Hereditary Paraganglioma-Phaeochromocytoma Syndrome

What can I do to manage my increased risk of tumours and cancer?

Screening

Screening helps to detect (and in some cases treat) tumours and/or cancer at an early and manageable stage.

• Paraganglioma-phaeochromocytoma
  • Blood pressure and blood or urine tests to monitor for signs which may suggest the presence of a tumour.
  • Imaging scans may be recommended to check for the presence of a tumour.
• Kidney cancer (renal cell carcinoma)
  • Regular imaging (e.g., MRI or ultrasounds) can help to detect if any tumours have formed in the kidney.
• Gastric GIST / pituitary tumours
  • An endoscopy can be considered if symptoms like gastrointestinal problems or anaemia are present.
• Thyroid cancer / parathyroid tumours
  • Imaging (e.g., thyroid ultrasounds) can be considered to screen for tumours/cancer. Further blood tests can be considered if needed.

Your managing doctor(s) will discuss these options with you in greater detail. The age, onset and frequency for screening may change according to the faulty SDHx gene that you have, and your personal and/or family history of tumours/cancer. Screening guidelines may change as more information is known.

Lifestyle adjustments/considerations

• Avoid smoking
• Pregnant women with this condition should be closely monitored
• Avoid long-term exposure to highaltitude areas
• Encouraged to seek medical attention if experiencing unusual symptoms like headaches, heart palpitations and excessive sweating