Pancreatic cancer and the FAMMM syndrome

Henry T. Lynch, Ramon M. Fusaro, Jane F. Lynch, Randall Brand

Research output: Contribution to journalArticle

71 Citations (Scopus)

Abstract

Hereditary cancer syndromes provide excellent models for molecular genetic studies that may aid significantly in case detection, surveillance, and management. Ultimately, molecularly based designer pharmaceuticals may emerge from this research, such as the case of trastuzumab (Herceptin) in HER-2/neu positive breast cancer, and imatinib (Gleevec) in chronic myelocytic leukemia and gastrointestinal stromal tumors. Importantly, these molecular findings may fuel significant clues to cancer control. This background is mentioned since surveillance and management of pancreatic cancer, a major concern of this manuscript, has been uniformly unsuccessful as evidenced by the close correspondence between its incidence and its mortality. Yet knowledge about its genetic and molecular pathology will hopefully ameliorate this vexing problem. One molecular genetic clue is the recently identified palladin mutation in two pancreatic cancer prone families. However, caution must be used toward the palladin mutation, as several recent publications have questioned its significance as a pancreatic cancer causing mutation. We provide a concise description of pancreatic cancer in concert with malignant melanoma in the familial atypical multiple mole melanoma (FAMMM) syndrome as a potential preventive model. This knowledge should help clinicians and basic scientists seize on the opportunity to develop more sensitive and specific screening and management programs in this disease; while a relatively small subset of pancreatic cancer may be readily identifiable through its FAMMM phenotype, coupled with its CDKN2A mutation, this hereditary disorder, given a keen knowledge of its natural history and molecular genetics, may prove to be an effective clinical preventive model.

Original languageEnglish
Pages (from-to)103-112
Number of pages10
JournalFamilial Cancer
Volume7
Issue number1
DOIs
StatePublished - 2008

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Dysplastic Nevus Syndrome
Pancreatic Neoplasms
Molecular Biology
Mutation
Hereditary Neoplastic Syndromes
Designer Drugs
Gastrointestinal Stromal Tumors
Molecular Pathology
Leukemia, Myelogenous, Chronic, BCR-ABL Positive
Natural History
Melanoma
Breast Neoplasms
Phenotype
Mortality
Incidence
Research
Neoplasms

All Science Journal Classification (ASJC) codes

  • Cancer Research
  • Genetics
  • Oncology
  • Genetics(clinical)

Cite this

Pancreatic cancer and the FAMMM syndrome. / Lynch, Henry T.; Fusaro, Ramon M.; Lynch, Jane F.; Brand, Randall.

In: Familial Cancer, Vol. 7, No. 1, 2008, p. 103-112.

Research output: Contribution to journalArticle

Lynch, HT, Fusaro, RM, Lynch, JF & Brand, R 2008, 'Pancreatic cancer and the FAMMM syndrome', Familial Cancer, vol. 7, no. 1, pp. 103-112. https://doi.org/10.1007/s10689-007-9166-4
Lynch, Henry T. ; Fusaro, Ramon M. ; Lynch, Jane F. ; Brand, Randall. / Pancreatic cancer and the FAMMM syndrome. In: Familial Cancer. 2008 ; Vol. 7, No. 1. pp. 103-112.
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