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OJHAS Vol. 10, Issue 2:
(Apr-Jun 2011) |
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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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Madhusmita Jena,
Associate Professor, Department of Pathology,
MVJ Medical College & Research Hospital, Bangalore, India |
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Address for Correspondence |
11C4, Krishna Nagar Apt,
Annasandrapalya,
HAL Post, Bangalore -560017,
India.
E-mail:
jena_madhusmita@hotmail.com |
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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 |
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Submitted: Apr 30,
2011; Accepted: Jul 16, 2011; Published: Jul 30, 2011 |
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| Abstract: |
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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
Key Words:
Rosai-Dorfman Disease; Cervical Lymphadenopathy; Nasal Mass; Fine
needle aspiration cytology
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A 46 years old male presented with complaints of stuffiness and blockade
of nose on the left side since six months. On examination 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,
neutrophils 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 engulfed lymphocytes (emperipolesis)
within them (Fig 1 & 2). A cytological diagnosis of Rosai-Dorfan
disease was suggested based on the characteristic cytomorphology.
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Fig 1: Smears
showing histiocyte with evidence of emperipolesis (Pap, X400) |
Fig 2: Smear
showing histiocyte with engulfed intact lymphocytes (Giemsa, X400) |
Following biopsy,
Haematoxylin & Eosin sections of the excised 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
histology.
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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) |
Rosai-Dorfman
Disease, also referred to as Sinus histiocytosis with massive lymphadenopathy,
is a rare non neoplastic proliferative disorder of the cells of macrophage-histiocyte
family that was first described by Destombes in 1965.1
It was recognized as a distinct clinicopathologic entity by Rosai
and Dorfman 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 eosinophilic 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 infectious
agents are suspected, but none of them are documented so far.4
The expression of HHV6 (Human Herpes Virus) specific 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 involvement.4,7 Rarely
sites other than lymph nodes are involved such as skin and soft tissue,
upper respiratory system, genitourinary 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 suspected
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 entity.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 occasional neutrophils.6-8
In the present case the diagnosis was initially 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 emperipolesis and therefore a diagnosis of Rosai-Dorfman disease
was made. Besides cytomorphology, the histiocyes on immunostaining 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 emperipolesis are encountered.3,5 The patient
had an extra nodal involvement 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 differential diagnosis on
FNAC of the lymph nodes include reactive lymphoid hyperplasia with
sinus histiocytosis, malignant histiocytosis, lymphoma and tuberculosis.3,6,8
Prominent emperipolesis, 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 patients with progressive disease.7
In this patient surgery was done to confirm the diagnosis of the nasal
mass. The patient received 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 histiocytes is required whenever they are less
in number as the diagnosis 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 cytological 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 invasive procedure can be avoided.
- 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.
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Rosai J, Dorfman RF. Sinus histiocytosis with massive lymphadenopathy: a newly
recognized benign clinical entity. Arch Pathol.1969;87:63-70.
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Jinyung
Ju, Yong Soon Kwon, Kae Jung Jo et al. Sinus Histiocytosis with Massive
Lymphadenopathy: A Case Report with Pleural Effusion and Cervical Lymphadenopathy.
J
Korean Med Sci 2009;24:760-762.
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Sujata G.
Multifocal, Extranodal Sinus Histiocytosis With Massive Lymphadenopathy.
An Overview. Arch Pathol Lab Med 2007;131:1117-1121.
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Li S, Yan
Z, Jhala N, Jhala D. Fine needle aspiration diagnosis of Rosai-Dorfman
disease in an osteolyic lesion. CytoJournal. 2010;7:12.
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Das DK,
Gulati A, Bhatt NC, Sethi GR. Sinus Histiocytosis with massive lymphadenopathy (Rosai-Dorfman
disease): report on two cases with fine needle aspiration cytology. Diagn
Cytopathol. 2001;24:42-45.
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Sachdev
R, Setia N, Jain S. Sinus histiocytosis with massive lymphadenopathy.
Is the lymph node enlargement always massive? Med Oral Patol Oral Cir
Buccal. 2007;12:198-200.
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Kushwaha
R, Ahluwalia C, Sipayya K. Diagnosis of sinus histiocytosis with massive
lymphadenopathy (Rosai-Dorfman disease) by fine needle aspiration cytology. Journal
of Cytology. 2009;26:83-85.
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