Table 2 Risk of malignant neoplasm in patients with neurofibromatosis type 1 compared with the reference cohortb, number of each observed (O) and expected (E) cancer, ratio of ratea (RR) with 95% confidence intervals (95% CI) and P-values

From: Risk of benign tumours of nervous system, and of malignant neoplasms, in people with neurofibromatosis: population-based record-linkage study

 

All years

Excluding first year

Cancers (ICD-10 codesc)

O

E

RR (95% CI)

P -value

O

E

RR (95% CI)

P -value

Oesophagus (C15)

18

5.4

3.3 (2.0–5.3)

<0.001

12

4.5

2.7 (1.4–4.7)

0.001

Stomach (C16)

13

4.7

2.8 (1.5–4.7)

<0.001

7

3.8

1.8 (0.7–3.8)

0.174

Small intestine (C17)

12

0.8

14.5 (7.5–25.3)

<0.001

6

0.7

9.2 (3.4–20.0)

<0.001

Colon (C18)

26

13.2

2.0 (1.3–2.9)

0.001

14

11.0

1.3 (0.7–2.1)

0.446

Rectosigmoid junction (C19)

7

2.9

2.4 (1.0–4.8)

0.042

d

   

Rectum (C20)

9

6.4

1.4 (0.6–2.7)

0.434

8

5.3

1.5 (0.7–3.0)

0.340

Liver (C22)

10

2.7

3.8 (1.8–6.8)

<0.001

7

2.2

3.1 (1.3–6.5)

0.004

Unspecified malignancies of biliary tract (C24)

5

0.6

8.2 (2.7–19.3)

<0.001

d

   

Pancreas (C25)

16

4.7

3.4 (2.0–5.6)

<0.001

8

3.8

2.0 (0.9–4.1)

0.062

Other digestive organs (C26)

10

2.1

4.8 (2.3– 8.8)

<0.001

8

1.7

4.7 (2.0–9.3)

<0.001

Lung/bronchus (C34)

66

21.4

3.0 (2.4–3.9)

<0.001

39

17.2

2.3 (1.6–3.1)

<0.001

Heart, mediastinum and pleura (C38)

7

0.4

19.0 (7.6–40.0)

<0.001

5

0.3

16.6 (5.4–39.0)

<0.001

Retroperitoneum and peritoneum (C48)

10

0.7

14.5 (6.9–26.6)

<0.001

d

   

Malignant skin melanoma (C43)

19

5.3

3.6 (2.2–5.6)

<0.001

12

4.3

2.8 (1.5–4.9)

<0.001

Non-melanoma skin cancer (C44)

53

34.0

1.6 (1.2–2.0)

0.002

43

27.9

1.5 (1.1–2.1)

0.006

Bone and cartilage of limbs (C40)

5

0.3

15.6 (5.1–36.7)

<0.001

5

0.2

23.1 (7.5–54.5)

<0.001

Bone and cartilage of other sites (C41)

17

0.9

19.6 (11.4–31.5)

<0.001

7

0.6

11.3 (4.5–23.4)

<0.001

Other connective and soft tissue (C49)

65

1.6

42.1 (32.4–53.8)

<0.001

28

1.2

23.8 (15.8–34.5)

<0.001

Thyroid gland (C73)

7

1.4

4.9 (2.0–10.2)

<0.001

6

1.2

5.1 (1.9–11.1)

<0.001

Adrenal gland (medulla) (C74.1)

12

0.1

304 (148–572)

<0.001

6

0.1

192 (67–451)

<0.001

Diffuse non-Hodgkin’s lymphoma (C83)

11

3.3

3.3 (1.7–6.0)

<0.001

d

   

Unspecified non-Hodgkins lymphomas (C84)

14

6.2

2.3 (1.2–3.8)

0.004

7

4.6

1.5 (0.6–3.2)

0.361

Multiple myeloma (C90)

7

2.9

2.4 (1.0–4.9)

0.036

d

   

Lymphoid leukaemia (C91)

9

3.6

2.5 (1.1–4.6)

0.011

5

2.4

2.1 (0.7–4.9)

0.172

Acute myeloid leukaemia (C92.0)

8

1.9

4.2 (1.8–8.2)

<0.001

6

1.5

4.0 (1.5–8.6)

0.001

Chronic myeloid leukaemia (C92.1)

5

0.7

6.7 (2.2–15.8)

<0.001

d

   

Breast (women) (C50)

58

25.7

2.3 (1.7–2.9)

<0.001

43

20.9

2.1 (1.5–2.8)

<0.001

Body of uterus (C54)

5

4.9

1.0 (0.3–2.4)

0.867

d

  

0.679

Ovary (C56)

14

3.8

3.7 (2.0–6.2)

<0.001

10

3.0

3.3 (1.6–6.1)

<0.001

Prostate (C61)

17

23.1

0.7 (0.4–1.2)

0.247

14

17.7

0.8 (0.4–1.3)

0.444

  1. Abbreviation: ICD=International Classification of Diseases.
  2. Adjusted for age in 5-year bands, time period in single calendar years, region of residence and deprivation score associated with patients’ area of residence, in quintiles.
  3. Conditions used in reference cohort, with Office of Population, Censuses and Surveys (OPCS) code edition 4 for operations and ICD-10 code for diagnosis (with equivalent codes used for other coding editions): appendectomy (OPCS4 H01–H03), adenoidectomy (E20), dilation and curettage (Q10–Q11), hip replacement (W37–W39), knee replacement (W40–W42), squint (ICD-10 H49–H51),cataract (H25), otitis (H60–H67), upper respiratory tract infections (J00–J06), varicose veins (I83), haemorrhoids (I84), deflected septum, nasal polyp (J33+J34.2), impacted tooth and other disorders of teeth (K00–K03), inguinal hernia (K40), head injury (S06), in-growing nail, toenail and other diseases of nail (L60), contraceptive management (Z30), internal derangement of knee (M23), bunion (727.1), dislocations, sprains and strains (S03, S13, S23, S33, S43, S53, S63, S73, S83, S93), selected limb fractures (S42, S52, S62, S82, S92), superficial injury and contusion (S00, S10, S20, S30, S40, S50, S60, S70, S80, S90). From analyses of colorectal cancers, we excluded appendectomy, haemorrhoids and inguinal hernia from the reference cohort. From analysis of uterine cancer we excluded dilation and curettage.
  4. ICD-10 codes for each cancer.
  5. Data are not shown for fewer than five observed cases.
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