Journal Information
Vol. 34. Issue 5.
Pages 268-272 (September - October 2023)
Share
Share
Download PDF
More article options
Visits
11
Vol. 34. Issue 5.
Pages 268-272 (September - October 2023)
Case Report
Craniofacial fibrous dysplasia and aneurismal bone cyst in a patient with McCune-Albright syndrome. A case report and review of the literature
Displasia fibrosa craneofacial y quiste óseo aneurismático en una paciente con síndrome de McCune-Albright. Presentación de un caso y revisión de la literatura
Visits
11
Julián Castro Castroa,
Corresponding author
juliancastrocastro@yahoo.es

Corresponding author.
, Juan Manuel Villa Fernándezb, Jesús Patricio Agulleiro Díazc
a Servicio de Neurocirugía, Complexo Hospitalario Universitario de Ourense, Instituto de Investigación Sanitaria Galicia Sur, Ourense, Spain
b Servicio de Neurocirugía, Complexo Hospitalario Universitario de A Coruña, A Coruña, Spain
c Servicio de Neurocirugía, Complexo Hospitalario Universitario de Santiago, Santiago de Compostela, A Coruña, Spain
This item has received
Article information
Abstract
Full Text
Bibliography
Download PDF
Statistics
Figures (3)
Show moreShow less
Abstract

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.

Keywords:
Aneurismal bone cyst
Fibrous dysplasia
McCune-Albright syndrome
Craniotomy
Resumen

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.

Palabras clave:
Craneotomía
Displasia fibrosa
Quiste óseo aneurismático
Síndrome de McCune-Albright

Article

These are the options to access the full texts of the publication Neurocirugía (English edition)
Member
Member of the Sociedad Española de Neurocirugía

If it is the first time you have accessed you can obtain your credentials by contacting Elsevier Spain in suscripciones@elsevier.com or by calling our Customer Service at902 88 87 40 if you are calling from Spain or at +34 932 418 800 (from 9 to 18h., GMT + 1) if you are calling outside of Spain.

If you already have your login data, please click here .

If you have forgotten your password you can you can recover it by clicking here and selecting the option ¿I have forgotten my password¿.

Subscriber
Subscriber

If you already have your login data, please click here .

If you have forgotten your password you can you can recover it by clicking here and selecting the option “I have forgotten my password”
Subscribe
Subscribe to

Neurocirugía (English edition)

Purchase
Purchase article

Purchasing article the PDF version will be downloaded

Price 19.34 €

Purchase now
Contact
Phone for subscriptions and reporting of errors
From Monday to Friday from 9 a.m. to 6 p.m. (GMT + 1) except for the months of July and August which will be from 9 a.m. to 3 p.m.
Calls from Spain
932 415 960
Calls from outside Spain
+34 932 415 960
Email
Idiomas
Neurocirugía (English edition)
Article options
Tools
es en

¿Es usted profesional sanitario apto para prescribir o dispensar medicamentos?

Are you a health professional able to prescribe or dispense drugs?