Secondary aneurysmal bone cyst in McCune-Albright syndrome

Abstract

SummaryPolyostotic fibrous dysplasia in combination with café-au-lait macules and hyperfunctioning endocrinopathies consists of a rare clinical condition termed as McCuneAlbright syndrome. Aneurysmal bone cysts are tumorlike cystic lesions, composed of blood-filled compartments. They may occur as primary lesions or secondary to other pathologies; most commonly giant cell tumors of bone. However, secondary aneurysmal bone cysts in McCune-Albright syndrome are exceptional. We present a 28-year-old female with McCune-Albright syndrome. She experienced precocious puberty at age 3 months. In childhood, she experienced multiple long bone fractures, facial deformity and progressive visual and hearing impairment. One year ago, she experienced a painful, gradually enlarging bone lesion involving the right ilium, pubic and ischial bone with groundglass appearance, septa, marginal sclerosis, endosteal scalloping and blow-out expansion resulting in localized thinning of the cortex. CT-guided needle biopsy of the pelvic lesion showed aneurysmal bone cyst. Selective arterial embolization was recommended, however, the patient and her relatives did not consent to proceed to treatment, and she remained in close surveillance thereafter.

Auxological and Endocrinological Features in Children With McCune Albright Syndrome: A Review

“…Magnetic Resonance could show typical signal intensity and enhancement in active lesions but it cannot discriminate FD from other entities ( 16 , 17 , 19 ). On the other hand it could be necessary for the evaluation of challenging cases of FD, such as in patients with compressions of neurological structures and in suspicion of aneurysmal bone cyst ( 16 ), a benign, tumor-like bone lesion composed of multiple cystic blood-filled compartments, exceptionally associated with MAS ( 20 , 21 ).…”

A rare pediatric case of McCune–Albright syndrome with acute visual disturbance

“…In addition, secondary changes of ABC in MAS complicated with FD are exceptional, and only 2 cases have been reported. The first reported case was in a 14-year-old girl [5] whose lesion was present in the bilateral occipital bones, and the second case was a 28-year-old woman [4] who had a pelvic lesion (Table 1). The 14-year-old girl was treated with resection and the 28-year-old woman did not agree to treatment.…”

“…[3] ABC is a rare complication of FD, and only 2 cases of ABC complicated with FD in MAS were previously reported. [4,5] We report an extremely rare case of ABC in a MAS patient who presented acute visual disturbance and who was successfully treated by percutaneous surgery and steroids.…”

“…The etiology of ABC is unclear, and secondary ABC co-existing with other legions such as giant cell tumor and chondromyxoid fibroma accounts for 30% of whole ABC [3] . ABC is a rare complication of FD, and only 2 cases of ABC complicated with FD in MAS were previously reported [4,5] …”

Rare aneurysmal bone cysts: multifocal, extraosseous, and surface variants

“…Recurrence is not uncommon, ranging from 10 to 50%. Secondary ABCs arising in association with other benign or malignant bone tumors have been reported, accounting for approximately 1/5 of the cases [1][2][3][4][5][6][7][8][9][10][11][12][13].…”