Rosai-Dorfman Disease

Diagnosis

Rosai-Dorfman Disease

History

Fifteen month-old boy presenting himself with upper respiratory tract infection and bilateral painless cervical lymph nodes. The patient was otherwise asymptomatic. Laboratory findings demonstrated microcytic anemia. F18-FDG PET/CT requested for staging, following left cervical lymph node biopsy.

Findings

Multiples hypermetabolic lymph nodes, predominant in both cervical regions but also present in both hila, sub-carenal region, left axilla, splenic ahd hepatic hila. No evidence of bone lesions.

DDx

Lymphoma

Histiocytosis

Discussion

Rosai Dorfman disease  (RDD)  or Sinus Histiocytosis with Massive Lymphadenopathy ( SHML) is a rare disease. It is characterized by massive, painless bilateral cervical adenopathies. In some patients, more extensive disease can be found. However, affected lymph nodes will be less enlarged than cervical adenopathies, as in our patient. 

Extranodal disease will be found in 40% of patients. Some may present themselves with isolated cutaneous RDD. Other affected organs are CNS, lacrymal glands, salivary glands, ENT region and bone.  Thymus, liver and pancreas are rarely involved.

Associated symptoms are low grade fever without alteration of general condition. Laboratory findings will include anemia, leukocytosis, increased ESR, hypergammaglobulinemia.

Spontaneous resolution is frequent but relapse can occurs and chronic disease not uncommon. In patients with extensive disease, treatment with steroids or clofarabine or imatinib may be needed. Due to the extension of cervical disease, the patient was put on steroids with good  clinical response.

Typical findings on histopathology is the presence of numerous intrasinusal histiocytes with large clear cytoplasm. A characteristic finding is emperipolesis  with phagocytosis of mature lymphocytes and plasma cells by histiocytes.  In RDD, histiocytes are positive for S100 and CD163 but negative for CD1a staining, the latter being typical of Langerhans cell histiocytosis.

All RDD lesions visualized  with PET/CT showed significant avidity for FDG. Only one pediatric patient imaged with FDG PET/CT  has been reported to date.

Special thanks to Dre Anne-Sophie Carret

References

Sinus Histiocytosis with Massive Lymphadenopathy: a pseudolymphomatous benign disorder. Analysis of 34 cases. J Rosai, R Dorfman. Cancer 1972;30:1174-1188

FDG PET of Rosai Dorfman  disease of the thymus.  R Lim, C Wittram,  JA  Ferry,  JAO Sherpard. AJR 2004;182:514

Rosai-Dorfman  disease: FDG PET/CT in a patient presenting with pyrexia and cervical adenopathy. ATE Hock, MTM Long, K Sittampalam, DNC Eng Clin Nucl Med 2010;35:576-578

Rosai-Dorfman Disease in Neuroradiology: Imaging Findings in a Series of 10 Patients. OA Raslan, D Schellingerhout, GN Fuller, LM Ketonen. AJR 2011;196:W187-W193

FDG PET/CT findings in purely cutaneous Rosai Dorfman disease. Jei-Yie Huang, Ching-Chu Lu, Cheng-Hsiang Hsiao,  Kai-Yuan Tzen,Clin Nucl Med 2011;36:e13-e15

The use of FDG-PET/CT in extranodal Rosai–Dorfman disease of bone Jeffrey S. Tsang , Marina-Portia Anthony , Maria P. Wong, C. S. Wong Skelet Radiol 2012;41:715-717

18F-FDG PET/CT Findings in a Patient With Isolated Intracranial Rosai-Dorfman Disease. Emmanuel Deshayes,  Jean-Philippe Le Berre, Emmanuel Jouanneau,  Alexandre Vasiljevic, Gerald Raverot, Pascal Seve. Clin Nucl Med 2013;38:e50-e52

18F-FDG PET/CT Imaging Features of Rosai Dorfman Disease A Rare Cause of Massive Generalized Lymphadenopathy Sellam Karunanithi,  Harmandeep Singh, Punit Sharma, Niraj Naswa,  Rakesh Kumar. Clin Nucl Med 2014;36:268-269

Rosai-Dorfman Disease Mimics Lymphoma on FDG PET/CT in a Pediatric Patient. Bin Liu,  Nam Ju Lee, Hansel J. Otero,   Sabah Servaes, Hongming Zhuang, . Clin Nucl Med 2014;39:206-208

18F-FDG PET/CT Follow-up of Rosai-Dorfman Disease Domenico Albano, , Giovanni Bosio, , Francesco Bertagna, Clin Nucl Med 2015;40:e420-e422

18F-FDG PET/CT of Widespread Rosai-Dorfman Disease Varun Singh Dhull, Averlicia Passah, Neelima Rana, Kavneet Kaur, Madhavi Tripathi,  Rakesh Kumar. Clin Nucl Med 2016;41:57-59

18F-FDG PET/CT Imaging of Extranodal Rosai-Dorfman Disease with Hepatopancreatic Involvement - A Pictorial and Literature Review. Faiq Shaikh , Omer Awan , Sohaib Mohiuddin , Saleem Farooqui , Salman A. Khan , William McCartney. Cureus 2015;7(12): e392. DOI 10.7759