British Journal of Radiology (2005) 78, 845-847
© 2005 British Institute of Radiology
doi: 10.1259/bjr/23127241
Rosai-Dorfman disease: unusual cause of diffuse and massive retroperitoneal lymphadenopathy
K S Sodhi, MD
1
S Suri, MD, FICR, DABR
1
R Nijhawan, MD
2
M Kang, MD
1 and
V Gautam, MD
1
Department of 1 Radiodiagnosis and 2 Cytology & Gynaecological Pathology, Postgraduate Institute of Medical Education and Research (P.G.I.M.E.R.), Chandigarh, 160012 India
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Abstract
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Rosai-Dorfman disease (RDD), originally described as sinus histiocytosis with massive lymphadenopathy, is a rare histiocytic proliferative disorder with a distinctive microscopic appearance. Patients usually present with massive cervical lymphadenopathy. However other nodal sites (mediastinal and inguinal) are also frequently involved. The retro-peritoneum is an infrequent site of involvement. This uncommon entity should also be included in the list of differential diagnoses of diffuse and massive lymphadenopathy, which includes includes infectious/granulomatous conditions, lymphomatous and metastatic disease.
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Introduction
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A 34-year-old male patient presented with diffuse non-colicky abdominal pain, with bilateral, cervical lymphadenopathy and fever for 2 months.
On examination, the patient had bilateral large, mobile and non tender-cervical lymphadenopathy, along with axillary and inguinal lymphadenopathy. The abdomen was soft with mild hepatomegaly and normal bowel sounds. Prominent veins were seen over the chest and abdomen. The blood pressure was 130/90 mm Hg and the pulse rate was 96/min.
Laboratory investigations revealed anaemia, leukocytosis, and an elevated erythrocyte sedimentation rate. Renal and liver function tests were normal, and blood culture was negative.
Upper gastrointestinal endoscopy was normal. Chest X-ray revealed mediastinal widening suggestive of mediastinal lymphadenopathy. CT revealed multiple enlarged homogeneously enhancing lymph nodes in the pre-vascular space (Figure 1
), paratracheal regions, aortopulmonary window, and subcarinal region. There was no evidence of nodal calcification or central necrosis. The lungs were normal. CT of the abdomen (Figures 2 and 3) revealed mild hepatomegaly with multiple enlarged homogeneous lymph nodes in the gastrohepatic ligament, peripancreatic, paraortic, aortocaval, retrocaval, pre-aortic, renal hilum, mesentery, iliac and inguinal regions. These enlarged lymph nodes were seen to encase the aorta and inferior vena cava (IVC), which were displaced anteriorly. The coeliac artery, superior mesentery artery and both renal arteries were also encased. There was bilateral hydronephrosis likely due to ureteric compression, by these enlarged nodes. The intra-abdominal IVC (infrahepatic course) was also significantly compressed. No alternative cause of his abdominal pain or any other abnormality was detected.
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Figure 2. CT scan of abdomen shows multiple enlarged retroperitoneal lymphnodes, which are encasing the aorta and inferior vena cava. Enlarged nodes are also seen at the renal hilum.
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Ultrasound guided fine needle aspiration cytology from a retroperitoneal lymph node revealed lymphoid cells and many histocytes with few clusters of epitheloid cells and many of these histiocytes (Figure 4) showed marked emperipolesis features suggestive of Rosai-Dorfman disease (RDD). There was no evidence of a malignant or granulomatous pathology. Cervical lymph node biopsy also revealed similar features of prominent histiocytes with emperipolesis consistent with RDD. The patient is presently only on close observation and has not received treatment so far.
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Discussion
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RDD (also known as sinus histiocytosis with massive lymphadenopathy) is a unique disease of unknown aetiology that may occur in any age group, but is most commonly seen in children and young adults. There is a slight predilection for males (58%) and for individuals of African descent [1, 2].
Reports have established the worldwide distribution of this disorder [1]. Although peripheral lymphadenopathy is the most common mode of presentation, numerous studies have established the fact that 30–40% of affected individuals have extranodal manifestations [1–4]. The most frequent sites are the head and neck paranasal sinuses, nasal cavity, soft tissue, skin, eye and orbit, upper respiratory tract and the including salivary glands [1, 3].
Although the aetiology remains unknown, the disease is thought to be a disorder of immune regulation or response to a presumed infectious agent (HHV-6 / EBV) with its major manifestation in lymph nodes with resultant proliferation of sinusoidal histiocytes. Most efforts to identify a pathogen have culminated in indeterminate or negative results [1]. The most frequent clinical manifestation (seen in nearly 90% of patients) is bilateral, non tender, painless, cervical lymphadenopathy; "proconsular" or bull neck is a term applied to the symmetrical cervical adenopathy which resembles the findings in Hodgkin's disease of the neck.
High grade fever, night sweats, malaise, weight loss, leukocytosis, neutrophillia, and an elevated erythrocyte sedimentation rate are associated clinical and laboratory findings. Additionally, 80% of patients have a polyclonal hypergamma globulinaemia and 65% have a hypochromic or normochromic normocytic anaemia [1, 2].
Cervical lymphadenopathy is bilateral and often massive with individual nodes measuring up to 6 cm in diameter. All major lymph node groups in the neck may be involved, but there are no distiniquishing features of adenopathy by ultrasound, CT or MRI [5]. These lymph nodes are hyper (PET) metabolic and are positive on positron emission tomography [6].
Axillary and inguinal lymph nodes are enlarged in 38% and 44% of cases, respectively. These nodes are usually smaller than those in the neck and may be detected by CT scanning. Mediastinal and hilar adenopathy with a unilateral or bilateral distribution, is present in 30–40% of patients [7], however, not all affected nodes are necessarily enlarged, but the microscopic changes of RDD are present on biopsy. When the lung parenchyma is involved, there is extension from the hila along vascular septae producing accentuated and widened interstitial markings. Reticulo-nodular infiltrates are unusual.
The retroperitoneum is an infrequent site of disease. If involved, the retroperitoneal nodes may distort vessels and also produce ureteric obstruction and/or parenchymal involvement of the kidneys. Bilateral hydronephrosis was also seen in our patient. The distortion and displacement of the ureters by a fibrous reaction are similar to that seen in retroperitoneal fibrosis and malignant lymphoma [8]. Renal arteries can be stretched and splayed on arteriography, a finding that was evident on the CT scan in our patient.
The disease typically pursues an indolent clinical course. In approximately: 50% of patients, the disease resolves without appreciable sequelae, one third have residual asymptomatic adenopathy and 17% have persistent symptomatology after 5 to 10 years. Regression is usually heralded by diminution of extranodal disease. Pulsoni et al [9], in an extensive review of the literature on treatment strategies of RDD, concluded that clinical observation without treatment is advisable when possible. Surgical debulking may be necessary in the presence of vital organ compression. Chemotherapy is in general ineffective, while radiotherapy has shown limited efficacy. Others [10] have also advocated the use of long term prednisolone.
The diagnosis of RDD is readily established by the presence of the histological alterations in an affected lymph node, but frequently the diagnosis may only be made on lymph node biopsy. Distension of sinusoids by pale staining histiocytes intermixed with a variable number of mature plasma cells is said to be the characteristic feature of RDD [1].
Individual histiocytes contain one or more intact lymphocyte in the cytoplasm, where these lymphocytes continue to have free movement in a process known as emperipolesis [1, 3]. In extranodal sites, the presence of lymphocyte containing histiocytes is an important due to the pathological diagnosis.
The histiocytes of RDD are immunoreactive with S-100, an antigen found in normal antigen presenting cells and in some abnormal histiocytes, which distinguishes them from histocytes found in granulamatous disease. Although some histological features are shared between Langerhans cell histiocytosis (LCH) and RDD, there are several distinguishing features. In LCH, the histiocytes do not exhibit lympho-phagocytosis or erythrophagocytosis and eosinophils are conspicuously present. Moreover, electron microscopy often reveals the pathognomnic Birbeck granules (the cytoplasmic pentilaminar complex seen in Langerhans cells) [11].
The radiological features of RDD are not pathognomonic, but suggest a differential diagnosis of conditions producing either focal or diffuse lymphadenopathy. The appropriate imaging of patients with this disease depends upon their presenting symptoms and signs, although ultrasound and CT are most helpful.
Received for publication September 12, 2004.
Revision received October 22, 2004.
Accepted for publication March 24, 2005.
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References
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Abstract
Introduction
