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Cancer risk in patients with Peutz-Jeghers syndrome: A retrospective cohort study of 336 cases.

Abstract Peutz-Jeghers syndrome is a rare autosomal dominant inherited disorder characterized by mucocutaneous pigmentation and hamartomatous gastrointestinal polyposis. A growing body of evidence has shown that Peutz-Jeghers syndrome could cause an increased risk of various cancers, yet the range of cancer risk estimates was wide among different studies. In this retrospective cohort study, 336 patients with Peutz-Jeghers syndrome in China were enrolled. The clinical characteristics, cancer spectrum, relative cancer risks, and cumulative cancer risks were analyzed. In total, 52 patients were diagnosed of cancer in the follow-up period, at a median age of 41 years (range: 21-67). The relative risk for cancer in Peutz-Jeghers syndrome patients was 63.858 (confidence interval: 47.514-85.823), and the cumulative cancer risk at the age of 60 years was 55%. Colorectal cancer was the most common cancer for Peutz-Jeghers syndrome patients (relative risk: 237.918, confidence interval: 154.417-366.572) and the cumulative cancer risk at the age of 60 years was 28%. There was a statistically significant difference in the cumulative cancer risk between patients with family history and those without family history, as well as between patients living in rural area and those living in urban areas ( p < 0.05), while no significant effects of gender and intussusception history on the cumulative cancer risk was found ( p > 0.05). Hopefully, our study may contribute to the management of this rare disorder and establishment of related surveillance projects, especially in China.
PMID
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Authors

Mayor MeshTerms
Keywords

China

Peutz–Jeghers syndrome

cancer risk

Journal Title tumour biology : the journal of the international society for oncodevelopmental biology and medicine
Publication Year Start




PMID- 28653895
OWN - NLM
STAT- MEDLINE
DA  - 20170627
DCOM- 20170711
LR  - 20170713
IS  - 1423-0380 (Electronic)
IS  - 1010-4283 (Linking)
VI  - 39
IP  - 6
DP  - 2017 Jun
TI  - Cancer risk in patients with Peutz-Jeghers syndrome: A retrospective cohort study
      of 336 cases.
PG  - 1010428317705131
LID - 10.1177/1010428317705131 [doi]
AB  - Peutz-Jeghers syndrome is a rare autosomal dominant inherited disorder
      characterized by mucocutaneous pigmentation and hamartomatous gastrointestinal
      polyposis. A growing body of evidence has shown that Peutz-Jeghers syndrome could
      cause an increased risk of various cancers, yet the range of cancer risk
      estimates was wide among different studies. In this retrospective cohort study,
      336 patients with Peutz-Jeghers syndrome in China were enrolled. The clinical
      characteristics, cancer spectrum, relative cancer risks, and cumulative cancer
      risks were analyzed. In total, 52 patients were diagnosed of cancer in the
      follow-up period, at a median age of 41 years (range: 21-67). The relative risk
      for cancer in Peutz-Jeghers syndrome patients was 63.858 (confidence interval:
      47.514-85.823), and the cumulative cancer risk at the age of 60 years was 55%.
      Colorectal cancer was the most common cancer for Peutz-Jeghers syndrome patients 
      (relative risk: 237.918, confidence interval: 154.417-366.572) and the cumulative
      cancer risk at the age of 60 years was 28%. There was a statistically significant
      difference in the cumulative cancer risk between patients with family history and
      those without family history, as well as between patients living in rural area
      and those living in urban areas ( p &lt; 0.05), while no significant effects of
      gender and intussusception history on the cumulative cancer risk was found ( p &gt; 
      0.05). Hopefully, our study may contribute to the management of this rare
      disorder and establishment of related surveillance projects, especially in China.
FAU - Chen, Hong-Yu
AU  - Chen HY
AD  - 1 Department of Gastroenterology, Clinical College of Air Force General Hospital,
      Anhui Medical University, Beijing, China.
FAU - Jin, Xiao-Wei
AU  - Jin XW
AD  - 1 Department of Gastroenterology, Clinical College of Air Force General Hospital,
      Anhui Medical University, Beijing, China.
FAU - Li, Bai-Rong
AU  - Li BR
AD  - 1 Department of Gastroenterology, Clinical College of Air Force General Hospital,
      Anhui Medical University, Beijing, China.
FAU - Zhu, Ming
AU  - Zhu M
AD  - 1 Department of Gastroenterology, Clinical College of Air Force General Hospital,
      Anhui Medical University, Beijing, China.
FAU - Li, Jing
AU  - Li J
AD  - 1 Department of Gastroenterology, Clinical College of Air Force General Hospital,
      Anhui Medical University, Beijing, China.
FAU - Mao, Gao-Ping
AU  - Mao GP
AD  - 1 Department of Gastroenterology, Clinical College of Air Force General Hospital,
      Anhui Medical University, Beijing, China.
FAU - Zhang, Ya-Fei
AU  - Zhang YF
AD  - 1 Department of Gastroenterology, Clinical College of Air Force General Hospital,
      Anhui Medical University, Beijing, China.
AD  - 2 Department of Gastroenterology, Zhongnan Hospital of Wuhan University, Wuhan,
      China.
FAU - Ning, Shou-Bin
AU  - Ning SB
AD  - 1 Department of Gastroenterology, Clinical College of Air Force General Hospital,
      Anhui Medical University, Beijing, China.
LA  - eng
PT  - Journal Article
PL  - United States
TA  - Tumour Biol
JT  - Tumour biology : the journal of the International Society for Oncodevelopmental
      Biology and Medicine
JID - 8409922
SB  - IM
MH  - Adult
MH  - Aged
MH  - China
MH  - Colorectal Neoplasms/complications/*epidemiology/*pathology
MH  - Databases, Genetic
MH  - Female
MH  - Humans
MH  - Male
MH  - Middle Aged
MH  - Peutz-Jeghers Syndrome/complications/*epidemiology/*pathology
MH  - Retrospective Studies
MH  - Risk Factors
OTO - NOTNLM
OT  - China
OT  - Peutz-Jeghers syndrome
OT  - cancer risk
EDAT- 2017/06/28 06:00
MHDA- 2017/07/14 06:00
CRDT- 2017/06/28 06:00
AID - 10.1177/1010428317705131 [doi]
PST - ppublish
SO  - Tumour Biol. 2017 Jun;39(6):1010428317705131. doi: 10.1177/1010428317705131.