Korean J Med.  2016 Feb;90(2):154-158. 10.3904/kjm.2016.90.2.154.

Three Types of Cancer in a Patient with Neurofibromatosis Type I: Gastrointestinal Stromal Tumor, Breast and Ampulla of Vater Cancer

Affiliations
  • 1Department of Internal Medicine, Gangnam Severance Hospital, Yonsei University College of Medicine, Seoul, Korea. CHOJY@yuhs.ac

Abstract

Neurofibromatosis type 1 (NF-1) is an autosomal dominant disorder with a prevalence of approximately 1 in 3,500 live births. NF-1 predisposes to various benign and malignant neoplasms. Neurological malignancies are most frequent, but the risks of non-nervous system tumors, such as of the esophagus, stomach, colon, liver, biliary tract, pancreas, lung, melanoma, thyroid gland, female breast and ovaries, are also increased. Malignant tumors are the most common cause of death in patients with NF-1. Cases with double primary tumors have been reported, but cases involving three or more primary cancers are rarely reported. Therefore, we present the case of a NF-1 patient diagnosed with gastrointestinal stromal tumor, breast cancer and ampulla of Vater cancer.

Keyword

Neurofibromatosis; Gastrointestinal stromal tumors; Breast neoplasm; Ampulla of vater

MeSH Terms

Ampulla of Vater*
Biliary Tract
Breast Neoplasms
Breast*
Cause of Death
Colon
Esophagus
Female
Gastrointestinal Stromal Tumors*
Humans
Live Birth
Liver
Lung
Melanoma
Neurofibromatoses*
Neurofibromatosis 1*
Ovary
Pancreas
Prevalence
Stomach
Thyroid Gland
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