Sinus Histiocytosis with Massive Lymphadenopathy
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. 
Sinus histiocytosis with massive lymphadenopathys most frequently harbor alterations in KRAS, MAP2K1, TERT, SH2B3, and NOTCH3 .
KRAS Mutation, MAP2K1 Mutation, MAP2K1 G128D, MAP2K1 Exon 3 Mutation, and KRAS K117N are the most common alterations in sinus histiocytosis with massive lymphadenopathy .
There is 1 clinical trial for sinus histiocytosis with massive lymphadenopathy, of which 1 is open and 0 are completed or closed. Of the trial that contains sinus histiocytosis with massive lymphadenopathy as an inclusion criterion, 1 is phase 2 (1 open).
Cobimetinib is the most common intervention in sinus histiocytosis with massive lymphadenopathy clinical trials.
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.