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Table 1 The HLRCC-RCC patients with immunotherapy

From: Case report: response to immunotherapy and association with the fh gene in hereditary leiomyomatosis and renal cell cancer-associated renal cell cancer

Reference

Design

N

line

Histology (N)

Treatment

ORR (%)

PFS (m)

OS (m)

Wang t al [14].

Retrospective

1

1 L

HLRCC-RCC

pembrolizumab

100

NR

NR

Iribe et al. [13]

Retrospective

1

1 L

HLRCC-RCC

Nivolumab + ipilimumab

100

NR

NR

Yonese et al. [4]

Retrospective

1

> 1 L

HLRCC-RCC

Axitinib + Nivolumab

100

NR

NR

Feng et al. [16]

Retrospective

1

1 L

HLRCC-RCC

Sintilimab + Axitinib

100

NR

NR

Gleeson et al. [17]

Retrospective

3

> 1 L

HLRCC-RCC

ICI = 2

ICI + TKI = 1

ICI = 0

ICI + TKI = 100

NA

NA

Sun et al. [18]

Retrospective

5

1 L

HLRCC-RCC

ICI + TKI

16.7

13.3

NR

Lee et al. [12]

Phase 2

5

1 L

HLRCC-RCC

Cabozantinib + Nivolumab = 5

100

NA

NA

Carril et al. [11].

Retrospective

11

1 L/>1 L

HLRCC-RCC

ICI

18

2.7

NA

Xu et al. [10].

Retrospective

48

1 L/>1 L

HLRCC-RCC

ICI + TKI

33

18

NR

  1. NA: not available.NR: not reached. N: population. ORR: overall response rate. PFS: progression-free survival. M: months. OS: overall survival. HLRCC: hereditary leiomyomatosis and renal cell cancer. HLRCC-RCC: HLRCC-associated RCC. ICI: immune checkpoint inhibitor. TKI: tyrosine kinase inhibitor