Indian Journal of Nuclear Medicine
Home | About IJNM | Search | Current Issue | Past Issues | Instructions | Ahead of Print | Online submissionLogin 
Indian Journal of Nuclear Medicine
  Editorial Board | Subscribe | Advertise | Contact
Users Online: 610 Print this page  Email this page Small font size Default font size Increase font size


 
 Table of Contents     
INTERESTING IMAGE
Year : 2019  |  Volume : 34  |  Issue : 4  |  Page : 319-320  

Isolated Extranodal Rosai-Dorfman Disease on 18F-FDG PET-CT Scan


1 Department of Nuclear Medicine and Positron Emission Tomography-Computed Tomography, Mahajan Imaging Centre, Sir Ganga Ram Hospital, New Delhi, India
2 Department of Pathology, Sir Ganga Ram Hospital, New Delhi, India

Date of Web Publication23-Sep-2019

Correspondence Address:
Dr. Nitin Gupta
Department of Nuclear Medicine and Positron Emission Tomography-Computed Tomography, Mahajan Imaging Centre, Sir Ganga Ram Hospital, New Delhi - 110 060
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijnm.IJNM_152_19

Rights and Permissions
   Abstract 


Rosai–Dorfman disease (RDD) is an uncommon proliferative histiocytic disorder. Patients usually present with painless massive cervical lymphadenopathy with fever and leukocytosis. Isolated extranodal disease is rare and more severe fibrosis, fewer histiocytosis in lesions make diagnosis more difficult as compared to nodal disease. Here, we report a case of isolated extranodal RDD on fluorodeoxyglucose (FDG) positron emission tomography-computed tomography (PET-CT) scan. FDG-avidity of RDD lesions is attributable to the intense glucose dependence of the proliferating histiocytes. PET-CT scan not only demonstrates the complete staging of the disease but also provide functional information about the disease activity to guide biopsy.

Keywords: Extranodal, fluorodeoxyglucose positron emission tomography-computed tomography scan, Rosai–Dorfman disease


How to cite this article:
Gupta N, Verma R, Belho ES, Manocha A. Isolated Extranodal Rosai-Dorfman Disease on 18F-FDG PET-CT Scan. Indian J Nucl Med 2019;34:319-20

How to cite this URL:
Gupta N, Verma R, Belho ES, Manocha A. Isolated Extranodal Rosai-Dorfman Disease on 18F-FDG PET-CT Scan. Indian J Nucl Med [serial online] 2019 [cited 2019 Oct 22];34:319-20. Available from: http://www.ijnm.in/text.asp?2019/34/4/319/267510



A 52-year-old female presented with nasal mass and difficulty in breathing. In view of suspicion of mass being neoplastic, the patient was referred for fluorodeoxyglucose (FDG) positron emission tomography-computed tomography (PET-CT) scan. Maximum intensity projection image [Figure 1]a showed FDG avid lesion in the nasal region only. Axial CT and fused axial PET-CT images [Figure 1]b, [Figure 1]c, [Figure 1]d, [Figure 1]e revealed FDG avid lesion involving bilateral nasal cavity, causing erosion of nasal septum, laterally extending and involving the bilateral maxillary sinus. The corresponding coronal and sagittal CT and fused PET-CT images [Figure 1]f, [Figure 1]g [Figure 1]h, [Figure 1]i showed FDG avid lesion causing erosion of hard palate and adjacent alveolar margins. The whole-body survey showed the absence of any FDG avid visible lymph node or FDG avid visible disease elsewhere in the regions of the body surveyed. Biopsy of the FDG avid nasal mass was compatible with Rosai–Dorfman disease (RDD).
Figure 1: Maximum intensity projection image (a) showing fluorodeoxyglucose avid lesion in nasal region. Axial computed tomography (c and e) and fused axial positron emission tomography-computed tomography images (b and d) showing fluorodeoxyglucose avid lesion involving bilateral nasal cavity and corresponding coronal and sagittal computed tomography (g and i) and fused positron emission tomography-computed tomography coronal and sagittal images (f and h) showing fluorodeoxyglucose avid lesion causing erosion of hard palate and adjacent alveolar margins

Click here to view


RDD, which was first described by Rosai and Dorfman in 1969, is a rare, benign lymphoproliferative disease.[1] Concurrent nodal and extranodal disease are seen in approximately 40% of cases.[2] The sole extranodal disease is seen in approximately 20%–25%.[3] Patients with involvement of extranodal sites tend to have a fulminant course. The most common extranodal sites are skin, nasal cavity, eyes, and bone.[4] Definitive diagnosis can be performed by histopathological examination [Figure 2] which reveals intense histiocytic infiltration, emperipolesis, and positivity for S100 and CD68. Differential diagnosis are lymphoma, tuberculosis, sarcoidosis, reactive hyperplasia, and nasopharyngeal carcinoma. Few case reports of extranodal RDD, from nasal septal mucosa,[5] osseous involvement,[6] and extranodal sites[7] have been reported previously. F18-FDG PET-CT scan is a valuable imaging technique for evaluating the extent of disease, demonstrating the complete staging of the disease, and guiding the biopsy.
Figure 2: Histopathology and immunohistochemistry images of the patient. Section (a) shows tissue fragments capped with respiratory mucosa (H and E, ×4). The subepithelium section (b) shows a cellular lesion which is infiltrating the bone (H and E, ×4). The lesion (c) is composed of sheets of histiocytes, admixed with many lymphocytes and plasma cells. Some of the histiocytes are foamy (H and E, ×20). Some of the histiocytes (d) show emperipolesis (arrow) (H and E, ×40). Immunohistochemistry images show histiocytic cells positive for CD68 (e) and diffusely positive for S100 (x20) (f)

Click here to view


Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Rosai J, Dorfman RF. Sinus histiocytosis with massive lymphadenopathy. A newly recognized benign clinicopathological entity. Arch Pathol 1969;87:63-70.  Back to cited text no. 1
    
2.
Duan HG, Zheng CQ, Wang DH, Ding GQ, Luo JQ, Zang CP, et al. Extranodal sinonasal Rosai-Dorfman disease: A clinical study of 10 cases. Eur Arch Otorhinolaryngol 2015;272:2313-8.  Back to cited text no. 2
    
3.
Zhu F, Zhang JT, Xing XW, Wang DJ, Zhu RY, Zhang Q, et al. Rosai-Dorfman disease: A retrospective analysis of 13 cases. Am J Med Sci 2013;345:200-10.  Back to cited text no. 3
    
4.
Gaitonde S. Multifocal, extranodal sinus histiocytosis with massive lymphadenopathy: An overview. Arch Pathol Lab Med 2007;131:1117-21.  Back to cited text no. 4
    
5.
Akyigit A, Akyol H, Sakallioglu O, Polat C, Keles E, Alatas O. Rosai-Dorfman disease originating from nasal septal mucosa. Case Rep Otolaryngol 2015;2015:232898.  Back to cited text no. 5
    
6.
Patel MH, Jambhekar KR, Pandey T, Ram R. A rare case of extra nodal Rosai-Dorfman disease with isolated multifocal osseous manifestation. Indian J Radiol Imaging 2015;25:284-7.  Back to cited text no. 6
[PUBMED]  [Full text]  
7.
Albano D, Bosio G, Bertagna F. 18F-FDG PET/CT follow-up of Rosai-Dorfman disease. Clin Nucl Med 2015;40:e420-2.  Back to cited text no. 7
    


    Figures

  [Figure 1], [Figure 2]



 

Top
  
 
  Search
 
  
    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
    Access Statistics
    Email Alert *
    Add to My List *
* Registration required (free)  

 
  In this article
    Abstract
    References
    Article Figures

 Article Access Statistics
    Viewed32    
    Printed0    
    Emailed0    
    PDF Downloaded17    
    Comments [Add]    

Recommend this journal