About the Clinic

The Pediatric and Rare Tumor Clinic at Huntsman Cancer Institute (HCI) focuses on hereditary syndromes that cause an increased risk for cancer and tumors in children and adults. These specialists work to meet all of your family's needs:

  • Pediatric oncologists
  • Genetic counselors
  • Clinical psychologists
We can help families understand genetic testing results and develop personalized cancer screening plans to help detect cancer at the earliest possible stage. We can also inform families about research opportunities.

Frequently Asked Questions

Who can visit the clinic?

The Pediatric and Rare Tumor Clinic is for children and adults who have an increased risk of cancer due to family history or rare hereditary cancer conditions. 

Specific syndromes or gene mutations include the following:

  • Li-Fraumeni syndrome (LFS) due to genetic mutations in TP53
  • DICER1 Tumor Predisposition Syndrome due to genetic mutations in DICER1
  • PTEN Hamartoma Tumor Syndrome (also called Cowden Syndrome)
  • Hereditary Retinoblastoma due to mutations in RB1
  • Gorlin Syndrome or Basal Cell Nevus Syndrome due to mutations in PTCH1 or SUFU
  • Familial Wilms Tumor
  • Multiple Endocrine Neoplasia
  • Familial Neuroblastoma due to mutations in ALK or PHOX2B
  • Constitutional Mismatch Repair Deficiency
  • Rhabdoid Tumor Predisposition Syndrome/Schwannomatosis due to mutations in SMARCB1
  • Predisposition to Small Cell Carcinoma of the Ovary Hypercalcemic Type due to SMARCA4

Some families may have a history of cancers in childhood or rare tumors, but a specific genetic cause cannot be identified. These families may still benefit from a cancer screening plan.

Families with concerns and questions about their history of cancer can also be seen in this clinic. Examples include families with multiple cases of brain tumors, sarcomas, or relatives diagnosed with cancer in childhood.

Please call 801-587-9555 if your genetic tests show different mutations or a syndrome not listed above. Our genetic counselor can help you decide if this clinic is right for you.

When I visit the clinic, what will I do?

  • Review your personal and family health history.
  • Review your risks for cancer based on your type of gene mutation or syndrome.
  • Meet with a genetic counselor, clinical psychologist, and oncologist specializing in hereditary cancer syndromes.
  • Get a physical exam.
  • Discuss enrollment in research projects.
  • Plan follow-up screening and surveillance.

Do I need genetic testing before I come?

No. Your family history of cancer may be strong enough to qualify you for this clinic. If you haven’t been tested, your visit can start with genetic counseling to determine what tests you may need.

About Li-Fraumeni Syndrome

Li-Fraumeni syndrome (LFS) raises the risk of getting many types of cancer. LFS happens as a result of changes (also called mutations or variants) in the TP53 gene. LFS-related cancers often happen when the person is a child or young adult, though they can also occur at older ages.

People with LFS have a 1 in 2 chance to get cancer by age 30. Almost all women with LFS (93 in 100) get cancer in their lifetime. The lifetime cancer risk is somewhat lower for men with LFS (68 in 100).

The risk of getting cancer depends on gene mutation type, personal cancer history, and family cancer history. Classic LFS patients may be diagnosed with a core LFS cancer at an earlier age. Non-classic LFS patients may develop a core LFS cancer or have completely different cancers at older ages.

People with LFS often get one or more of these core cancers:

They may also get these cancers:

Cancer screening helps find cancer at an early stage, when it is easier to treat. HCI’s LFS experts have a dedicated screening program that includes MRI surveillance. Contact us at 801-587-9555 to find out if you need genetic testing.

Contact Us

If you or a family member would like to learn more, please call us to get in touch with a genetic counselor: 801-587-9555