Cancer incidence among persons Prader-Willi syndrome in Finland

Patja, Kristiina; Sund, Reijo; Kaski, Markus; Pukkala, Eero

doi:10.1515/ijdhd.2008.7.1.69

Cited by 6 publications

(9 citation statements)

References 24 publications

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“…Most studies reporting incidence examined cancer (S10 Table in S4 File ). Crude incidence rates for all cancers ranged from 12.8 to 33.1 per 10 000 person-years in people with ID of any aetiology [ 16 , 77 ], from 6.4 to 12.6 per 10 000 person-years in people with DS [ 54 , 115 – 117 ] and 29 per 10 000 person-years in people with PWS [ 55 ]. The incidence of solid tumours was low, regardless of ID aetiology, with the highest incidence observed for gastrointestinal cancer in people with mild to moderate ID [ 16 ].…”

Section: Resultsmentioning

confidence: 99%

Prevalence and incidence of physical health conditions in people with intellectual disability – a systematic review

et al. 2021

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Objective To synthesize evidence on the prevalence and incidence of physical health conditions in people with intellectual disability (ID). Methods We searched Medline, PsycInfo, and Embase for eligible studies and extracted the prevalence, incidence, and risk of physical health conditions in people with ID. Results Of 131 eligible studies, we synthesized results from 77 moderate- to high-quality studies, which was mainly limited to high-income countries. The highest prevalence estimates were observed for epilepsy, ear and eye disorders, cerebral palsy, obesity, osteoporosis, congenital heart defects, and thyroid disorders. Some conditions were more common in people with a genetic syndrome. Compared with the general population, many health conditions occur more frequently among people with ID, including asthma and diabetes, while some conditions such as non-congenital circulatory diseases and solid cancers occur at the same or lower rate. The latter associations may reflect under-detection. Conclusions People with ID have a health profile more complex than previously known. There is a pressing need for targeted, evidence-informed population health initiatives including preventative programs for this population.

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Section: Resultsmentioning

confidence: 99%

Prevalence and incidence of physical health conditions in people with intellectual disability – a systematic review

et al. 2021

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“…7 In a cohort of PWS patients from Finland a non-significant increase for testicular cancer, breast cancer and leukaemia was observed. 8 Similarly, a large US survey, involving 1852 PW patients, showed an increase in the total number of observed cancer cases (8 versus 4.8 expected in the general US population) albeit a significantly increased risk for myeloid leukaemia was noticed (3 observed cases versus 0.075 expected). 9 No convincing mechanism has been proposed for this excess of cases.…”

Section: Introductionmentioning

confidence: 93%

“…7 It is associated with significant morbidity and mortality 7 including an increased incidence of malignant conditions. 8,9 We present a young patient with PWS and medulloblastoma in complete remission that recurred at an unusual site, three years after the end of treatment. To our knowledge, this is the first report of a medulloblastoma in a paediatric patient with PWS and the first report of medulloblastoma recurrence at the pons.…”

Section: Introductionmentioning

confidence: 99%

“…9 Another explanation for the excess testicular tumours could be the increased incidence of cryptorchidism -a known risk factor for testicular cancer-among PW patients. 8 An epigenetic phenomenon called genomic imprinting plays a central role in the pathogenesis of PWS. Imprinting causes genes to be expressed in a parent-oforigin-specific manner, by way of "silencing" the genes from the other sex parent via methylation.…”

Section: Introductionmentioning

confidence: 99%

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Challenges in the Diagnosis of Medulloblastoma Recurrence at an Unusual Site in a Patient With Prader-Willi Syndrome

Panagopoulou¹,

Sattar²,

Aquilina³

et al. 2019

Journal of Pediatric Hematology/Oncology

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Medulloblastoma is the most common malignant paediatric brain tumour. Survival rates range between 50-80% depending on histology and other biological features, metastases and treatment approach. Prader-Willi syndrome (PWS) is a genetically inherited disorder characterized by dysmorphic features, mental retardation, obesity and hypogonadism among other features. We describe a 10.5-year-old girl with PWS and previous standard-risk medulloblastoma that relapsed in the pons three years after the end of treatment. Diagnosis of relapse was delayed by a preceding varicella infection, an initial clinical/radiological response to steroids and the unusual location and was confirmed with a stereotactic biopsy. The patient did not respond to secondline treatment. This is the first report of a medulloblastoma in a patient with PWS

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“…Patients diagnosed with Mixed Gonadal Dysgenesis (45X/46XY) are at increased risk for gonadal tumors, impaired fertility, and fibrosis as a result of ongoing structural changes within the reproductive tissue [ 41 ]. Genetic conditions related to poor testicular development often observed in patients diagnosed with the Down (47, XX/XY, +21) [ 42 ] or Prader-Willi syndrome (15q11-13 deletions) [ 43 ] may lead to elevated risks for TC occurrence. On the contrary, it was postulated that men suffering from the Klinefelter syndrome (47, XXY)—the most common sex chromosome disorder in males affecting approximately one out of every 600 men [ 44 ] have lower chances for developing a prostate malignancy [ 45 ] due to hypogonadism and chronically low circulating androgen levels [ 46 ].…”

Section: Causes and Factors Governing The Association Between Malementioning

confidence: 99%

Male Reproductive Cancers and Infertility: A Mutual Relationship

Tvrdá

Agarwal

Alkuhaimi

2015

IJMS

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Reproductive dysfunction and malignancies related to the male gender represent a serious health concern, whose incidence has significantly risen over the past years. Prior to treatment, testicular or prostate cancer patients often display poor semen characteristics similar to subfertile or infertile patients. This fact is underscored by cases where the malignancy is often diagnosed in males who undergo a general fertility screening. This review aims to examine the associations between male infertility and reproductive cancers focusing on common etiologies and biological mechanisms underlining these pathologies. Furthermore, we discuss compelling epidemiological data hypothesizing that male reproductive failure may act as a precursor of future andrological malignancies, including testicular or prostate cancer, thus providing a stimulus for a more specific research in male reproductive health and emphasizing the importance of this relation for physicians taking care of male patients with a reproductive disease.

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Cancer incidence among persons Prader-Willi syndrome in Finland

Cited by 6 publications

References 24 publications

Prevalence and incidence of physical health conditions in people with intellectual disability – a systematic review

Prevalence and incidence of physical health conditions in people with intellectual disability – a systematic review

Challenges in the Diagnosis of Medulloblastoma Recurrence at an Unusual Site in a Patient With Prader-Willi Syndrome

Male Reproductive Cancers and Infertility: A Mutual Relationship

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