AUTHOR(S): Kim, Miriam, M.D.;
Provias, John, M.D.; Bernstein, Mark, M.D.
Division of Neurosurgery, Department of Pathology,
The Toronto Hospital, University of Toronto,
Toronto, Ontario, Canada
Neurosurgery 36; 1185-1187, 1995
ABSTRACT: ROSAI-DORFMAN DISEASE IS a
rare idiopathic histioproliferative disease affecting
the lymph nodes. Extranodal involvement has also
been recognized, but central nervous system
manifestations are extremely rare. Only 12 patients
with intracranial involvement have been reported
previously, and they all presented with clinical and
radiological findings suggestive of meningioma. We
report multiple meningeal nodules in a patient
presenting with seizures whose pathological findings
at surgery confirmed the diagnosis of Rosai-Dorfman
disease. A review of all previously reported
intracranial lesions is presented.
KEY WORDS: Lymphadenopathy; Meningioma;
Rosai-Dorfman disease; Sinus histiocytosis
In 1969, Rosai and Dorfman (11) described a newly
recognized benign histioproliferative disease
characterized clinically by massive cervical
lymphadenopathy, fever, and leukocytosis and
characterized pathologically by enlarged lymph node
sinuses containing large histiocytes with
phagocytosed lymphocytes. They named the entity
sinus histiocytosis with massive lymphadenopathy
(SHML or Rosai-Dorfman disease). Since its original
description, Rosai-Dorfman disease has become a
well-established clinicopathological entity whose
registry now numbers more than 400 cases (5). The
patients tend to be young at onset, tend to have
painless, bilateral, massive lymphadenopathy, and
usually tend to have a protracted uncomplicated
clinical course, despite various therapeutic
interventions (4,11-13). Approximately one-third have
concurrent extra nodal involvement, most commonly
in the skin, salivary glands, and upper respiratory
tract (13). Only 12 cases of intracranial involvement
have been described previously (1-3,5-7,9,14-16). They are
all dural-based lesions presenting clinically and
radiologically as meningioma. We present a patient
with multiple extra-axial convexity lesions,
confirmed to be Rosai-Dorfman disease at surgery by
pathological examination. A review of the previously
reported intracranial manifestations is also presented.
CASE REPORT
A 50-year-old pesticide controller born in Trinidad
presented after suffering from a first-time
tonicoclonic seizure. He had been healthy previously,

had not been receiving medications, and had no drug
allergies or significant family history. He denied
having a recent fever or chills. His physical
examination revealed a left homonymous hemianopia
and a left hemisensory inattention in the arm. The rest
of his neurological examination was normal. He was
afebrile with no lymphadenopathy. Routine
laboratory investigations, including a complete blood
count, were normal. A computed tomographic scan
with contrast showed two lesions in the right parietal
convexity. A magnetic resonance imaging scan better
defined these lesions to be dural based, gadolinium
enhancing nodules with some mass effect (Fig. 1).
The patient was diagnosed preoperatively with
multiple meningioma and began receiving Decadron
and Dilantin. He underwent a parietal craniotomy for
the complete removal of both lesions.
Postoperatively, his neurological deficits gradually
improved. At 6 months, he is neurologically intact
and the follow-up magnetic resonance imaging scan
was negative for a residual or recurrent lesion.
Pathological findings
Grossly, the tumors were firm and xanthochromic
with some adherence of the anterior lesion to the
underlying brain, but there was a good plane between
the posterior lesion and the brain. Microscopic
examination showed predominant histiocytes with
abundant pale pink cytoplasm and vesicular nuclei,
some plasma cells, and lymphocytes. The histiocytes
stained strongly for S-100, and many showed
emperipolesis, i.e., phagocytosed but intact
lymphocytes (Fig. 2). Ultrastructural examination by
electron microscopy confirmed the predominant
tumor cells to be single or multinucleated histiocytes,
often with prominent vacuoles of lipid accumulation
in the cytoplasm (Fig. 3). There were no
microorganisms, necrosis, or granuloma formation.
DISCUSSION
Since the original description, Rosai-Dorman
disease has become a definite clinicopathological
entity. All ages are affected, but 80% of patients are
20 years old or younger at onset. Ninety percent of
the patients have cervical adenopathy that is painless,
bilateral, and massive (12). Anemia, leukocytosis,
polyclonal hypergammaglobulinemia, and raised
erythrocyte sedimentation rate are common but are
not always present. One-third of the patients have
extra nodal involvement, such as the eyes and its
appendages, skin, upper respiratory tract, bone
salivary gland, testis, and meninges of the cranium
and spine (5,13). Occasionally, only extra nodal disease
has been found, and for these patients, the lack of
lymphadenopathy makes the term sinus histiocytosis
with massive lymphadenopathy inappropriate.
The origin and pathogenesis of this disease are still
unknown. It often occurs in the setting of nonspecific
immune dysfunction, many cases occur after a viral
illness (12). Although the diagnosis is based on the
morphological characteristics, there are other disease
entities with a predominance of histiocytes, as in
certain cases of leukemia, lipid storage disease, and
histiocytosis X. The classical findings of dilated

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Neurosurgery 1992-98
June 1995, Volume 36, Number 6
1185 Rosai-Dorfman Disease Mimicking Multiple
Meningioma: Case Report
Case Report

Received, August 5, 1994.
Accepted, November 16, 1994.
Reprint requests: Mark Bernstein, The Toronto
Hospital-Western Division, Mc 2-405, 399 Bathurst
Street, Toronto, Ontario, Canada M5T 2S8.
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COMMENT
The authors present a patient with a rare disorder
called Rosai-Dorfman disease. Magnetic resonance
imaging was employed for the diagnosis. Given the
increasing availability of high resolution imaging, I
believe it is important for neurosurgeons to keep

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nodal sinuses filled with foamy histiocytes,
phagocytosed lymph nodes (emperipolesis), and
plasma cells make this disease easily distinguishable
(4)
. Emperipolesis is not a unique phenomenon to
Rosai-Dorfman disease and has been seen in both
normal and leukemic processes (11), but it appears to
be a prerequisite for the diagnosis.
One of the largest series reported described 200
patients, of whom 3 had intracranial manifestations
(6)
. Nine other case reports described intracranial
involvement (Table 1). The average age of these 12
patients was 35. The presenting symptoms depended
on the location of the lesions and were manifested by
cranial nerve deficits or nonlocalizing symptoms of
raised intracranial pressure or seizures. The
preoperative diagnosis was usually meningioma.
More than half the patients had no clinical lymph
node involvement. One other patient (Patient 9) also
had more than one intracranial lesion. Most were
treated with surgical excision, albeit subtotal. The
follow-up period varied from 1 month to 8 years in
the seven patients for whom this information is
available. The lesion appears to have grown in only
one patient (Patient 5). We know from information
available about patients with nonintracranial RosaiDorfman disease, that the clinical course tends to be
protracted and unaltered by treatment, including
radiation and chemotherapy (5,8,13). Some have
complete resolution of the adenopathy, whereas in
others the condition persists but is asymptomatic. The
adenopathy is rarely intermittent and recurrent.
In conclusion, Rosai-Dorfman disease is a wellrecognized clinicopathological entity, which rarely
affects the central nervous system where it mimics
the clinical and radiological appearance of
meningiomas (as in our patient). Although enough
follow-up information is not yet available, it appears
that these are slow-growing lesions for which surgical
excision of symptomatic lesions appears to be the
appropriate treatment.

clearly in mind that a number of lesions may mimic
meningiomas. It is presumed that the appropriate
treatment for these intracranial lesions is surgical
resection, although there is little information
regarding the natural history of these dural base
lesions.

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William F. Chandler
Ann Arbor, Michigan

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Figure 2. Population of large histiocytes with
phagocytosed lymphocytes (emperipolesis)
(arrowheads). Surrounding lymphocytes awaiting
phagocytosis (arrow) (hematoxylin and eosin;
original magnification, ×250).

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Figure 1. Gadolinium-enhanced magnetic resonance
imaging scan showing two enhancing dural-based
lesions with mild mass effect.

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Figure 3. Cytoplasm of a histiocyte showing
prominent lipid vacuolation and an emperipoletic
lymphocyte (electron microscopy; original
magnification, ×6000).

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Table 1. Intracranial Rosai-Dorfman Diseasea
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