AbstractAbstract

Rosai-Dorfman Disease with Extranodal LesionsKaori Hashimoto, MD 1)2); Shin Kariya, MD, PhD 3); Tetsuo Ooue, MD 2); Yasuhiko Yamashita, MD, PhD 2); Kikuko Naka, MD 2); Kazunori Nishizaki, MD, PhD 3)

1) Department of Otolaryngology-Head and Neck Surgery, Japanese Red Cross Okayama Hospital, Okayama, Japan2) Department of Otolaryngology-Head and Neck Surgery, Fukuyama City Hospital, Hiroshima, Japan

3) Department of Otolaryngology-Head and Neck Surgery, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama, Japan

Rosai-Dorfman Disease with Extranodal LesionsKaori Hashimoto, MD 1)2); Shin Kariya, MD, PhD 3); Tetsuo Ooue, MD 2); Yasuhiko Yamashita, MD, PhD 2); Kikuko Naka, MD 2); Kazunori Nishizaki, MD, PhD 3)

1) Department of Otolaryngology-Head and Neck Surgery, Japanese Red Cross Okayama Hospital, Okayama, Japan2) Department of Otolaryngology-Head and Neck Surgery, Fukuyama City Hospital, Hiroshima, Japan

3) Department of Otolaryngology-Head and Neck Surgery, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama, Japan

IntroductionIntroduction

Case ReportCase Report

ReferencesReferences

Rosai-Dorfman disease is considered a benign disease, however the

disease can be fatal because of combination of cellular infiltration and mass

forming. Very careful management is required.

DiscussionDiscussion

Figure 2: Axial and Coronal T1-weighted MR images with gadolinium show the intracranial tumors.

ConclusionsConclusions

Objectives: Rosai-Dorfman disease is a rare condition of marrow

hemopoietic stem-cell origin. Most patients present with lymphatic

involvement, whereas less than one third of cases are extranodal

presentations. Only about 5% of the extranodal cases involve bone. The

purpose of this study is to present a case of Rosai-Dorfman disease with

lymphadenopathy, intracranial tumors and bone lesions mimicking malignant

neoplasm.

Methods: Case report concerning the diagnosis and treatment of Rosai-

Dorfman disease with extranodal presentation including intracranial tumors

and multifocal bone lesions.

Results: A 53 year-old man was admitted to our hospital because of three-

month history of bilateral cervical lymphadenopathy. He also had a five-year

history of dizziness. Computed tomography, magnetic resonance imaging

and positron emission tomography showed cervical, mediastinal and

inguinal lymphadenopathy. Intracranial tumors and pelvic bone lesions were

also detected. The cervical lymph node biopsy confirmed the diagnosis of

Rosai-Dorfman disease. The patient received oral prednisolone, and his

neck pain was improved. The massive cervical lymph nodes and bone

lesions didn’t progress.

Conclusion: Rosai-Dorfman disease should be considered in the differential

diagnosis of lymphadenopathy, even though the disease is rare. The

predominant clinical manifestation of the disease is painless cervical

lymphadenopathy. Rosai-Dorfman disease is considered a benign disease,

however the disease can be fatal because of cellular infiltration and mass

forming. The treatment for Rosai-Dorfman disease has not been established.

Figure 1: Axial CT and positron emission tomography show multiple bilateral cervical lymphadenopathy (SUV MAX: 14.25).

Rosai-Dorfman disease is a rare, benign, idiopatic, and non-neoplastic

histiocytic disorder. It’s characterized by massive but painless cervical

lymphadenopathy, fever, leukocytosis, increased erythrocyte sedimentation

rate, and polyclonal hypergammaglobulinemia [1]. Most patients present

with lympthatic involvement, whereas 43% of cases are extranodal

presentations: eyelids, orbit, respiratory tract, salivary glands, skin, bone,

testis, lung, kidney, central nervous system, thyroid and gastrointestinal tract

[2]. Figure 3: Axial MR image shows multiple bone lesions of the skull.

Figure 4: (A) Photomicrograph shows the foamy histiocytes, which contain intact lymphocytes and/or plasma cells within their cytoplasm, termed emperipolesis. (hematoxylin and eosin staining); (B) The histiocytes show positive reactions for S-100 protein in immunohistochemical staining.

lymph node and intracranial tumor identified histiocytes that engulfed

lymphocytes and plasma cells in their cytoplasm (emperipolesis) (Figure 4A).

The immunohistochemical staining revealed positive S-100 staining of

histiocytes (Figure 4B). Histopathological and immunohistochemical studies

established the diagnosis of Rosai-Dorfman disease. The neck pain

appeared, and the oral administration of prednisolone improved his

symptoms. No evidence of residual or recurrent tumor in the intracranial

lesion was identified. The size of bone lesions and lymph nodes were stable.

Sinus histocytosis with massive lymphadenopathy, also known as

Rosai-Dorfman disease, is an idiopathic histiocytic proliferation affecting

lymph nodes. Histologically, the lesions consisted of variable numbers of

pale-staining histiocytes with emperipolesis. In this case, the patient had

multiple lymphadenopathy, central nervous system involvements and bone

lesions. The differential diagnosis of Rosai-Dorfman disease is broad and

includes malignant glioma, malignant lymphoma, histiocytosis X

(Langerhans cell histiocytosis), sarcoidosis, metastatic tumors,

granulomatous disease and meningioma [3][4]. Rosai-Dorfman disease has

a long natural history with mostly benign course, and special treatment is not

required. However, massive lymphadenopathy, extranodal involvement of

multiple organs (kidney, lungs and liver), immunologic abnormalities and

anemia can lead to a poor prognosis. The treatment modalities for Rosai-

Dorfman disease include corticosteroids, chemotherapy with a combination

of vinca alkaloids and alkylating agents, low-dose interferon, antibiotic

therapy, radiation therapy and surgical treatment [5]. However, there is no

established treatment for Rosai-Dorfman disease.

A 53 year- old man was admitted to our hospital because of three-

month history of bilateral massive painless cervical lymphadenopathy and

five-year history of dizziness. On physical examination, he had enlarged

bilateral cervical lymph nodes. He had normal vital signs and no

neurological abnormality. Hematological and biochemical examinations were

normal without leukocytosis and hypergammaglobulinemia. Computed

tomography, magnetic resonance imaging and positron emission

tomography showed cervical, mediastinal and inguinal lymphadenopathy.

Intracranial tumors and multiple lytic bone lesions of the skull and pelvic

bone were also detected (Figure 1-3). The patient underwent cervical lymph

node biopsy and intracranial tumor resection. The histological examinations

of the cervical

1) Rosai J, Dorfman RF. Arch Pathol. 1969 Jan;87(1):63-70.

2) Foucar E, Rosai J, Dorfman R. Semin Diagn Pathol. 1990 Feb;7(1):19-73.

3) Loh SY, Tan KB, Wong YS, et al. J Bone Joint Surg Am. 2004 Mar;86-A(3):595-8.

4) Fukushima T, Yachi K, Ogino A, et al. Neurol Med Chir (Tokyo). 2011;51(2):136-40.

5) Komp DM. Semin Diagn Pathol. 1990 Feb;7(1):83-6.

A B

Figure 5: Frontal (left) and lateral (right) view of CT-angiography show intracranial tumor with hypervascularity.