PMC9852710_01
Representative images of the patient indicating McCune-Albright syndrome. Photographs of the back (A) from this patient demonstrates characteristic light brown pigmented birthmarks with jagged borders. These flat patches occur around the midline of the back and are known as Cafe-au-lait spots. The appearance of the patient's face (B) is indicative of a fibrous dysplasia disorder.
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F1
The patient, a now 28-year-old male, was born with cafe-au-lait macules on the left side of the back (Figure 1A). In 2014, his facial deformities worsened, most notably in the left mandible, while he presented with more symptoms including widened teeth, wide lips, enlarged nose, occasional palpitations, and sleep snoring (Figure 1B).
Radiographic features of the patient before surgery demonstrating fibrous dysplasia and an enlarged thyroid. The lateral skull radiograph acquired from a CT (A) indicates polyostotic fibrous dysplasia on the head (blue arrows). X-ray from the patient (B,C) shows thoraco-lumbar scoliosis. CT image of patient upper chest (D) demonstrates the enlarged retrosternal goiter.
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F2
Polyostotic fibrous dysplasia was prominent on CT with the presence of a thickened skull plate, asymmetrical facial bone, and the osteolytic changes of the mandible (Figure 2A). Chest radiograph revealed scoliosis and kyphosis, together with a thoracic deformity (Figures 2B,C). Due to the compression from the goiter, the diameter of the trachea was approximately 18 mm at the narrowest point (Figure 2D).
Resected thyroid gland from the patient.
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F3
Cut surface reveled asymmetrical multinodular changes, the largest being 2.5 cm in diameter (Figure 3).
Histopathological images of the resected thyroid specimen. (A) Adenomatous nodular goiter with cystic changes (magnification 40x). (B) Some thyroid follicular epithelial cells with papillary hyperplasia (magnification 100x).
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F4
In H&E-stained paraffin sections, variably-sized dilated follicles showed nodular hyperplasia, with some uniform-sized follicles showing adenomatous hyperplasia (Figures 4A,B).