Fibrous dysplasia and aneurysmal bone cyst of the skull base presenting with blindness: a report of a rare locally aggressive example
© Terkawi et al; licensee BioMed Central Ltd. 2011
Received: 10 January 2011
Accepted: 11 March 2011
Published: 11 March 2011
Fibrous dysplasia (FD) and aneurysmal bone cyst (ABC) are uncommon benign intraosseous lesions. Simultaneous occurrence of both lesions is extremely rare. We present an example of concomitant FD and ABC in a 7 year-old with left eye blindness and discharge of one month duration. Physical examination revealed a proptotic left eye and bulging of the hard palate. CT and MRI are consistent with FD and ABC that involved the sphenoid and ethmoidal bones bilaterally. Incomplete combined endonasalcranial resection was performed. The patient presented five months postoperatively with a large recurrence and subsequent follow up was lost. Concomitant FD with ABC may occur in paranasal sinuses and may develop rapidly and exhibit locally aggressive behavior.
Fibrous dysplasia (FD) consists of rare and benign osseous lesions of unknown etiology. They represent 2.5% of all bone tumors and 7% of benign bone tumors , in young, predominantly male patients [2, 3]. The salient pathological feature of FD consists of the replacement of medullary bone by histologically benign fibro-osseous tissue . FD may present as monostotic (70%), polystotic (30%), or as the main feature of McCune-Albright syndrome . Craniofacial bones can be affected by monostotic (25%-30%) and polystatic (in 50%) lesions . The most affected craniofacial bones are maxilla, mandible, frontal, sphenoid and temporal bones .
Aneurysmal bone cyst (ABC) is rare, benign vascular lesion, and considered secondary to certain pathological bone lesions . ABC represents approximately 1.4% of all bone tumors, and only 3% among those are located in the cranium . These lesions occur most commonly in patients under 20 years old of both genders . Radiographs reveal an eccentric, lytic lesion typically with an expanded, remodeled "blown-out" or "ballooned" bony contour of the affected bone, with a delicate trabeculated appearance frequently. Fluid-filled spaces are common and may be seen on CT scans and MR images .
Concomitant FD and ABC are extremely rare with only 13 cases reported previously [9–12]. The clinical presentation of both FD and ABC depends on their location and the extent of involvement. The majority of reported cases are predominant in male children and young adults that are presented with symptomatic or asymptomatic mass. The development of ABC in FB may hastens the course of presentation . In general, a complete excision of these lesions is recommended, and reports of aggressive behavior are occasionally found.
We report a rare example of concomitant FD and ABC of the skull base that presented with blindness and rapid local recurrence in a 7 year-old female patient. Also, we are presenting the multidisciplinary team approach of treating such cases.
A 7 year-old female, is presented at a local hospital with left eye blindness and discharge of one-month duration.
A combined endonasal-cranial approach to resect this lesion that entailed an extradural bi-coronal craniotomy (pericranium saving technique for closure) and medial bilateral orbitotomy was performed (Figure 2, B). The preoperative assessment of the tumor (using the neuronavigation system) revealed a volume of 88.67 cc, approximately 95% (84.17 cc) of the tumor was removed. 4.5 cc was left over the right optic nerve to preserve the vision on the unaffected eye. The intraoperative blood loss was estimated about 1700 cc. And the patient recovered without any appreciable complications, along with improvement of her proptosis. She was discharged with clinical and imaging follow up.
Five months after surgery, a recurrent large tumor was noticed, estimated to be 30.5 cc (Figure 2, C). The patient's parents declined any further treatment, and the patient was lost to follow up.
Clinical, radiological findings, and treatment of reported combined fibrous dysplasia and aneurysmal bone cyst of the skull
Age (years) Gender
Składzień et al., 2008 
Rt Maxillary, orbital
Epistaxis and chronic rhinosinusitis
Large cystic lesion
9 mo disease free
Rappaport, 1989 
CT: pagetoid changes and hypodense lesion
Wojno and McCathy, 1994 
CT: nonhomogeneous cystic lesion
2 yr disease free
Wojno and McCathy, 1994 
Mass appear after head trauma
CT: diffuse thickening of the calvarium, Lt frontal cyst
Haddad et al., 1998 
Rt painless temporal mass
CT: nonhomogeneous cystic mass.
4 yr disease free
Saito et al. 1998 
nasal cavity, sphenoid bone, and skull base
Nasal obstruction and headache for 1 yr
CT and MRI, irregular multilobulated tumor
3.5 yr disease free
Branch et al., 1986 
Lt parietal & fronto-temporal
Painful Lt and frontal parietal mass for 1 mo
CT: large cystic bone lesion in the parietal area
Branch et al., 1986 
k/c of FD for 15 yr
CT: cystic expansion of the skull
Itshayek et al., 2002 
Lt occipital bone and clivus
Painless mass of the occipital area
CT: occipital cyst lesion
Selective embolization. Followed by surgical resection.
1 yr disease free
Pasquini et al., 2002 
Rt maxillary sinus
Progressive and persistent Rt side epiphora and rhinosinusitis for 2 yr.
CT: Cyst-like lesion.
Transnasal endoscopic surgery
Lin et al., 2004 
Lt frontal bone
Progressive enlargement of the mass with severe headache
CT: several expansile cystic spaces
Iseri et al., 2005 
Lt occipital bone
Progressive severe headache
FD of clivus, temporal, and occipital bones.
Mattei et al., 2005 
As SAH; severe headache and nuchal rigidity.
Hemmorage and cyst
Partial surgical resection
Sphenoidal and ethmoidal bones
Lt eye loss of vision
Lt nasal obstruction
Cystic expansible lesion
Endonasal - cranial resection
Recur after 5 mo
The presentation of these lesions, as in our case, depends on the location, rate of growth and site of involvement. Symptoms may include painless mass, pressure symptoms, nasal obstruction, headache and loss of vision. Acute hemorrhage into the cyst(s) may causes pain, rapid enlargement and/or rupture and subarachnoid hemorrhage . All reported cases were treated surgically with generally good outcomes.
ABC in distal tibia has been treated by high-energy, low-dose radiation on one patient and was successful, with no recurrence during the five years follow up . However, radiation for ABC of skull base or concomitant ABC with FD residual tumors has not been reported. The aggressive nature of the present case and others suggests that adjuvant therapy may be required in some cases.
Our case represents a rapid developing fibrous dysplasia with aneurismal bone cyst at the skull base leading to unilateral blindness. Awareness of such cases may lead to early detection and diagnosis for effective therapy.
A multidisciplinary team is needed for both diagnosis (clinical, radiological, and histopathological) and management (otolaryngology, neurosurgery, and neuronavigation) in this kind of diseases.
Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.
We are grateful to Dr. Adel El-Naggar for his great influence in reviewing this manuscript, Dr. Suhail Maqbool for his helpful guidance in writing the manuscript and patient management, and Dr. Mahmoud Al-Yamany for helping in innovating and performing the skull base approach.
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