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Table 1 Clinical features of patients reported with VCP disease and malignancies

From: A clinicopathologic study of malignancy in VCP-associated multisystem proteinopathy

Case

Cancer Type

Current Age y

(Age deceased)

Sex

VCP variant (c.DNA;protein)

Myopathy/Age of onset y

PDB/Age of onset y

FTD/Age of onset y

Cancer age of onset y

Rate in VCP disease

Incidence in general population

1

Peripheral nerve sheath tumor

56

M

c.464G > A; p.R155H

 + /36

 + /33

–

45

1/231

1/100000[30]

2

Pleomorphic xanthoastrocytoma

(35)

M

c.572G > A; p.R191Q

–

–

–

32

1/231

 < 1/285714[31]

3

Thymoma

(48)

F

c.463C > T; p.R155C

 + /45

–

–

30

1/231

1/769231[32]

4

Breast invasive ductal carcinoma

76

F

c.476G > A; p.R159H

–

–

 + /71

35

1/231

1/8 [33]

5

Pancreatic ductal adenocarcinoma

(55)

M

c.463C > T; p.R155C

 + /50

 + /40

–

54

1/231

1/7576[34]

6

Endometrial adenocarcinoma

(60)

F

c.463C > T; p.R155C

 + /34

 + / 45

 + /59

55

1/231

1/6667[35]

7

Prostate adenocarcinoma

72

M

c.464G > A; p.R155H

 + /33

-

–

69

1/231

1/14[36]

  1. FTD = frontotemporal dementia. PDB = Paget disease of bone