La malattia di Rosai-Dorfman può simulare la presenza di un tumore e può risultare positiva alla PET

La malattia di Rosai-Dorfman (istiocitosi dei seni) può apparire come un tumore maligno, risultando positiva alla PET
Carlo Gazia, Luca Giordano, Maria Grazia Diodoro, Iacopo Compalati, Riccardo Avantifiori, Gian Luca Grazi: Differential diagnosis in Rosai-Dorfman disease: A rare case of isolated hepatic presentation mimicking a metastatic tumor with positive 18-FDG uptake. In: Intractable and Rare Diseases Research, vol. 11, no. 2, pp. 90 – 92, 2022, ISSN: 21863644, (Cited by: 0; All Open Access, Bronze Open Access, Green Open Access).


Rosai-Dorfman disease (RDD) is also called sinus histiocytosis with massive lymphadenopathy, and it is caused by a histiocytic disorder with unclear etiology. It usually involves cervical lymph nodes, but it may also present with extranodal involvement. We report a rare condition of isolated hepatic RDD without nodal involvement, clinically manifested with three-month abdominal pain and tenderness of the right hypochondrium. CT- and PET-CT scans were compatible with a secondary lesion from an unknown primary tumor. Therefore, the patient underwent an atypical liver resection. Immunohistochemistry and histological results were compatible with a diagnosis of RDD. RDD is characterized by phenomena of emperipolesis, histiocytic proliferation and positive immunostaining for CD14, CD68 and S-100 protein. Cases of isolated gastrointestinal localization of RDD are particularly rare, especially in the liver. Instrumental exams might confuse RDD with other malignancies. RDD is a rare entity, which might be misdiagnosed using PET-CT due to its similarities with malignant tumors. An accurate multidisciplinary approach may help to clear diagnostic clues of this uncommon disease. © 2022 International Advancement Center for Medicine and Health Research. All rights reserved.

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