytological  diagnosis  of  Rosai-Dorfan disease  was suggested 
based on the characteristic cytomorphology.
Following biopsy,  Haematoxylin & Eosin sections of the ex-
cised cervical lymph nodes showed a normal architecture with 
dilated  sinuses  filled  with  histiocytes  having  abundant  pale 
cytoplasm and some of them showing emperipolesis (Fig 3 & 
4).The excised nasal mass showed the similar features on histo-
logy.
Fig 1: Smears showing histiocyte with evidence of emperi-
polesis (Pap, X400) 
Fig 2: Smear showing histiocyte with engulfed intact lymph-
ocytes (Giemsa, X400)
1
This work is licensed under a
Creative Commons Attribution-
No Derivative Works 2.5 India License
Online Journal of Health and Allied Sciences
Peer Reviewed, Open Access, Free Online Journal
Published Quarterly :  Mangalore, South India : ISSN 0972-5997
Volume 10, Issue 2; April-June 2011
Fig 3: Excisional biopsy of the cervical lymph node showing 
dilated sinuses filled with histiocytes with abundant pale 
eosinophilic cytoplasm (H&E, X100)
Fig 4: Excisional biopsy of the cervical lymph node showing 
histiocytes with evidence of emperipolesis (H&E, X400)
Discussion:
Rosai-Dorfman Disease, also referred to as Sinus histiocytosis 
with massive lymphadenopathy, is a rare non neoplastic prolif-
erative  disorder  of  the  cells  of  macrophage-histiocyte  family 
that was first described by Destombes in 1965.1 It was recog-
nized as a distinct clinicopathologic entity by Rosai and Dorf-
man in 1969.2 It  commonly affects  the cervical  lymph nodes 
giving rise to painless enlargement of lymph nodes which may 
clinically mimic a lymphoma. It is characterised by expansion 
of  sinuses of the lymph nodes and the lymphatics of extranodal 
sites by proliferation of histiocytes with abundant pale eosino-
philic cytoplasm containing engulfed lymphocytes.
The exact etiology of the disease is unknown.3  It is presumed 
that an aberrant exaggerated immune response to an infectious 
agent causes a proliferation of histiocytes. Although several in-
fectious agents are suspected, but none of them are documented 
so far.4 The expression of HHV6 (Human Herpes Virus) specif-
ic  p101k  antigen  was  found  in  follicular  dendritic  cell  in 
SHML.4
SHML is a disease of childhood and early adulthood. Clinically 
the mean age of onset is second decade.4,6,7 The patient in this 
case  was  a  middle  aged  (46yrs)  man.  The  most  common 
presenting symptom of this disease is painless bilateral cervical 
lymphadenopathy which is seen in 90% of the patients and 43% 
of the cases the patients have at least one site of extra nodal in-
volvement.4,7 Rarely sites other than lymph nodes are involved 
such as skin and soft tissue, upper respiratory system, genitour-
inary tract, eye, orbit, kidney, thyroid, breast, bone.3 The other 
accompanying  features  are  fever,  leucocytosis,  elevated  ESR 
and polyclonal hypergammaglobulinaemia.7  In the present case 
the patient had bilateral cervical lymphadenopathy and a nasal 
mass with an elevated ESR. Clinically the patient was suspec-
ted of lymphoma and the possibility of Rosai-Dorfman disease 
was not considered until FNAC was performed.
As per the review of literature there are only few reports or  
small series of cases on the (FNA) cytologic features of this en-
tity.6 The  characteristic  cytomorphology  of  this  entity  is  the 
presence of large histiocytes with abundant cytoplasm having 
variable number of intact lymphocytes within it; a phenomenon 
referred  to  as  lymphophagocytosis  or  emperipolesis.4,6-8 The 
background typically shows lymphocytes, plasma cells and oc-
casional neutrophils.6-8 In the present case the diagnosis was ini-
tially missed on FNAC as the number of histiocytes were not 
many and so were probably overlooked, but on a review of the 
smears the histiocytes showed the characteristic feature of em-
peripolesis and therefore a diagnosis of Rosai-Dorfman disease 
was made. Besides cytomorphology, the histiocyes on immun-
ostaining  show  positivity  for  S100  protein,  CD14,  CD33  & 
CD68 in cytological smears.8 Extranodal involvement is seen in 
upto 40% of cases which show similar morphological features 
to its nodal counterpart although fewer histiocytes with emperi-
polesis  are encountered.3,5 The patient  had an extra nodal  in-
volvement in the form of a nasal mass which on biopsy showed 
features of sinus histiocytosis with few histiocytes showing the 
characteristic emperipolesis.
Although  the  cytomorphological  features  are  well  described, 
diagnostic difficulties can sometimes arise. The main differen-
tial  diagnosis  on FNAC of  the lymph nodes include reactive 
lymphoid hyperplasia with sinus histiocytosis, malignant histio-
cytosis, lymphoma and tuberculosis.3,6,8 Prominent emperipoles-
is, polymorphous cell population, absence of grooved nuclei of 
histiocytes, absence of eosinophils, absence of Reed Sternberg 
cells and absence of epitheloid granulomas rule out the above 
conditions in the present case.
The course of this disease is usually self limiting in most of the 
patients and so treatment is not necessary in majority of them. 
Surgery is generally limited to biopsy to confirm the diagnosis 
or to relieve obstructive symptoms. Steroids are given to the pa-
tients  with  progressive  disease.7 In  this  patient  surgery  was 
done to confirm the diagnosis of the nasal mass. The patient re-
ceived steroid therapy to which he responded well.
The author likes to emphasize firstly that the the possibility of 
SHML as a differential diagnosis  has be kept in mind of the 
cytopathologist while examining FNA smears of a lymph node. 
Secondly a careful interpretation of the morphology of the histi-
ocytes is required whenever they are less in number as the dia-
gnosis may be missed at the initial stages of the disease or if the 
aspirate is not from a representative area of the lymph node. 
Thirdly if in a case there is an extra nodal manifestation where 
the lymph node aspirate shows a possibility of SHML, then a 
FNA can be performed on the extranodal site and the cytologic-
al  findings  of  both  the  sites  can  be  correlated.  Fourthly  the 
cytological  findings  should  be  interpreted  in  the  appropriate 
clinical context. Thereby FNA can be used as a reliable tool to 
establish a diagnosis and an unnecessary biopsy which is an in-
vasive procedure can be avoided.
References:
1. Destombes  P.  Adenitis  with  lipid  excess  in  children or 
young  adults,seen in  the  Antilles  and in  Mali(4 cases). 
Bull Soc Pathol Exot Filiales.1965;58:1035-1039. 
2. Rosai  J,  Dorfman RF.  Sinus  histiocytosis  with  massive 
lymphadenopathy: a newly recognized benign clinical en-
tity. Arch Pathol.1969;87:63-70.
3. Jinyung Ju, Yong Soon Kwon, Kae Jung Jo et al. Sinus 
Histiocytosis  with  Massive  Lymphadenopathy:  A  Case 
Report with Pleural Effusion and Cervical Lymphadeno-
pathy. J Korean Med Sci 2009;24:760-762.
2
4. Sujata G. Multifocal, Extranodal Sinus Histiocytosis With 
Massive  Lymphadenopathy.  An Overview.  Arch Pathol  
Lab Med 2007;131:1117-1121.
5. Li S, Yan Z, Jhala N, Jhala D. Fine needle aspiration dia-
gnosis  of  Rosai-Dorfman disease in an osteolyic  lesion. 
CytoJournal. 2010;7:12.
6. Das DK, Gulati A, Bhatt NC, Sethi GR. Sinus Histiocyt-
osis with massive lymphadenopathy (Rosai-Dorfman dis-
ease):  report  on  two  cases  with  fine  needle  aspiration 
cytology. Diagn Cytopathol. 2001;24:42-45.
7. Sachdev  R,  Setia  N,  Jain  S.  Sinus  histiocytosis  with 
massive  lymphadenopathy.  Is  the  lymph  node  enlarge-
ment always massive?  Med Oral Patol Oral Cir Buccal. 
2007;12:198-200.
8. Kushwaha R, Ahluwalia C, Sipayya K. Diagnosis of sinus 
histiocytosis with massive lymphadenopathy (Rosai-Dorf-
man disease) by fine needle aspiration cytology.  Journal  
of Cytology. 2009;26:83-85.
3


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Diagnosis of Rosai-Dorfman Disease by Fine Needle Aspiration Cytology in a Case with Cervical Lymphadenopathy and Nasal Mass

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