Table 1 Summary of patients evaluated in this study.

RPLND, retroperitoneal lymph node dissection; LND, lymph node dissection; M, male; F, female; N/A, not applicable.

PatientM/FAgeFH germline
mutation
TNM stageAJCC stageClinical
stage
Surgical
procedure
Clinical statusAge at
death
1F49Q439PIIIT3a N1 M0MetastaticRPLNDDead of disease49
2F25H235DIT1a N0 M0LocalizedLeft partial
nephrectomy
No evidence of
disease
N/A
3F50L244RIIIT3a N1 M0MetastaticLeft radical
nephrectomy,
adrenalectomy,
and RPLND
Dead of disease54
4F40K371*IVT1b N1 M1MetastaticRPLNDAlive with
disease
N/A
5M38V322GIVT2a N1 M1MetastaticRight video-
assisted
thoracoscopic
surgery with
parietal pleural
mass biopsy
Dead of disease41
6F42R343XIVT3a N1 M1MetastaticCytoreductive
left
nephrectomy,
adrenalectomy,
and RPLND
Alive with
disease
N/A
7F24dupT,p.M266fsIVT4 N1 M1MetastaticDistal
pancreatectomy,
splenectomy, left
colectomy,
RPLND, and
resection of
retroperitoneal
recurrence
Dead of disease26
8M27Q439PIIIT3b N1 M0MetastaticLeft radical
nephrectomy
with inferior
vena cava
thrombectomy
Dead of disease32
9M68A101*IIIT3a N0 M0Locally
advanced
Right radical
nephrectomy
and RPLND
No evidence of
disease
N/A
10M31Complete
deletion
IVT3a N1 M1MetastaticLeft partial
nephrectomy
and RPLND with
bilateral pelvic
LND
Dead of disease32
11M21G280DIVT3a N0 M1MetastaticRight
hepatectomy,
partial left
hepatectomy,
and
cholecystectomy
Dead of disease24
12M63Complete
deletion
IIIT3a N1 M0MetastaticRight radical
nephrectomy,
RPLND, bilateral
pelvic LND, and
cholecystectomy
Dead of disease63
13F56R233HIVT3a N1 M1MetastaticRPLND, pelvic
LND,
omentectomy,
left
oopherectomy,
splenectomy, left
lower lobe
pulmonary
wedge resection,
abdominal mass
resection
Dead of disease61
14F43N340KIVT1a N1 M1MetastaticRPLNDAlive with
disease
N/A
15M49dupT,p.M266fsIVT3a N1 M1MetastaticRPLNDAlive with
disease
N/A
16M27T147AIVT3a N1 M1MetastaticRight partial
nephrectomy
and RPLND
Dead of disease29
17F38S365GIT1a N0 M0LocalizedRight partial
nephrectomy
No evidence of
disease
N/A
18M52S365GIVT4 N1 M1MetastaticRight lung lower
lobectomy and
mediastinal LND
Alive with
disease
N/A
19M70I81Dfs14IVT4 N1 M1MetastaticLeft
nephrectomy
and RPLND
Dead of disease71
20M40Complete
deletion
IIIT1b N1 M0Locally
advanced
Left radical
nephrectomy
and RPLND
Alive with
disease
N/A
21M30Q439PIVT3b N1 M1MetastaticRPLNDDead of disease32
22M32delA p.E432fsIIIT1a N1 M0Locally
advanced
Left radical
nephrectomy
Alive with
disease
N/A
23M38R233CIIT2b N0 M0LocalizedLeft radical
nephrectomy
Dead of disease48
24M23R233HIVT2b N1 M1MetastaticLeft radical
nephrectomy
(autopsy)
Dead of disease23
25M59N107TIIIT3a N1 M0Locally
advanced
Right partial
nephrectomy,
left partial
adrenalectomy,
and RPLND
No evidence of
disease
N/A

*stop codon mutation.