Table 1 Risk of all cancers combined, and of malignant and benign neoplasms of nervous system, in patients with neurofibromatosis (all NF) and neurofibromatosis type 1 (NF1): observed (O) and expected (E) number of each cancer, ratio of ratea (RR) in the NF and NF1 cohort to that in the reference cohortb, and 95% confidence intervals (95% CI) with P-value

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

 

All NF

NF1

Cancers (ICD codes) c

O

E

RR (95% CI)

P -value

O

E

RR (95% CI)

P -value

All cancers (C00–C75, C81–C97)

769

178.4

4.3 (4.0–4.6)

<0.001

697

174.2

4.0 (3.7–4.3)

<0.001

All cancers, excluding nervous system and brain (C00–C46, C48–C68, C73–C75, C81–C97)

489

180.6

2.7 (2.5–3.0)

<0.001

459

174.9

2.7 (2.4–2.9)

<0.001

Malignant neoplasm

Peripheral nerves (C47)

231

0.5

1047 (858–1280)

<0.001

204

0.3

1394 (1133–1716)

<0.001

Meninges (C70)

6

0.1

50.1 (18.3–110.5)

<0.001

0

   

Brain (C71)

164

4.0

41.8 (35.5–48.8)

<0.001

134

3.6

37.9 (31.7–45.0)

<0.001

Spinal cord, cranial nerves and other parts of central nervous system (C72)

136

0.6

332 (271–405)

<0.001

105

0.4

377 (300–471)

<0.001

Eye and eye adnexa (C69)

14

0.7

21.5 (11.7–36.2)

<0.001

11

0.6

20.0 (9.7–35.2)

<0.001

Benign neoplasm

Meninges (D32)

73

2.4

31.0 (24.2–39.0)

<0.001

0

   

Brain, central nervous system, spinal cord (D33)

158

1.6

105 (89–123)

<0.001

20

0.5

42.7 (26.0–66.4)

<0.001

Eye and eye adnexa (D31)

16

0.9

18.1 (10.3–29.5)

<0.001

11

0.9

11.9 (5.9–21.3)

<0.001

  1. Abbreviation: ICD=International Classification of Diseases.
  2. aAdjusted 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. bConditions 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. cICD-10 codes for each cancer.
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