Table II.
Types and ages of malignancies in NS, CS, CFCS and NSML literature case (References: Supplementary File)
| Syndrome and Type of cancer (excluding myeloproliferative disease) |
No. of cancers |
Median age, y (range) |
Median age in general population, ya |
Pediatric age peak or range, yb |
|---|---|---|---|---|
| Noonan Syndrome | ||||
| Neuroblastoma | 8 | 0.8 (0.4–4) | 1 | 0–0.5 |
| Acute lymphoblastic leukemia | 8 | 2.3 (0.8–17) | 13 | 2–3 |
| Glioma | 6 | 9.5(1.8–20) | 43 | 0–19c |
| Rhabdomyosarcoma | 6 | 4(1.7–9) | 49 | <5d |
| Acute myeloid leukemia | 3 | 4, 9, 34 | 67 | 0–1 |
| Testicular cancer | 3 | 4, 22, 26 | 34 | - |
| Non Hodgkin lymphoma | 2 | N/A | 67 | - |
| Colon carcinoma | 2 | 70,70 | 72 | - |
| Chronic lymphocytic leukemia | 1 | 56 | 72 | - |
| Wilms tumor | 1 | N/A | 3 | 3 |
| Hepatoblastoma | 1 | 0.1 | 1 | 1 |
| Hodgkin disease | 1 | 35 | 38 | 14 |
| Chronic myelomonocytic leukemia | 1 | 35 | 76 | - |
| Bile duct tumor | 1 | N/A | 71 | - |
| Malignant schwannoma | 1 | 14 | 52 | - |
| Breast Cancer | 1 | 45 | 61 | - |
| Costello Syndrome | ||||
| Rhabdomyosarcoma | 19 | 2.3 (0.5–6.0) | 49 | <5d |
| Neuroblastoma | 5 | 1.4(0–3.0) | 1 | 0–0.5 |
| Bladder cancer | 4 | 13.5(0.8–20) | 73 | - |
| Fibrosarcoma | 1 | 2.5 | 54.5 | - |
| Cardiofaciocutaneous Syndrome | ||||
| Acute lymphoblastic leukemia | 4 | 5.0(1.8–41.0) | 13 | 2–3 |
| Non Hodgkin lymphoma | 2 | 35.1 (0.2–70) | 67 | - |
| Hepatoblastoma | 1 | 2.9 | 1 | 1 |
| Rhabdomy o sarcoma | 1 | 1.7 | 49 | <5d |
| Noonan Syndrome with Multiple Lentigines or LEOPARD Syndrome | ||||
| Acute myeloid leukemia | 2 | 9.5(9–13) | 67 | 0–1 |
| Acute lymphoblastic leukemia | 1 | 8 | 13 | 2–3 |
| Neuroblastoma | 1 | N/A | 1 | 0–0.5 |
| Melanoma | 1 | 60 | 59 | - |
a
According to [SEER, 2000–2007]
b
According to [Pizzo and Poplack, 2001; WHO, 2002; WHO, 2007]
c
This age refers to juvenile pilocytic astrocytoma
d
This age refers to embryonal rhabdomyosarcoma