Table 3 Standardised incidence ratios for specific cancers in patients with Noonan syndrome, Costello syndrome, and KRAS syndrome

From: Cancer spectrum and frequency among children with Noonan, Costello, and cardio-facio-cutaneous syndromes

   

Cases of cancer

  

Syndrome

Cancer type

n

Observed

Expected

PY

SIR, 95% CI

NS combined

JMML

632

3

0.004

6535.6

717 (148–2094)

 

ALL

632

2

0.282

6535.6

7.1 (0.9–25.6)

 

NBL

632

1

0.093

6535.6

10.8 (0.3–59.9)

CS

ERMS

32

2

0.001

278.2

1630 (197–5887)

KRAS

Astrocytoma

17

1

0.002

175.2

410 (10–2287)

 

JMML

17

1

0.000

175.2

10172 (258–56672)

  1. Abbreviations: ALL=acute lymphoblastic leukaemia; CI=confidence interval; CS=Costello syndrome; ERMS=embryonal rhabdomyosarcoma; JMML=juvenile myelomonocytic leukaemia; KRAS=RASopathy with a germline mutation of KRAS; NBL=neuroblastoma; NS=Noonan Syndrome; PY=person-years.
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