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  Table of Contents    
Year : 2017  |  Volume : 60  |  Issue : 3  |  Page : 435-436
Primary intraosseous Rosai-Dorfman disease: Long-term follow-up with recurrence after surgical excision

1 Department of Pathology, Faculty of Medicine, University of Colombo, Colombo, Sri Lanka
2 Orthopedic Unit, National Hospital of Sri Lanka, Colombo, Sri Lanka

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Date of Web Publication22-Sep-2017

How to cite this article:
Priyani AA, Shirani SA, Amila G, Asiri GW. Primary intraosseous Rosai-Dorfman disease: Long-term follow-up with recurrence after surgical excision. Indian J Pathol Microbiol 2017;60:435-6

How to cite this URL:
Priyani AA, Shirani SA, Amila G, Asiri GW. Primary intraosseous Rosai-Dorfman disease: Long-term follow-up with recurrence after surgical excision. Indian J Pathol Microbiol [serial online] 2017 [cited 2021 Oct 16];60:435-6. Available from: https://www.ijpmonline.org/text.asp?2017/60/3/435/215389

   Introduction Top

 Rosai-Dorfman Disease More Details (RDD), or sinus histiocytosis with massive lymphadenopathy, is a rare, histiocytic proliferative disorder, with some patients presenting as isolated extranodal disease. Isolated intraosseous involvement of RDD is very rare. Follow-up information of intraosseous RDD is even rare.

We report a case of isolated intraosseous RDD presenting as a lytic lesion in the tibia with follow-up information for 75 months after surgical excision.

   Case Report Top

A 28-year-old female presented with a painful swelling over the anterior aspect of the right leg below the knee joint. Pain has worsened progressively and has continued to awake her at night. However, she had been able to participate in day-to-day activities. The pain was partly relieved by nonsteroidal anti-inflammatory drugs. She had no other symptoms.

Musculoskeletal and neurological examination of the right leg was normal except for a tender lumpiness over the anterior aspect of the proximal tibia. There was no lymphadenopathy. No hepatosplenomegaly or other significant physical sign was elicited.

X-ray showed a poorly defined lytic lesion in the right proximal tibial metaphysis extending into the epiphysis. Radiological features were suggestive of chronic osteomyelitis or a neoplasm.

Magnetic resonance imaging (MRI) of the right leg showed a contrast enhancing bone lesion at the anterior aspect of the right tibia. The lesion extended into the medullary cavity and into the soft tissue forming a mass surrounding the bone lesion. There was no periosteal reaction. MRI appearance was suggestive of a low-grade neoplasm. The blood counts and the urinalysis were normal.

The surgical curettage of the lesion showed fragmented fleshy material with no necrosis or hemorrhage. Microscopic examination showed nodular infiltrates of sheets of foamy histiocytes, plasma cells, and lymphocytes [Figure 1]a. The histiocytes contained round to oval vesicular nuclei with abundant amphophilic, granular, and foamy cytoplasm. The nuclei did not demonstrate nuclear lobation, indentation, or longitudinal grooving. Some histiocytes showed emperipolesis. The phagocytized cells were lymphocytes and red cells [Figure 1]b. The infiltrate extended in between bony trabeculae and into adjacent soft tissue. Histochemical stains for fungi and acid-fast bacilli were negative. The histiocytes were positive for S100 and CD68 monoclonal antibodies on immunoperoxidase staining [Figure 2]a and [Figure 2]b. Histopathological and immunohistochemical appearance was consistent with the diagnosis of RDD.
Figure 1: Microscopy of the lesion (a) Sheets of foamy histiocytes admixed with plasma cells and lymphocytes (H and E, ×200). (b) Histiocytes with phagocytized lymphocytes and neutrophils (H and E, ×400)

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Figure 2: Immunohistochemical staining demonstrating S100 (a) and CD68 (b) positivity of histiocytes (Immunoperoxidase, ×400)

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The patient was disease free after surgery and successfully completed her first pregnancy. Thirty-two months after the first surgery, she presented with similar symptoms. There was no lymphadenopathy. The MRI of the right leg showed a more aggressive lytic lesion involving the bone and surrounding soft tissue. A wide local excision of the lesion with scooping of the medullary cavities was done to ensure complete excision. Following confirmation of diagnosis and completeness of excision by histology, reconstruction with transposition of ipsilateral fibula with vascular pedicle and plating was done as the second stage.

The excised lesion showed similar histopathological and immunohistochemical profile to previously diagnosed RDD at the same site and confirmed a recurrence. The patient is disease free until 43 months of postsurgical follow-up after second surgery.

   Discussion Top

Histopathological examination in combination with immunohistochemistry is important in the diagnosis of intraosseous RDD as it can mimic a low-grade neoplasm or an inflammatory lesion both clinically and radiologically.

Pathological characteristics of RDD include large histiocytes containing abundant eosinophilic cytoplasm and demonstrating emperipolesis of lymphocytes, plasma cells, and neutrophils. The histiocytes are admixed with a variable number of inflammatory cells mainly lymphocytes and plasma cells. Dense aggregates of neutrophils resembling microabscesses and necrotizing granulomata have been reported.[1] The histiocytes stain positively with immune histochemical staining of S100 protein and CD68. Granulomatous infections due to fungi and mycobacteria come into the differential diagnosis and need exclusion by negative histochemical stains.

Limited data are available regarding clinical behavior and follow-up of extranodal- RDD. Follow-up information of intraosseous RDD treated with curettage or surgical resection is available in a few cases.[1],[2],[3],[4],[5],[6] Sixteen of these cases have not shown any evidence of disease after surgery for a follow-up period ranging from 9 to 108 months.[1],[2],[3],[4],[5],[6] Two cases (8.9%) have shown stable disease at the primary site until 27 and 30 months after surgery.[1],[4] New lesions elsewhere, in soft tissue in two cases (in 2 and 4 months), in bones in two cases and in a lymph node in one case have been appeared in five patients with follow-up available ranging from 8 to 60 months.[1],[4]

   Conclusion Top

RDD disease should be considered in the differential diagnosis of low-grade intraosseous lesions at any age. Histopathological examination of tissue is diagnostic. The patients should be closely followed up even after surgical excision as recurrences at the primary site as well as new lesions elsewhere can appear even several years after the primary disease.


We would like to acknowledge the technical staff, Department of Pathology, Faculty of Medicine, University of Colombo, Sri Lanka.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

   References Top

Demicco EG, Rosenberg AE, Björnsson J, Rybak LD, Unni KK, Nielsen GP. Primary Rosai-Dorfman disease of bone: A clinicopathologic study of 15 cases. Am J Surg Pathol 2010;34:1324-33.  Back to cited text no. 1
Kang RW, McGill KC, Lin J, Gitelis S. Chronic ankle pain and swelling in a 25-year-old woman: An unusual case. Clin Orthop Relat Res 2011;469:1517-21.  Back to cited text no. 2
Orvets ND, Mayerson JL, Wakely PE Jr. Extranodal Rosai-Dorfman disease as solitary lesion of the tibia in a 56-year-old woman. Am J Orthop (Belle Mead NJ) 2013;42:420-2.  Back to cited text no. 3
Patterson FR, Rooney MT, Damron TA, Vermont AI, Hutchison RE. Sclerotic lesion of the tibia without involvement of lymph nodes. Report of an unusual case of Rosai-Dorfman disease. J Bone Joint Surg Am 1997;79:911-6.  Back to cited text no. 4
Tripathy K, Misra A, Sahu AK, Patnaik K. Extranodal Rosai-Dorfman disease in a carpal bone. Indian J Orthop 2012;46:487-9.  Back to cited text no. 5
[PUBMED]  [Full text]  
Walczak BE, Halperin DM, Bdeir RW, Irwin RB. Orthopaedic case of the month: A 50-year-old woman with persistent knee pain. Clin Orthop Relat Res 2011;469:3527-32.  Back to cited text no. 6

Correspondence Address:
Amarathunga A. H. Priyani
Department of Pathology, Faculty of Medicine, University of Colombo, No 25, Kinsey Road, Colombo 8
Sri Lanka
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/IJPM.IJPM_509_16

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