NCI Definition: A rare disorder of unknown etiology characterized by distention of the lymph node sinuses and sinusoidal histiocytic infiltration. The histiocytes characteristically contain ingested lymphocytes. Patients present with cervical lymphadenopathy, fever, leukocytosis, and hypergammaglobulinemia. It can affect extranodal sites, including skin, bones, and the respiratory tract. It usually regresses spontaneously. [1]

Sinus histiocytosis with massive lymphadenopathys most frequently harbor alterations in KRAS, MAP2K1, TERT, SH2B3, and NOTCH3 [2].

Most Commonly Altered Genes in Sinus Histiocytosis with Massive Lymphadenopathy

KRAS Mutation, MAP2K1 Mutation, MAP2K1 G128D, MAP2K1 Exon 3 Mutation, and KRAS K117N are the most common alterations in sinus histiocytosis with massive lymphadenopathy [2].

Top Alterations in Sinus Histiocytosis with Massive Lymphadenopathy

Disease Details

Sinus Histiocytosis, Rosai-Dorfman Disease
Non-langerhans-cell Histiocytosis
OncoTree Name
Rosai-Dorfman Disease
OncoTree Code


1. National Cancer Institute. NCI Thesaurus Version 18.11d. https://ncit.nci.nih.gov/ncitbrowser/ [2018-08-28]. [2018-09-21].

2. The AACR Project GENIE Consortium. AACR Project GENIE: powering precision medicine through an international consortium. Cancer Discovery. 2017;7(8):818-831. Dataset Version 8. This dataset does not represent the totality of the genetic landscape; see paper for more information.

3. All assertions and clinical trial landscape data are curated from primary sources. You can read more about the curation process here.