Table 1:

Summary of patients evaluated in this study.

Patient	M/F	Age	FH Germline Mutation	TNM Stage	AJCC Stage	Clinical Stage	Surgical Procedure†	Clinical Status	Age at Death
1	F	49	Q439P	III	T3a N1 M0	Metastatic	RPLND	Dead of disease	49
2	F	25	H235D	I	T1a N0 M0	Localized	Left partial nephrectomy	No evidence of disease	N/A
3	F	50	L244R	III	T3a N1 M0	Metastatic	Left radical nephrectomy, adrenalectomy, and RPLND	Dead of disease	54
4	F	40	K371*	IV	T1b N1 M1	Metastatic	RPLND	Alive with disease	N/A
5	M	38	V322G	IV	T2a N1 M1	Metastatic	Right video-assisted thoracoscopic surgery (VATS) with parietal pleural mass biopsy	Dead of disease	41
6	F	42	R343X	IV	T3a N1 M1	Metastatic	Cytoreductive left nephrectomy, adrenalectomy, and RPLND	Alive with disease	N/A
7	F	24	dupT,p.M266fs	IV	T4 N1 M1	Metastatic	Distal pancreatectomy, splenectomy, left colectomy, RPLND and resection of retroperitoneal recurrence	Dead of disease	26
8	M	27	Q439P	III	T3b N1 M0	Metastatic	Left radical nephrectomy with inferior vena cava thrombectomy	Dead of disease	32
9	M	68	A101*	III	T3a N0 M0	Locally Advanced	Right radical nephrectomy and RPLND	No evidence of disease	N/A
10	M	31	Complete deletion	IV	T3a N1 M1	Metastatic	Left partial nephrectomy and RPLND with bilateral pelvic LND	Dead of disease	32
11	M	21	G280D	IV	T3a N0 M1	Metastatic	Right hepatectomy, partial left hepatectomy, and cholecystectomy	Dead of disease	24
12	M	63	Complete deletion	III	T3a N1 M0	Metastatic	Right radical nephrectomy, RPLND, bilateral pelvic LND and cholecystectomy	Dead of disease	63
13	F	56	R233H	IV	T3a N1 M1	Metastatic	RPLND, pelvic LND, omentectomy, left oopherectomy, splenectomy, left lower lobe pulmonary wedge resection, abdominal mass resection	Dead of disease	61
14	F	43	N340K	IV	T1a N1 M1	Metastatic	RPLND	Alive with disease	N/A
15	M	49	dupT,p.M266fs	IV	T3a N1 M1	Metastatic	RPLND	Alive with disease	N/A
16	M	27	T147A	IV	T3a N1 M1	Metastatic	Right partial nephrectomy and RPLND	Dead of disease	29
17	F	38	S365G	I	T1a N0 M0	Localized	Right partial nephrectomy	No evidence of disease	N/A
18	M	52	S365G	IV	T4 N1 M1	Metastatic	Right lung lower lobectomy and mediastinal LND	Alive with disease	N/A
19	M	70	I81Dfs14	IV	T4 N1 M1	Metastatic	Left nephrectomy and RPLND	Dead of disease	71
20	M	40	Complete deletion	III	T1b N1 M0	Locally Advanced	Left radical nephrectomy and RPLND	Alive with disease	N/A
21	M	30	Q439P	IV	T3b N1 M1	Metastatic	RPLND	Dead of disease	32
22	M	32	delA p.E432fs	III	T1a N1 M0	Locally Advanced	Left radical nephrectomy	Alive with disease	N/A
23	M	38	R233C	II	T2b N0 M0	Localized	Left radical nephrectomy	Dead of disease	48
24	M	23	R233H	IV	T2b N1 M1	Metastatic	Left radical nephrectomy (autopsy)	Dead of disease	23
25	M	59	N107T	III	T3a N1 M0	Locally Advanced	Right partial nephrectomy, left partial adrenalectomy, and RPLND	No evidence of disease	N/A

^†All surgical procedures were performed open, unless otherwise stated. RPLND: Retroperitoneal lymph node dissection; LND: Lymph node dissection; M: Male. F: Female.