INDIAN JOURNAL OF MEDICAL & PAEDIATRIC ONCOLOGY Vol. 25 No.

4, 2004 39
Rosai-Dorfman Disease : A Case Report With Review of
Li t er at ur e
GULL MOHD BHAT, SURENDER KUMAR, ATUL SHARMA
ABSTRACT
Si nus hi sti ocytosi s wi th massi ve
lymphadenopathy (Rosai Dorfman disease)
i s an uncommon beni gn condi ti on, often
confused with lymphoma. Patients usually
have massive enlargement of cervical lymph
nodes. Cl i ni cal course i s vari abl e. We
describe here one such young male patient
wi th tonsi l l ar enl argement. Perti nent
literature is reviewed.
INTRODUCTION
Cast l eman s di sease, dermopat hi c l ympha-
denitis, mucocutaneous lymph node syndrome
(Kawasaki s di sease), hi sti ocyti c necroti si ng
lymphadenopathy (kikuchis disease), vascular
t ransf ormat i on of l ymph nodes and
inflammatory pseudotumour of the lymph node
are among t he rare causes of l ymph node
enlargement. Sinus histiocytosis with massive
l ymphadenopat hy (SHML) i s an i mport ant
addition to this list. An oncologist comes across
a case every now and t hen wi t h a cl i ni cal
picture hard to tell from malignant lymphomas.
Histopathology usually comes as a surprise. At
the same time it is a relief to the physician and
the patient, as this disease has essentially a
benign course.
Case Report: -
MSA, a 10 years young boy, resident of Bihar was
apparently alright about 3-4 months when he
noticed a right neck mass, insidious in onset
and slowly increasing in size. Similar painless
swellings appeared on the other side of the neck,
Dept. of Medical Oncology, IRCH
All India Institute of Medical Sciences, New Delhi.
Correspondence to : GULL MOHD BHAT
Email : drgull8125@yahoo.com
both maxilla and epitroclear areas. The upper
neck swellings progressed to attain the size of a
wal nut. There was accom-panyi ng hi story of
intermittent low grade fever, lasting for a couple
of days unaccompani ed by chi l l s but usual l y
associ at ed wi t h cough and bl ood t i nged
expect orat i on. He had a si ngl e epi sode of
epi st axi s; (on f ol l ow up vi si t had repeat
epi sodes of nasal bl eed) and had been
transfused a unit of blood during this period.
There has been no accompanyi ng hi story of
weight loss, night sweats or pruritis. His past,
personal, and family history has been normal.
Cl i ni cal exami nati on showed that hi s wei ght
was 25 kgs, hei ght 129 cms and had fi nger
clubbing, bilateral tonsillar hypertrophy, a bull
neck wi th mul ti pl e, general i zed, non-tender,
f i rm, and mat t ed (i n cervi cal regi on)
lymphadenopathy. In other areas lymph nodes
varied from 1 to 2 cms in size and (during follow
up developed lymph node which increased in
si ze i n armpi ts). Hi s abdomi nal exami nati on
revealed moderate non-tender and soft / firm
hepatosplenomegaly (5 & 2 cms below costal
margi ns) wi t h no f ree f l ui d. Chest ,
cardi ovascul ar and cent ral nervous syst em
examination was unremarkable.
Investigations showed a hemoglobin of 8.2
WBC - 8500/cmm, defferential - N 63, E7, L30,
pl atel ets 180000/cmm. Peri pheral bl ood fi l m
showed mi crocyt i c, hypochromi c red bl ood
cel l s. ESR 58 mm/ hr. Bi ochemi cal prof i l e
revealed normal renal and liver functions, with
serum alkaline phosphates 225 units and LDH-
64 units/ dl. Chest x-ray showed bilateral Hilar
and Parat racheal l ymphadenopat hy. Chest
i magi ng st udi es reveal ed medi ast i nal
lymphadenopathy. USG scan of the abdomen
Case Report-III
INDIAN JOURNAL OF MEDICAL & PAEDIATRIC ONCOLOGY Vol. 25 No.4, 2004 40
reveal ed many peri pancreati c and peri portal
lymphadenopathy. FNAC of left cervical node
showed sheets of sinus histiocytes, some of
which had evidence of emperopolesis and there
was presence of histiocytes and necrosis on the
right side.
Lymph node bi opsy showed markedl y
dilated sinuses containing sheets of histiocytes
with eosinophilic cytoplasm and small nuclei;
some of the histiocytes show small lymphocytes
in cytoplasm (emperipolesis). Scanty lymphoid
t i ssue i s seen i n bet ween. There was no
evidence of malignancy or granulomas.These
features were consi stent wi th a di agnosi s of
si nus hi st i ocyt osi s wi t h massi ve l ymphade-
nopathy (Rosai-Dorfman Disease) (Fig. 1.A & B).
DISCUSSION:
Thi s condi ti on was fi rst descri bed by Robb-
Smith in 1947 in children and was termed as
giant cell sinus reticulosis . Sinus histiocytosis
wi t h massi ve l ymphadenopat hy (SHML) has
been recognized as a distinct clinicopathological
entity, though first given this name by Rosai and
Dorfman i n 1969,1972. It has morphol ogi cal
features of greatly exaggerated reactive process.
There i s usual l y no demonstrabl e eti ol ogi cal
agent. The lymph nodes may attain enormous
size and may remain persistently enlarged for a
prolonged period. The disease is not confined to
a particular race or geographical area, although
African Negroes are more commonly involved
than whites. Characterized by massive, painless
and general l y bi l ateral cervi cal l ymph node
enlargement with relatively minor involvement
of other groups of nodes i n most i nstances.
About 25% of cases have involvement of extra
nodal si tes, especi al l y the upper respi ratory
t ract , sal i vary gl ands, orbi t and t est i s and
lesions at other sites and skin may appear before
the appearance of lymph nodes.
1
Patients with
extensive lung and liver involvement may not
have as innocent course.
2
There may be osseous
involvement in pediatric age group. Other rare
si t es i ncl uded sol i t ary i nvol vement of tal us,
triquetrum,sclera, conjunctiva, thymus, pleura,
nasopharynx, genitourinary and central nervous
system. These patients usually have moderate
pyrexia with mild anemia, neutrophilia, a raised
ESR and polyclonal gammapathy. Autoimmune
hemolysis can also occur. Suggestion has been
made that it may be an unusual reaction to a
klebsiella infection.
3
Association to subsequent
mal i gnanci es i s al so descri bed.
4
An i nf ant
present i ng wi t h hepat ospl enomegal y,
bi cytopeni a consi dered as congeni tal form of
Rosai dorfman desease has been described.
5
The
di agnosi s i s usual l y made on charact eri st i c
hi st opat hol ogi cal and cyt ol ogi cal f eat ures.
HUMARA assay shows t he di sease t o be
moncl onal .The cel l s have hi stochemi cal and
phenotypi c features of macrophages.The cel l
cont ai n l arge amount of est erases and aci d
phosphates and show positivity for CD 68 and
alpha-1 antitrypsin.They also exhibit some of
the phenotypic features of dendritic cells such
as S100, cathepsin E, fascin and at times CD 1a.
6,7
The natural history is that of a regression and
Fig 1. a Low power view (lymph node)
showing massive distension of the sinuses
by the histocytic infilterate.
Bone marrow exami-nation revealed no evidence
of neoplastc infiltration, granulomas or parasitic
infections. Patient continued to be symptomatic
and had high ESR. Lymph node aspiration was
repeated. It reconfirmed the diagnosis. Repeat
bone marrow aspi rat i on/ bi opsy was unrem-
arkabl e. The chi l d was gi ven a course of
ant i bi ot i cs and pl anned f or bri ef l ow dose
courses of steroids.
Fig 1. b High power view showing
lymphophagocytosis (omperipolesis) by
the sinus histiocytes.
INDIAN JOURNAL OF MEDICAL & PAEDIATRIC ONCOLOGY Vol. 25 No.4, 2004 41
resurgence f ol l owed event ual l y by compl et e
resolution. No form of therapy has been found
to be of any benefit. In the Rosai and Dorfman
seri es of 34 pat i ent s, one di ed of renal
amyl oi dosi s, another after cytotoxi c therapy.
The latter form of treatment is contraindicated
in this disease. Surgical option may be reserved
f or compressi ve sympt oms.
8, 9
Course i n our
patient was more protracted / progressive with
no abatement of lymph node masses as with
about 6 mont hs f ol l ow up; rat her addi t i onal
lymph node enlargement appeared in both axill
not i ced i n recent f ol l ow up. Domi nant
nasopharyngeal involvement probably caused
recurrent epistaxis.
REFERENCES :
1. Foucar El i ot and Rosai Juan and Dorf man RF: Si nus
hi st i ocyt osi s and massi ve l ymphadeno-pat hy.
Ear, nose, t hr oat mani f estat i ons. , Ar ch Ot ol ar yngol . ,
1978,104:687-693.
2. Foulard E, Rosa J, Dorman RF. Sinus histocytosis with
massi ve l ymphadenopat hy (Rosi a- Dorf man di sease):
Review of the entity: Semin Diagn Patho 1990;7:19.
3. Lamper t F. and Lenner t K: Si nus hi st i ocyt osi s wi t h
massive lymphadenopathy. Fifeteen new cases. Cancer,
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Pediatr Pathol Mol MED 2003 Sep-Oct;22(5) :399-403
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l ymphadenopat hy (Rosai - Dorf man di sease) : Semi n
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di agnosi s of chi l dhood hi sti ocyti c l esi ons. Pedi atrol &
Dev Pathol 1998;1:216.
8. Dodson KM, Power s CN, Rei t er ER, Rosai Dor f man
di sease present i ng as synchronous nasal and i nt ra-
cranial mass Am J Otolaryngol 2003Nov-Dec;24(6),USA.
9. Raveent hi ran V, Dhanal akashmi M, Hayavadana
Rao,Vushwanath Rosai-Dorfman disease:report of a 3-
year-ol d gi rl wi th cri ti cal revi ew of treatment opti ons.
Eur J Pediatr Surg. Oct; 2003;13(5):350-4.