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Table 2 Suggested follow-up imaging for patients with fibrous dysplasia, McCune-Albright syndrome and Mazabraud syndrome (an expert opinion, based on the NIH cohort)

From: Fibrous dysplasia for radiologists: beyond ground glass bone matrix

Involvement Organs involved Frequency of involvementa Clinical problem Suggested radiological follow-up
Bone lesions All lesions 100% Fractures, benign and malignant matrix transformation Initial bone scan to assess the extension of disease. CT of the affected area/bones to evaluate changes in pain, rapid enlargement, local changes.
Craniofacial bones 80% Vision/Hearing Head CT at baseline. Repeat periodically in childhood to monitor progression. Repeat as needed for symptomatic lesions in adulthood.
Femur 91% Deformities Measure neck-shaft angle to identify progressive femoral neck deformation on X-ray.
Axial skeleton 63% Scoliosis Closely monitor for scoliosis on X-ray; surgical fixation if Cobb angle > 50 degrees.
Extra-skeletal Thyroid 66% Hyperthyroidism (38%), autoimmune thyroiditis, thyroid cancer (1.3%) Thyroid ultrasound at baseline and periodically to follow abnormalities.
Pituitary 10–15% Adenoma, hyperplasia without adenoma MRI brain at baseline for patients with abnormal pituitary function.
Testicles 85% Macroorchidism, Leydig or Sertoli cell hyperplasia, testicular germ cell tumour Testicular ultrasound at baseline and periodically to follow abnormalities.
Ovaries 85% Autonomous ovarian cysts Pelvic US if breast development, vaginal bleeding or signs of estrogenisation below age 6–7 years.
GI tract 32% Pancreatic IPMN MRI of abdomen with MRCP follow-up in 6–12 months if IPMNs 10–20 mm; 6 months follow-up if > 20 mm or demonstrates suspicious features
Intramuscular myxomas in Mazabraud syndrome 100% Asymptomatic No follow-up
  1. aIn the NIH cohort