British Journal of Radiology (2006) 79, e117-e119
© 2006 British Institute of Radiology
doi: 10.1259/bjr/83681836
A rare case of extranodal Rosai-Dorfman disease
D Chopra, MBBS, DMRE
1
W E Svensson, LRCP&SI, FRCSI, FRCR
2
P Forouhi, MBChB, FRCS
3 and
S Poole, MBChB
4
1 5 Baronsmede Court, Baronsmede, London W5 4LN, 2 Nuclear Medicine, Imaging Department, Charing Cross Hospital, Fulham Palace Road, Hammersmith, London W6 8RFDepartments of , 3Surgery 4Histopathology, Ealing Hospital NHS Trust, Uxbridge Road, Southall, Middlesex UB1 3HW, UK
 |
Abstract
|
|---|
Rosai-Dorfman disease is a rare, benign histiocytic proliferative disorder which commonly affects the lymph nodes. Extranodal involvement along with concomitant nodal disease occurs in about 43% of cases. Isolated extranodal Rosai-Dorfman disease is relatively uncommon. We report this rare case of isolated extranodal Rosai-Dorfman disease and its ultrasound findings.
|
Introduction
|
|---|
Rosai-Dorfman disease, or sinus histiocytosis with massive lymphadenopathy, was first described as a distinct clinicopathological entity in 1969 [1]. It primarily involves the lymph nodes. In 43% of cases, extranodal sites are involved simultaneously and in only 23% does isolated extranodal Rosai-Dorfman disease occur [2]. The extranodal sites are skin, eye and adnexae, paranasal sinuses, genitourinary system, CNS, bone, breast, soft tissues and thyroid [2–8]. We describe a case of extranodal sinus histiocytosis in the soft tissue of left posterior axillary wall.
|
Case history
|
|---|
A 47-year-old Asian man presented with a large, solitary, painless lump in the left posterior axillary fold which had been present and unchanged for 8 months. There was no history of other lumps or any constitutional symptoms. Past history of recently diagnosed diabetes was noted. On examination, a 5 cmx4 cm sized firm, non-tender, mobile lump was felt in the subcutaneous region of left posterior axillary wall. No evidence of lymphadenopathy was present and there were no abnormal findings on clinical examination and laboratory investigations. Ultrasound examination (8–13 MHz linear array probe, ACUSON Sequoia; Siemens, Erlangen, Germany) of left axillary region showed an irregular area of heterogeneous echogenicity with mixed posterior acoustic shadowing and enhancement in the posterior axillary wall. The appearance was slightly lobulated with multiple cystic areas with internal echoes (Figure 1
). Colour Doppler examination showed blood flow in and around this lesion, but not in the cystic areas (Figure 2). The patient underwent wide local excision of the lesion and histology proved it to be extranodal Rosai-Dorfman disease where characteristic large histiocytes with abundant pale cytoplasm (Figure 3) and emperipolesis (Figure 4) were seen. Immunohistochemistry showed that the histiocytes were S-100 protein, CD68 and lysozyme positive, also characteristic of Rosai-Dorfman disease.
View larger version (61K):
[in this window]
[in a new window]
|
Figure 1. A 3.8 cmx2.1 cm sized area of heterogeneous echotexture with cystic change in the left axilla.
|
|
|
Discussion
|
|---|
Rosai-Dorfman disease is a rare, idiopathic, non-neoplastic, lymphoproliferative disorder known since 1969 when four cases were described [1]. Typically, it is characterized by bilateral cervical lymphadenopathy with fever, leukocytosis, increased erythrocyte sedimentation rate and hypergammaglobulinaemia [2]. It is a multifocal and multisystem condition in 43% of cases. The extranodal sites include skin, soft tissues, respiratory system, genitourinary system, bones, CNS, orbit, thyroid and breast [2–8]. The extranodal involvement usually occurs with lymphadenopathy, but in about 23% of patients isolated extranodal disease occurs [2]. Male to female ratio is 1.4:1 [2, 3]. It occurs in children as well as in adults and mean age of incidence is 20.6 years [2]. Patients with soft-tissue involvement tend to be older than patients who present with other extranodal sites [3].
The histological appearance is characteristic for this disease and is essentially similar regardless of the site of occurrence. The microscopic hallmark is large histiocytes with abundant pale cytoplasm (Figure 3
), round vesicular nuclei and showing lymphocytophagocytosis or emperipolesis (Figure 4). Extranodal cases are diagnosed easily if a classical nodal disease has been documented. However, in absence of diagnostic lymph nodes, a greater number of characteristic histiocytes with emperipolesis are required for extranodal disease to be defined [2]. Immunohistochemical diagnostic criteria of Rosai-Dorfman disease is cytoplasmic positivity of histiocytes for S-100 protein.
The aetiology, pathogenesis and natural history of Rosai-Dorfman disease are unknown. An immune-mediated origin is proposed [9]. At present, it is considered a benign or reactive proliferation showing spontaneous regression. There is no ideal therapeutic regimen for this disease [10]. The treatment options range from surgery, radiotherapy and steroids to chemotherapy. Our patient underwent excisional biopsy in order to establish the diagnosis as the ultrasound features were worrying and malignancy could not be excluded with ultrasound alone.
With Rosai-Dorfman being a multiorgan disorder, the clinical manifestations vary and so do the radiological features, depending on the site involvement [5]. The imaging features are occasionally confounding because of the differential diagnostic possibilities. The diagnosis of Rosai-Dorfman is seldom considered, largely attributable to the rarity of the disease and second due to low index of suspicion. To conclude, Rosai-Dorfman disease should be considered in the differential diagnosis of granulomatous infection, pseudogranulomatous lesion and malignancy.
In our case, ultrasound helped to delineate the soft-tissue swelling showing heterogeneous echotexture with cystic areas and raised the possibility of something more than just a simple benign lesion. To our knowledge this is a rare case of isolated extranodal Rosai-Dorfman involving the soft-tissue where ultrasound and colour Doppler alone has been used to evaluate the disease.
Received for publication December 3, 2003.
Revision received May 16, 2005.
Accepted for publication July 20, 2005.
|
References
|
|---|
- Rosai J, Dorfman RF. Sinus histiocytosis with massive lymphadenopathy: a newly recognised benign clinicopathological entity. Arch Pathol 1969;87:63–70.[Medline]
- Foucar E, Rosai J, Dorfman RF. Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman Disease): review of the entity. Semin Diagn Pathol 1990;7:19–73.[Medline]
- Brenn T, Calonje E, Granter SR, Leonard N, Grayson W, Fletcher CD, et al. Cutaneous Rosai-Dorfman disease is a clinical entity. Am J Dermatopathol 2002;24:385–91.[CrossRef][Medline]
- George J, Stacy G, Peabody T, Montag A. Rosai-Dorfman disease manifesting as a solitary lesion of the radius in a 41 year old woman. Skeletal Radiol 2003;32: 236–9.Epub 2003 Mar 08[Medline]
- McAlister WH, Herman T, Dehner LP. Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease). Pediatr Radiol 1990;20:425–32.[CrossRef][Medline]
- Deshmukh RR, Kumar V, Kumbhari D. Sinus histiocytosis of the thyroid with massive lymphadenopathy (Rosai- Dorfman Disease). J Indian Med Assoc 2003;101:597–8.[Medline]
- Ture U, Seker A, Bozkurt SU, Uneri C, Sav A, Pamir MN. Giant intracranial Rosai-Dorfman disease. J Clin Neurosci 2004;11:563–6.[CrossRef][Medline]
- Green I, Dorfman RF, Rosai J. Breast involvement by extranodal Rosai-Dorfman disease: report of seven cases. Am J Surg Pathol 1997;21:664–8.[CrossRef][Medline]
- Eisen RN, Buckley PJ, Rosai J. Immunophenotypic characterisation of sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease). Semin Diagn Pathol 1990;7:74–82.[Medline]
- Raveenthiran V, Dhanlakshmi M, Hayavadana Rao PV, Vishwanathan P. Rosai-Dorfman disease: report of a 3 year old girl with review of treatment options. Eur J Paediatric Surg 2003;13:350–4.[CrossRef]
Top
Abstract
Introduction
