Table 2.

KRAS G13C case reports.

Disease	Sex/Age (years)	Target mutation	Symptoms	Treatment regimen	mPFS (months)	mOS (months)
I. Rosai–Dorfman disease	Male/50	G13C	Fatigue, lymphadenopathy, rash	Prednisone (PO 40 mg QD) + rituximab (intravenous 500 mg/m2 weekly ×4, then q2mo) Lenalidomide (15 mg QD) + dexamethasone (4 mg QD) Sirolimus (PO 4 mg titrated to 6 mg QD)	18	/
II. RAS-related LPD	Female/11	G13C	Splenomegaly, bleeding	Sirolimus (1 mg/m2/day PO)	/	/
III. ITP + HSP + intestinal Behçet’s	Male/3	G13C	Abd. pain, purpura, arthralgia	Prednisone (PO, dose escalated on relapse) Adalimumab (q2w × 13 mo) + prednisone taper	13	/
IV. Rosai–Dorfman + SLE	Male/15	G13C	Lymphadenopathy (SVC obstruction), pericardial effusion	Prednisone (PO) + methotrexate + azathioprine + rituximab (intravenous monthly ×6)	/	/
V. Primary sarcomatoid carcinoma of small intestine	Male/54	G13C	Abd. distension, obstruction	Surgery + chemotherapy (ifosfamide + epirubicin)	2	2
VI. RAS-related ALPS	Female/11	G13C	Fever, rash, hepatosplenomegaly	Rituximab (intravenous weekly ×4, then monthly ×11)	24	24

PO, per OS; QD, quaque die; q2mo, quaque every 2 months; q2w, quaque 2 weeks; LPD, lymphoproliferative disease; ITP, immune thrombocytopenic purpura; HSP, Henoch–Schönlein purpura; Abd, abdominal; SVC, superior vena cava; SLE, systemic lupus erythematosus; MTX, methotrexate; AZA, azathioprine; ALPS, autoimmune lymphoproliferative syndrome.