McCune-Albright syndrome (MAS) is a rare heterogeneous genetic disorder that is characterized by a triad of polyostotic fibrous dysplasia (FD), café au lait spots (CAL), and multiple hyperfunctional endocrinopathies. In general, it is diagnosed clinically. From the triads, 2 of the findings are enough to make the diagnosis.
Craniofacial fibrous dysplasia is a term that is used to describe the fibrous dysplasia, which was localized at the craniofacial skeleton and is common in MAS patients.
Aneurysmal bone cyst (ABC) is a rare non-neoplastic bone lesion that involves mostly the long bones and vertebrae and may occur very rarely in the craniofacial bones. ABCs may occur as secondary bony pathologies in association with various benign and malignant bone tumors and with fibrous dysplasia. Secondary ABC occurring in craniofacial FD is extremely rare. We present the case of a 21-year-old patient treated at our center for a right orbital aneurysmal bone cyst associated with MAS and provide a review of the relevant literature.
El síndrome de McCune-Albright (SMA) es un trastorno genético heterogéneo que se caracteriza por la tríada de displasia fibrosa (DF) poliostótica, manchas café con leche y múltiples endocrinopatías hiperfuncionales. En general, se diagnostica clínicamente. De la tríada, 2 de los hallazgos son suficientes para hacer el diagnóstico. La DF craneofacial es un término que se usa para describir la displasia fibrosa, que se localizaba en el esqueleto craneofacial y es común en pacientes con SMA.
El quiste óseo aneurismático (QOA) es una lesión ósea no neoplásica infrecuente que afecta principalmente a los huesos largos y las vértebras, y puede ocurrir muy raramente en los huesos craneofaciales. Los QOA pueden ocurrir como enfermedades óseas secundarias en asociación con varios tumores óseos benignos y malignos y con displasia fibrosa. El QOA secundario que ocurre en la DF craneofacial es excepcional. Presentamos el caso de una paciente de 21 años tratada en nuestro centro de un quiste óseo aneurismático orbitario derecho asociado a SMA y realizamos una revisión de la literatura relevante.
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