Rosai-Dorfman Disease

By
  • Dr Sophie Turpin
  • Dr Marc-André Levasseur
  • Dr Raymond Lambert

Publication date: July 28, 2016 | Updated on July 28, 2016

Report

Diagnosis

Rosai-Dorfman Disease

History

Eleven year-old boy presenting a painless right sub-mandibular mass for 1 week.   He was otherwise asymptomatic. Diagnosis of adenitis. As there were no modifications after antibiotic treatment, biopsy was performed. F18-FDG PET/CT requested for staging.

Findings

Hypermetabolic right cervical mass measuring 3 x 2 cm, SUV max 5.9. Additional small hypermetabolic lymph node, 1.5 x 0.8 cm, SUV max 4.5, station I-B. No other lesion.

DDx

Lymphoma

Infectious adenitis

Discussion

Rosai Dorfman disease  (RDD)  or Sinus Histiocytosis with Massive Lymphadenopathy ( SHML) is a rare disease found mostly in children and young adults. It is included in the non Langerhans cell histiocytosis group. It is characterized by massive, painless bilateral cervical adenopathies. In some patients, such as this patient, disease can be localized to one lymphatic region. Biopsy revealed sinus infiltration by large histiocytes staining CD68 and S-100. As most lesions resolve spontaneously, the patient is follow-up clinically and by ultrasound, without further treatment.

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