Table 2.
Grading of recommendations for the percentage of agreement or disagreement (R8–R14)
| Recommendation | Likert scale (%) | Grade | ||||
|---|---|---|---|---|---|---|
| 1, strongly agree | 2, agree | 3, neutral | 4, disagree | 5, strongly disagree | ||
| R8: Tumour screening in asymptomatic SDHA, SDHB, SDHC and SDHD-pi mutation carriers should include imaging | NA | NA | NA | NA | NA | NA |
| R8 during childhood | 51.7 | 41.4 | 6.9 | 0 | 0 | A |
| R8 during adulthood | 27.6 | 69.0 | 0 | 3.4 | 0 | A |
| R8a for non-thoracic MRI | 34.5 | 44.8 | 10.3 | 10.3 | 0 | A |
| R8a for thoracic MRI | 37.9 | 37.9 | 13.8 | 10.3 | 0 | B |
| R8b for MRI | 31.0 | 48.3 | 6.9 | 10.3 | 3.4 | A |
| R8b for PET–CT | 27.6 | 44.8 | 10.3 | 10.3 | 6.9 | B |
| R8c | 24.1 | 58.6 | 10.3 | 6.9 | 0 | A |
| R8d during childhood | 34.5 | 37.9 | 13.8 | 13.8 | 0 | B |
| R8d during adulthood | 55.2 | 27.6 | 6.9 | 6.9 | 3.4 | A |
| R8e | 48.3 | 41.4 | 3.4 | 6.9 | 0 | A |
| R8f | 62.1 | 34.5 | 3.4 | 0 | 0 | A |
| R9: SDHA, SDHB, SDHC and SDHD-pi asymptomatic mutation carriers should be followed-up on a regular basis after a negative initial work-up | 72.4 | 24.1 | 3.4 | 0 | 0 | A |
| R10: During childhood and adulthood, follow-up of SDHA, SDHB, SDHC and SDHD-pi asymptomatic mutation carriers should include clinical examination (blood pressure measurement, ideally out-of-office, and a symptom questionnaire), the same biochemical investigations as for the initial screening (for example, metanephrine and normetanephrine) and imaging by MRI (head and neck and thoracic, abdominal, and pelvic) | NA | NA | NA | NA | NA | NA |
| R10 during childhood | 55.2 | 37.9 | 3.4 | 3.4 | 0 | A |
| R10 during adulthood | 69.0 | 24.1 | 3.4 | 3.4 | 0 | A |
| R10a during childhood | 34.5 | 55.2 | 6.9 | 3.4 | 0 | A |
| R10a during adulthood | 41.4 | 48.3 | 6.9 | 3.4 | 0 | A |
| R10b during childhood | 41.4 | 44.8 | 6.9 | 6.9 | 0 | A |
| R10b during adulthood | 31.0 | 58.6 | 6.9 | 3.4 | 0 | A |
| R10c during childhood | 27.6 | 58.6 | 10.3 | 3.4 | 0 | A |
| R10c during adulthood | 34.5 | 48.3 | 0 | 17.2 | 0 | A |
| R10d during childhood | 41.4 | 37.9 | 6.9 | 13.8 | 0 | A |
| R10d during adulthood | 48.3 | 37.9 | 3.4 | 6.9 | 3.4 | A |
| R10e during childhood | 51.7 | 37.9 | 3.4 | 6.9 | 0 | A |
| R11: If an SDHA, SDHB, SDHC or SDHD-pi mutation carrier never developed any tumour related to SDH deficiency and has been asymptomatic all their life, screening tests should be delayed to every 5 years after 70 years of age and follow-up should be stopped at 80 years of age | NA | NA | NA | NA | NA | NA |
| R11 for a delayed follow-up | 13.8 | 58.6 | 10.3 | 17.2 | 0 | B |
| R11 for end of follow-up | 34.5 | 44.8 | 6.9 | 13.8 | 0 | A |
| R12: Screening should not differ between male and female individuals; however, complete screening should be performed before planning a pregnancy | 34.5 | 44.8 | 13.8 | 3.4 | 3.4 | A |
| R13: Initial screening and follow-up should not differ for asymptomatic mutation carriers whose family members developed metastatic SDHx-related PPGL or those with non-metastatic PPGL | 31.0 | 55.2 | 3.4 | 6.9 | 3.4 | A |
| R14: No additional imaging should be performed for RCC, GIST and pituitary adenoma; nevertheless, RCC and GIST should be searched for on imaging performed for PPGL screening | 27.6 | 62.1 | 6.9 | 3.4 | 0 | A |
Summary of the recommendations and grading of the experts using the Likert scale. GIST, gastrointestinal stromal tumour; NA, not applicable; PPGL, phaeochromocytoma and/or paraganglioma; RCC, renal cell carcinoma.