The Prevalence of Cancer Predisposition Syndromes (CPSs) in Children with a Neoplasm: A Cohort Study in a Central and Eastern European Population
Abstract
:1. Introduction
2. Materials and Methods
2.1. Study Population
2.2. Clinical Evaluation
2.3. Data Analysis
3. Results
3.1. The General Characteristics of Patients
3.2. Prevalence of Specific Cancers Overall and by Age Group
3.3. Incidence of Cancer Predisposition Syndromes (CPSs) According to Age
3.4. Observed Age Ranges for the Highest Risk of CPS-Related Cancer Development in Children
3.5. Neoplasms Diagnosed in CPS-Positive Patients
4. Discussion
5. Conclusions
Supplementary Materials
Author Contributions
Funding
Institutional Review Board Statement
Informed Consent Statement
Data Availability Statement
Conflicts of Interest
References
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Characteristic | No. (%) | |
---|---|---|
Sex | ||
Male | 1233 (56.30) | |
Female | 957 (43.70) | |
Age at diagnosis of neoplasm, years old [y] | ||
0 to <5 y | 899 (41.05) | |
5 to <10 y | 463 (21.14) | |
10 to <15 y | 458 (20.91) | |
15 to <18 y | 370 (16.89) | |
Diagnosis | ||
ICD-10 | Mayor diagnosis | |
C11 | Malignant neoplasm of nasopharynx | 4 (0.18) |
C22 | Malignant neoplasm of liver and intrahepatic bile ducts | 42 (1.92) |
C30 | Malignant neoplasm of the nasal cavity and middle ear | 2 (0.09) |
C37 | Malignant neoplasm of thymus | 1 (0.05) |
C38 | Malignant neoplasm of the heart, mediastinum and pleura | 7 (0.32) |
C39 | Malignant neoplasm of other and ill-defined sites in the respiratory system and intrathoracic organs | 1 (0.05) |
C40 | Malignant neoplasm of bone and articular cartilage of limbs | 37 (1.69) |
C41 | Malignant neoplasm of bone and articular cartilage of other and unspecified sites | 27 (1.23) |
C43 | Malignant melanoma of the skin | 1 (0.05) |
C47 | Malignant neoplasm of peripheral nerves and autonomic nervous system | 177 (8.08) |
C48 | Malignant neoplasm of retro-peritoneum and peritoneum | 4 (0.18) |
C49 | Malignant neoplasm of other connective and soft tissue | 161 (7.35) |
C56 | Malignant neoplasm of ovary | 21 (0.96) |
C62 | Malignant neoplasm of testis | 23 (1.05) |
C64 | Malignant neoplasm of kidney, except renal pelvis | 130 (5.94) |
C67 | Malignant neoplasm of bladder | 4 (0.18) |
C68 | Malignant neoplasm of other and unspecified urinary organs | 1 (0.05) |
C69 | Malignant neoplasm of the eye and adnexa | 12 (0.55) |
C70 | Malignant neoplasm of meninges | 5 (0.23) |
C71 | Malignant neoplasm of the brain | 357 (16.30) |
C72 | Malignant neoplasm of the spinal cord, cranial nerves and other parts of the central nervous system | 20 (0.91) |
C73 | Malignant neoplasm of the thyroid gland | 73 (3.33) |
C74 | Malignant neoplasm of the adrenal gland | 17 (0.78) |
C75 | Malignant neoplasm of other endocrine glands and related structures | 5 (0.23) |
C76 | Malignant neoplasm of other and ill-defined sites | 13 (0.59) |
C79 | Secondary malignant neoplasm of other and unspecified sites | 3 (0.14) |
C80 | Malignant neoplasm, without specification of the site | 1 (0.05) |
C81 | Hodgkin lymphoma | 144 (6.58) |
C83 | Non-follicular lymphoma | 124 (5.66) |
C84 | Mature T/NK-cell lymphomas | 1 (0.05) |
C85 | Other and unspecified types of non-Hodgkin lymphoma | 10 (0.46) |
C88 | Malignant immunoproliferative diseases | 9 (0.41) |
C91 | Lymphoid leukemia | 505 (23.06) |
C92 | Myeloid leukemia | 187 (8.54) |
C94 | Other leukemias of specified cell type | 10 (0.46) |
C95 | Leukemia of unspecified cell type | 2 (0.09) |
C96 | Other and unspecified malignant neoplasms of lymphoid, hematopoietic and related tissue | 49 (2.24) |
Age [y] | C47 N = 177 | C49 N = 161 | C64 N = 130 | C70–72 N = 382 | C81 N = 144 | C83 N = 124 | C91 N = 505 | C92 N = 187 | Other N = 380 | Total N = 2190 |
---|---|---|---|---|---|---|---|---|---|---|
0 to <1 | 64 (36.2) | 31 (19.3) | 17 (13.1) | 34 (8.9) | 1 (0.7) | 3 (2.4) | 13 (2.6) | 9 (4.8) | 42 (11.1) | 214 (9.8) |
1 to <2 | 32 (18.1) | 16 (9.9) | 14 (10.8) | 29 (7.6) | 0 (0.0) | 5 (4.0) | 30 (5.9) | 18 (9.6) | 39 (10.3) | 183 (8.4) |
2 to <3 | 20 (11.3) | 17 (10.6) | 15 (11.5) | 34 (8.9) | 0 (0.0) | 5 (4.0) | 60 (11.9) | 12 (6.4) | 19 (5.0) | 182 (8.3) |
3 to <4 | 11 (6.2) | 13 (8.1) | 23 (17.7) | 30 (7.9) | 3 (2.1) | 4 (3.2) | 55 (10.9) | 9 (4.8) | 11 (2.9) | 159 (7.3) |
4 to <5 | 10 (5.6) | 12 (7.5) | 22 (16.9) | 26 (6.8) | 2 (1.4) | 5 (4.0) | 59 (11.7) | 9 (4.8) | 16 (4.2) | 161 (7.4) |
5 to <6 | 10 (5.6) | 4 (2.5) | 13 (10.0) | 21 (5.5) | 0 (0.0) | 7 (5.6) | 42 (8.3) | 7 (3.7) | 10 (2.6) | 114 (5.2) |
6 to <7 | 7 (4.0) | 6 (3.7) | 3 (2.3) | 23 (6.0) | 5 (3.5) | 11 (8.9) | 31 (6.1) | 5 (2.7) | 13 (3.4) | 104 (4.7) |
7 to <8 | 4 (2.3) | 1 (0.6) | 5 (3.8) | 23 (6.0) | 2 (1.4) | 8 (6.5) | 29 (5.7) | 10 (5.3) | 12 (3.2) | 94 (4.3) |
8 to <9 | 2 (1.1) | 5 (3.1) | 4 (3.1) | 19 (5.0) | 4 (2.8) | 7 (5.6) | 26 (5.1) | 5 (2.7) | 10 (2.6) | 82 (3.7) |
9 to <10 | 2 (1.1) | 3 (1.9) | 2 (1.5) | 13 (3.4) | 5 (3.5) | 6 (4.8) | 23 (4.6) | 3 (1.6) | 12 (3.2) | 69 (3.2) |
10 to <11 | 3 (1.7) | 4 (2.5) | 2 (1.5) | 17 (4.5) | 7 (4.9) | 9 (7.3) | 24 (4.8) | 13 (7.0) | 15 (3.9) | 94 (4.3) |
11 to <12 | 3 (1.7) | 6 (3.7) | 0 (0.0) | 15 (3.9) | 11 (7.6) | 5 (4.0) | 12 (2.4) | 16 (8.6) | 18 (4.7) | 86 (3.9) |
12 to <13 | 2 (1.1) | 4 (2.5) | 1 (0.8) | 15 (3.9) | 12 (8.3) | 6 (4.8) | 19 (3.8) | 10 (5.3) | 21 (5.5) | 90 (4.1) |
13 to <14 | 0 (0.0) | 9 (5.6) | 0 (0.0) | 12 (3.1) | 11 (7.6) | 12 (9.7) | 23 (4.6) | 14 (7.5) | 15 (3.9) | 96 (4.4) |
14 to <15 | 2 (1.1) | 5 (3.1) | 1 (0.8) | 13 (3.4) | 10 (6.9) | 7 (5.6) | 14 (2.8) | 13 (7.0) | 27 (7.1) | 92 (4.2) |
15 to <16 | 1 (0.6) | 8 (5.0) | 1 (0.8) | 19 (5.0) | 26 (18.1) | 13 (10.5) | 18 (3.6) | 10 (5.3) | 27 (7.1) | 123 (5.6) |
16 to <17 | 3 (1.7) | 6 (3.7) | 6 (4.6) | 17 (4.5) | 21 (14.6) | 6 (4.8) | 11 (2.2) | 13 (7.0) | 38 (10.0) | 121 (5.5) |
17 to <18 | 1 (0.6) | 11 (6.8) | 1 (0.8) | 22 (5.8) | 24 (16.7) | 5 (4.0) | 16 (3.2) | 11 (5.9) | 35 (9.2) | 126 (5.8) |
Legend | <5.0% | 5.0–9.9% | 10.0–14.9% | 15.0–19.9% | ≥20.0% |
Age, Year (s)/Month (s) Old [y/m] | CPS Presence | Statistical Analysis | |||
---|---|---|---|---|---|
No N = 1997 n (%) | Yes N = 193 n (%) | OR | 95% CI | p | |
0 to <1 y | 165 (8.3) | 49 (25.4) | 3.78 | 2.63–5.42 | <0.0001 |
0 to <1 m | 33 (1.7) | 11 (5.7) | 3.60 | 1.79–7.24 | 0.0001 |
1 to <2 m | 15 (0.8) | 6 (3.1) | 4.24 | 1.63–11.1 | 0.0048 |
2 to <3 m | 14 (0.7) | 3 (1.6) | 2.24 | 0.64–7.85 | 0.1839 |
3 to <4 m | 13 (0.7) | 3 (1.6) | 2.41 | 0.68–8.53 | 0.1613 |
4 to <5 m | 7 (0.4) | 1 (0.5) | 1.48 | 0.18–12.1 | 0.5225 |
5 to <6 m | 14 (0.7) | 5 (2.6) | 3.77 | 1.34–10.6 | 0.0216 |
6 to <7 m | 16 (0.8) | 4 (2.1) | 2.62 | 0.87–7.92 | 0.0926 |
7 to <8 m | 16 (0.8) | 3 (1.6) | 1.95 | 0.56–6.77 | 0.2313 |
8 to <9 m | 13 (0.7) | 3 (1.6) | 2.41 | 0.68–8.53 | 0.1613 |
9 to <10 m | 7 (0.4) | 5 (2.6) | 7.56 | 2.38–24.1 | 0.0004 |
10 to <11 m | 8 (0.4) | 2 (1.0) | 2.60 | 0.55–12.4 | 0.4891 |
11 to <12 m | 9 (0.5) | 3 (1.6) | 3.49 | 0.94–13.0 | 0.1408 |
1 to <2 y | 171 (8.6) | 12 (6.2) | 0.71 | 0.39–1.30 | 0.2609 |
2 to <3 y | 157 (7.9) | 25 (13.0) | 1.74 | 1.11–2.74 | 0.0144 |
3 to <4 y | 134 (6.7) | 25 (13.0) | 2.07 | 1.31–3.26 | 0.0014 |
4 to <5 y | 147 (7.4) | 14 (7.3) | 1.04 | 0.59–1.85 | 0.8848 |
5 to <6 y | 106 (5.3) | 8 (4.1) | 0.77 | 0.37–1.61 | 0.5997 |
6 to <7 y | 100 (5.0) | 4 (2.1) | 0.77 | 0.37–1.61 | 0.5997 |
7 to <8 y | 89 (4.5) | 5 (2.6) | 0.57 | 0.23–1.42 | 0.3005 |
8 to <9 y | 80 (4.0) | 2 (1.0) | 0.25 | 0.06–1.03 | 0.0606 |
9 to <10 y | 62 (3.1) | 7 (3.6) | 1.17 | 0.53–2.60 | 0.8565 |
10 to <11 y | 85 (4.3) | 9 (4.7) | 1.10 | 0.55–2.23 | 0.9334 |
11 to <12 y | 81 (4.1) | 5 (2.6) | 0.64 | 0.26–1.60 | 0.4472 |
12 to <13 y | 83 (4.2) | 7 (3.6) | 0.87 | 0.40–1.90 | 0.8698 |
13 to <14 y | 93 (4.7) | 3 (1.6) | 0.32 | 0.10–1.03 | 0.0678 |
14 to <15 y | 90 (4.5) | 2 (1.0) | 0.22 | 0.05–0.91 | 0.0351 |
15 to <16 y | 120 (6.0) | 3 (1.6) | 0.25 | 0.08–0.78 | 0.0163 |
16 to <17 y | 114 (5.7) | 7 (3.6) | 0.62 | 0.29–1.35 | 0.2966 |
17 to <18 y | 120 (6.0) | 6 (3.1) | 0.51 | 0.22–1.17 | 0.1420 |
Age, Year (s)/Month (s) Old [y/m] | CPS Presence | Statistical Analysis | |||
---|---|---|---|---|---|
No N = 1997 n (%) | Yes N = 193 n (%) | OR | 95% CI | p | |
11 to <18 y | 701 (35.1) | 33 (17.1) | 1.00 | reference | |
1 to <11 y | 1131 (56.6) | 111 (57.5) | 2.08 | 1.40–3.11 | 0.0002 |
2 to <12 m | 117 (5.9) | 32 (16.6) | 5.81 | 3.44–9.81 | <0.0001 |
0 to <2 m | 48 (2.4) | 17 (8.8) | 7.52 | 3.91–14.5 | <0.0001 |
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Telman-Kołodziejczyk, G.; Strauss, E.; Sosnowska-Sienkiewicz, P.; Januszkiewicz-Lewandowska, D. The Prevalence of Cancer Predisposition Syndromes (CPSs) in Children with a Neoplasm: A Cohort Study in a Central and Eastern European Population. Genes 2024, 15, 1141. https://doi.org/10.3390/genes15091141
Telman-Kołodziejczyk G, Strauss E, Sosnowska-Sienkiewicz P, Januszkiewicz-Lewandowska D. The Prevalence of Cancer Predisposition Syndromes (CPSs) in Children with a Neoplasm: A Cohort Study in a Central and Eastern European Population. Genes. 2024; 15(9):1141. https://doi.org/10.3390/genes15091141
Chicago/Turabian StyleTelman-Kołodziejczyk, Gabriela, Ewa Strauss, Patrycja Sosnowska-Sienkiewicz, and Danuta Januszkiewicz-Lewandowska. 2024. "The Prevalence of Cancer Predisposition Syndromes (CPSs) in Children with a Neoplasm: A Cohort Study in a Central and Eastern European Population" Genes 15, no. 9: 1141. https://doi.org/10.3390/genes15091141