Publication: 18F-FDG PET / CT findings of Rosai Dorfman Disease withdiffuse extranodal involvement
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2020-10-22
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Aim/Introduction: Rosai Dorfman disease (RDD) is a rare
benign disease characterized by permanent massive
lymphadenopathy mimicking malignant tumors. Extranodal
involvement, including the skin, upper respiratory tract,
salivary gland, bone, central nervous system and rarely
kidneys, may occur in approximately 43% of patients.
Although RDD can usually recover spontaneously, it may
persist in some patients and mimic lymphoproliferative
disorders clinically and visually. We present the 18F-FDG PET
/ CT findings of the patient with RDD with diffuse extranodal
involvement. Materials and Methods: An 18F-FDG PET / CT
examination was performed to investigate malignancy in
a 57-year-old male patient with multiple cervical lymph
nodes. Results: 18F-FDG PET / CT demonstrated 2.5 cm
sized intense hypermetabolic lymph nodes in the bilateral
cervical chain. Intense hypermetabolic soft tissue density
lesions were observed in the subcutaneous fatty plans
of the T12 vertebra left posterolateral section and the
right lower quadrant of the abdomen. 12 mm sized mild
hypermetabolic soft tissue density nodular lesion was seen
in the right adrenal gland medial crust and 3x2.5 cm sized
mild hypermetabolic soft tissue density nodular lesion
was found in the left adrenal gland corpus. In the skeletal
system, intense hypermetabolic lytic lesions compatible
with metastasis were observed in the left occipital
condyle, the posterior elements of the T6 vertebra, the C1
vertebra transvers process, the posterior part of the right
acetabulum and the posterior part of the right iliac bone.
Excisional biopsy was performed on the lymph node in
the submandibular area and the lesion observed in the
subcutaneous fatty plan in the right lower quadrant of the
abdomen and the histopathology was found compatible
with Rosai Dorfman disease. Conclusion: RDD, first
described by Rosai and Dorfman in 1969, is a rare benign
lymphoproliferative disease. RDD is usually manifested by
cervical lymphadenopathy and typically mimics lymphoma.
Diseases such as lymphoma, tuberculosis, sarcoidosis,
reactive hyperplasia and nasopharyngeal carcinoma can
be considered in the differential diagnosis. Extranodal
disease is observed in approximately 43% of cases,
most commonly in the skin, nasal cavity, eyes and bone.
Although Rosai-Dorfman disease is usually self-limited,
patients with extranodal involvement may have a more
fulminant course and need active treatment. Since lesions
show increased FDG uptake, 18F-FDG PET-CT may be useful
in determining the extent of the disease, including lymph
node involvement. Therefore, the present case emphasized
the importance of 18F-FDG PET-CT scan in the diagnosis,
staging and treatment selection of the disease. References:
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FİLİZOĞLU N., KESİM S., ŞAHİN C. Ö., Öksüzoğlu K., ÖZGÜVEN S., ÖNEŞ T., TUROĞLU H. T., ERDİL T. Y., \"18F-FDG PET / CT findings of Rosai Dorfman Disease with
diffuse extranodal involvement\", European Association of Nuclear Medicine October 22 – 30, 2020 Virtual, 22 - 30 Ekim 2020