British Journal of Radiology (2004) 77, 363-364
© 2004 British Institute of Radiology
doi: 10.1259/bjr/66863351
A case of multiple extra-axial masses
N Khan and
L D R Tsatsi
Medical University of Southern Africa, 209 Milner Street, Waterkloof 0181, Pretoria, South Africa
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Introduction
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A 59-year-old female patient presented with fever, right nasal blockage and multiple neck masses. On examination she had massive cervical lymphadenopathy, a right nasal mass and a mass involving the larynx. Direct laryngoscopy under general anaesthesia showed markedly oedematous vocal cords and a mainly left sided diffuse subglottic granular mass involving more than two thirds of the posterior tracheal wall. Tracheostomy was performed and multiple biopsies were taken of the nodes at this stage. She was then lost to follow up and re-presented after 5 years with deafness and left facial nerve paralysis.
What abnormalities are demonstrated on CT (Figure 1
) and MRI (Figures 2 and 3)? Considering the history, cytology (Figure 4) and imaging findings suggest a diagnosis.
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Answer
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There are homogeneously enhancing dural based left parafalcine, right parasellar and left cerebellopontine angle soft tissue masses on the post contrast CT (Figure 1
). Coronal and axial MRIs show that the masses are low signal on T1 weighted images (Figure 2), and enhance homogeneously after post gadolinium administration (Figure 3).
Several biopsies of the lymph nodes over preceding years revealed distended sinusoids containing abundant histiocytes (Figure 4), lymphophagocytosis was also noted. A diagnosis of sinus histiocytosis with massive cervical lymphadenopathy was made.
Rosai-Dorfman disease (RDD) also known as sinus histiocytosis was first described in 1969 by Rosai and Dorfman [1], as a benign histiocytic proliferative disorder with massive lymphadenopathy. Microscopically the enlarged lymph node sinuses contain large histiocytes with phagocytosed lymphocytes—emperipolesis [2]. Since then more than 400 cases have been reported. It is a benign disorder characterized by dilatation of the lymphatic sinuses due to proliferating histiocytes. In its classic form the disease predominantly affects children and young adults, with a slight male predominance [1, 3]. Lymphadenopathy is usually cervical with fever and leucocytosis. Inguinal and axillary lymphadenopathy is also a common feature. The extent of the lymphadenopathy is also striking with nodes larger than 6 cm noted [4], in some cases as in this patient leading to respiratory compromise. Rosai and Dorfman listed 43% of patients as having extranodal involvement, in their latest review of the disease [4]. These sites include the respiratory tract, skin, sinuses, nasal cavity, orbits, eyelid, skeletal system, salivary glands and testis [4, 5]. Involvement of the central nervous system is rare [3] and isolated involvement of the central nervous system is even rarer [1, 4]. When the central nervous system is involved, the disease typically manifests within the epidural or subdural compartment of the spine or skull base [4]. Suprasellar involvement has been reported in only three cases [3]. There is a predilection for males with the central nervous system variant of RDD, typically presenting during the 4th to 5th decade [1], in contrast to nodal disease, with a mean age of 37.5 years compared with 20.6 years for nodal disease [6]. The aetiology and pathogenesis of this disease are still unknown. It often occurs in the setting of non-specific immune dysfunction, many cases occur after a viral illness [5]. Serological evidence of Epstein-Barr virus is documented in about 50% of cases [5]. The treatment includes surgery, radiation therapy, steroids and chemotherapy [3–5]. Some have complete resolution of the disease, in others the condition persists but is asymptomatic [5]. The disease most often has a chronic, relapsing course although fatal cases have been reported [3, 4].
In conclusion although intracranial RDD is a rare entity, it should be included in the differential diagnosis of dural-based lesions that clinically and radiologically resemble meningioma or any diffusely enhancing extra-axial mass.
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Acknowledgments
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I would like to acknowledge Prof. E J Raubenheimer head of anatomical pathology, department of maxillofacial surgery for his contribution of a histology slide and report and Dr M E Kisansa for helping in the final modifications.
Received for publication July 8, 2003.
Revision received January 5, 2004.
Accepted for publication February 11, 2004.
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References
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- Andriko JA, Morrison A, Colegial CH, Davis BJ, Jones RV. Rosai-Dorfman disease isolated to central nervous system: a report of 11 cases. Mod Pathol 2001;14:172–8.[CrossRef][Medline]
- Udono H, Fukuyama K, Okamoto H, Tabuchi K. Rosai Dorfman disease presenting multiple intracranial lesions with unique findings on magnetic resonance imaging. Case report. J Neurosurgery 1999;91:335–9.[Medline]
- Woodcock RJ Jr, Mandell JW. Sinus histiocytosis (Rosai-Dorfman disease) of the suprasellar region: MR imaging findings—a case report. Radiology 1999;213:808–10.[Abstract/Free Full Text]
- Wang E, Anzai Y, Paulino A. Rosai-Dorfman disease presenting with isolated bilateral orbital masses report of two cases. AJNR 2001;22:1386–8.[Abstract/Free Full Text]
- Kim M, Provias J, Bernstein M. Rosai-Dorfman disease mimicking multiple meningiomas. Neurosurgery 1995;36:1185–8.[Medline]
- Deodhare SS, Ang LC, Bilbao JM. Isolated intracranial involvement in Rosai Dorfman disease. A report of two cases and literature review. Arch Pathol Lab Med 1998;122:161–5.[Medline]
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