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PTEN hamartomatous tumor syndromes (PHTS): rare syndromes with great relevance to common cancers and targeted drug development.

Publication ,  Journal Article
Lopiccolo, J; Ballas, MS; Dennis, PA
Published in: Crit Rev Oncol Hematol
September 2007

Phosphatase and tensin homologue deleted on chromosome 10 (PTEN) is a tumor suppressor gene located on chromosome 10q22-23 that negatively regulates the pro-survival PI3K/Akt/mTOR pathway by functioning as a lipid phosphatase. Signaling through this pathway promotes cellular transformation and survival as well as resistance to chemotherapy and radiation. Loss of PTEN function is commonly observed in human cancers through somatic mutation, hypermethylation, and/or enhanced degradation. PTEN hamartomatous tumor syndromes (PHTS) are a collection of rare clinical syndromes marked by germline PTEN loss. Compared to the general population, PHTS patients have an increased risk of developing certain cancers and can develop benign tumors in virtually any organ. These patients provide a unique opportunity to examine the role of PTEN in human tumorigenesis, as well as study genotype-phenotype relationships. Because these patients are at higher risk of developing malignancies and have no established medical therapies, early screening, surveillance, and preventive care are important issues. Inhibitors of the PI3K/Akt/mTOR pathway that are being developed as cancer therapeutics could provide new therapeutic options for these rare patients, and could be credentialed as pathway inhibitors prior to testing in the general oncology population.

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Published In

Crit Rev Oncol Hematol

DOI

ISSN

1040-8428

Publication Date

September 2007

Volume

63

Issue

3

Start / End Page

203 / 214

Location

Netherlands

Related Subject Headings

  • Signal Transduction
  • PTEN Phosphohydrolase
  • Oncology & Carcinogenesis
  • Neoplasms
  • Humans
  • Hamartoma Syndrome, Multiple
  • Drug Delivery Systems
  • 3211 Oncology and carcinogenesis
  • 3201 Cardiovascular medicine and haematology
  • 1102 Cardiorespiratory Medicine and Haematology
 

Citation

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ICMJE
MLA
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Lopiccolo, J., Ballas, M. S., & Dennis, P. A. (2007). PTEN hamartomatous tumor syndromes (PHTS): rare syndromes with great relevance to common cancers and targeted drug development. Crit Rev Oncol Hematol, 63(3), 203–214. https://doi.org/10.1016/j.critrevonc.2007.06.002
Lopiccolo, Jaclyn, Marc S. Ballas, and Phillip A. Dennis. “PTEN hamartomatous tumor syndromes (PHTS): rare syndromes with great relevance to common cancers and targeted drug development.Crit Rev Oncol Hematol 63, no. 3 (September 2007): 203–14. https://doi.org/10.1016/j.critrevonc.2007.06.002.
Lopiccolo, Jaclyn, et al. “PTEN hamartomatous tumor syndromes (PHTS): rare syndromes with great relevance to common cancers and targeted drug development.Crit Rev Oncol Hematol, vol. 63, no. 3, Sept. 2007, pp. 203–14. Pubmed, doi:10.1016/j.critrevonc.2007.06.002.
Lopiccolo J, Ballas MS, Dennis PA. PTEN hamartomatous tumor syndromes (PHTS): rare syndromes with great relevance to common cancers and targeted drug development. Crit Rev Oncol Hematol. 2007 Sep;63(3):203–214.
Journal cover image

Published In

Crit Rev Oncol Hematol

DOI

ISSN

1040-8428

Publication Date

September 2007

Volume

63

Issue

3

Start / End Page

203 / 214

Location

Netherlands

Related Subject Headings

  • Signal Transduction
  • PTEN Phosphohydrolase
  • Oncology & Carcinogenesis
  • Neoplasms
  • Humans
  • Hamartoma Syndrome, Multiple
  • Drug Delivery Systems
  • 3211 Oncology and carcinogenesis
  • 3201 Cardiovascular medicine and haematology
  • 1102 Cardiorespiratory Medicine and Haematology