- University of Iowa, Carver College of Medicine, Iowa City, IA, USA
- Department of Neurosurgery, University of Iowa Hospitals and Clinics, Iowa City, IA, USA
- Department of Pathology, University of Iowa Hospitals and Clinics, Iowa City, IA, USA
- Department of Radiology, University of Iowa Hospitals and Clinics, Iowa City, IA, USA
1Department of Neurosurgery, University of Iowa Hospitals and Clinics, Iowa City, IA, USA
DOI:10.4103/2152-7806.149848Copyright: © 2015 Park B. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
How to cite this article: Park B, Abode-Iyamah K, Lee SL, Kirby P, El-Khoury G, Wilson S. Fibro-osseous lesion of the cranium in an adolescent patient. Surg Neurol Int 22-Jan-2015;6:12
How to cite this URL: Park B, Abode-Iyamah K, Lee SL, Kirby P, El-Khoury G, Wilson S. Fibro-osseous lesion of the cranium in an adolescent patient. Surg Neurol Int 22-Jan-2015;6:12. Available from: http://sni.wpengine.com/surgicalint_articles/fibro%e2%80%91osseous-lesion-cranium-adolescent-patient/
Background:Fibrous dysplasia, ossifying fibroma, and desmoplastic fibroma are rare benign calvarial lesions, which can have local aggressive behavior. These tumors can present with similar clinical and radiologic characteristics making diagnosis difficult at times.
Case Description:A 16-year-old male presents after noting an indentation of his skull. Comparison with current and previous imaging revealed progressive erosion of the skull underlying the indentation.
Conclusion:Fibrous dysplasia, ossifying fibroma, and desmoplastic fibroma are rare fibro-osseous tumors with similar characteristics radiographically. Accurate diagnosis of these tumors can be difficult even with the combination of clinical presentation, imaging, and pathology. The treatment of choice is resection and cranial reconstruction, if necessary, with close follow-up as recurrence can occur.
Tumors of the skull are uncommon and account for 1–4% of all bone tumors.[
A 16-year-old male presented for evaluation of an indentation of his right parietal skull. The patient had first noted the indentation 2 weeks prior with no recent change in size. He had been involved in a 4-wheeler accident 2 years before and was evaluated for a possible concussion during a football game about one and a half years prior to presentation. The patient described having occasional headaches, but had no local tenderness or other neurological symptoms.
On examination, the patient was noted to have a palpable defect and indentation of the right parietal bone. The overlying scalp was unremarkable and he was neurologically intact.
The patient’s previous imaging was reviewed. The first study, from 2 years prior, showed a skull defect in the right parietal bone [
CT scan of the brain from March 16, 2012 (a) shows bony lesion with predominant ground glass appearance which is extracranial. Additional CT on September 14, 2012 (b) and February 21, 2014 (c) shows progression of the bony lesion with increase in the area of lucency. (d) post-op CT showing reconstruction with titanium mesh. (E-f) pre-operative showing extramedullary lesion with dural enhancement
Excision of the skull defect and right parietal cranioplasty with titanium plate was performed. The overlying tissue was grossly abnormal, measuring approximately 5 cm in diameter, and was removed en bloc. The underlying bone was thin and eggshell like with a trabeculated pattern, especially in the center. Peripherally, the bone was thickened with a sponge-like appearance. The abnormal tissue portion could be seen replacing the diploic space while leaving the inner and outer table intact at the periphery. The dura was not involved and was stripped from the bone and left intact. The abnormal bone was then removed and a mesh titanium plate was used to replace the cranial defect [Figures
Lateral (a) and AP (b) X-ray showing an osteofibrous dysplasia of the tibia (broken arrow). Sagittal postcontrast MRI (c) using enhancement of the tibial lesion (broken arrow). This lesion is synonymous to ossifying fibroma and is name based on its location. Sagittal (d) and axial (g) postcontrast MRI showing fibrous dysplasia (thin arrow). Coronal (e) and axial (f) CT scan showing fibrous dysplasia (thin arrow). Lateral skull X-ray (h) and axial CT (i) of a desmoplastic fibroma[ 8]
The patient tolerated the operation well and had no immediate operative or perioperative complications. The right parietal indentation was corrected and the patient was discharged on postoperative day 2.
On gross examination, the soft tissue lesion appeared rubbery, yellow, and avascular. The tissue consisted predominantly of dense fibrous tissue with a few foci of woven bone at the periphery adjacent to the eroded bone. Angulated spicules of bone were rare, some of which lacked osteoblastic rimming while others were rimmed. There was minimal inflammation. The final diagnosis was that of a benign fibro-osseous lesion most in keeping with a DF. The case was reviewed at the Mayo Clinic where it was felt to be an atypical FD. Conflicting pathology interpretations of these lesions is not uncommon.
FD accounts for 2.5% of benign bone tumors and 7% of all bone tumors.[
FD can present as monostotic, polyostotic, or McCune–Albright syndrome subtypes. The monostotic subtype is six times more common. FD involving the cranium tends to be monostotic in nature. FD involving the femur, tibia, pelvis, and spine tend to be polyostotic. Polyostotic FD is often associated with McCune–Albright syndrome and tends to be more severe with an earlier onset. McCune–Albright syndrome is characterized by Albright’s triad of early onset of puberty in females, cutaneous hyperpigmentation, and endocrinopathies. A somatic mutation in the G-protein alpha subunit gene GNAS1 may be responsible for causing FD.[
OF is a slowly growing benign tumor that is locally aggressive with a high recurrence rate. It tends to occur mostly in the third and fourth decades of life. Both sexes are affected equally. Aggressive behavior can occur in young patients with a gradual decrease in aggressiveness with age. OF affects the head and neck region and is analogous to osteofibrous dysplasia listed in the WHO classification, which mainly affects the tibia. OF is most often found in the mandible and maxilla and is less commonly found in the nasal bones, ethmoid cells, orbit, and cranium. Typical patients present with cranial asymmetry and swelling.[
DF is rare, making up 0.3% of benign bone tumors and 0.06% of all bone neoplasms.[
Gardini et al. first described a case of DF affecting the skull in 1978 and a total of 20 cases have been reported[
Radiographically, FD has a sclerotic, lytic, or pseudopagetic appearance. There is a characteristic ground glass matrix in the sclerotic variant, which is the most common type, making up 50% of FD. The lytic form is characterized by radiolucency, while the pseudopagetic variant is a combination of the sclerotic and lytic forms. FD is nonaggressive and often localized between diploe. There is normally no periosteal reaction or soft tissue extension. CT scans are particularly helpful in visualizing the ground glass pattern [Figure
OF is a radiolucent, well demarcated round or oval mass with thinning cortical bone boundaries resembling an eggshell. Older lesions are visibly calcified. CT scans show a cortical epicenter to the lesion that is separated from medullary bone by sclerosis and does not invade the soft tissue [Figure
DF is osteolytic with expansion of the bone with the original cortex being replaced by a thin shell of new bone. The lytic area has a trabeculated or bubble-like appearance.[
Although there are radiographic characteristics to look for that can help in differentiating these tumors, there are similarities that make this difficult and correlation with pathological evaluation is crucial.[
Grossly, FD is tan to gray or white with a gritty, firm texture. Cysts filled with yellowish fluid can be found. Histologically, FD tends to be well circumscribed with fibrous and osseous components. The fibrous component has bland spindle-shaped cells with a low mitotic rate and the osseous component has curvilinear trabeculae of bone lacking osteoblastic rimming, an important diagnostic feature. Foamy cells, multinucleate giant cells, secondary aneurysmal bone cyst, or myxoid change may be present [Figure
Desmoplastic fibroma. (a) Dense fibrous tissue with spicules of woven and laminated bone at periphery. (b) Uniform spindle cells in the stroma. (c) Laminated bone at periphery with osteoblastic rimming. (d) Woven bone with osteoblastic rimming. Fibrous dysplasia. (e) fibrous stroma with angulated spicules of bone. (f) Spicules of bone with no osteoblastic rimming. (g) Occasionally osteoclasts can be seen. (h) Spicule of bone without osteoblastic rimming. Ossifying fibroma.(i) Cellular stroma with spicules of bone. (j) Spicule of bone with osteoblastic rimming
OF is grossly white to yellow, or red in color and soft or gritty in texture. The cortex is thinned, but periosteum is intact. There is usually a sclerotic rim. OF shows irregular fragments or spicules of woven bone with osteoblastic rimming of lamellar bone with occasional osteoclasts. The fibrous component consists of bland spindle cells. The center of the lesion consists mainly of the spicules of bone and the peripheral lamellar bone often blends with the normal surrounding bone [Figure
DF is grossly white to tan in color with a rubbery texture. Histologically it is hypocellular composed of fibroblasts with ovoid to spindled nuclei in a variably hyalinized collagenous matrix [Figure
Treatment of skull FD involves surgical removal of the lesion. Total resection with craniofacial or cranial reconstruction is the preferred method of treatment, although nerve function and aesthetics should be taken into consideration. This yields a good prognosis with a low rate of recurrence. In a retrospective study following 81 patients with FD of the skull, 13.7% of the follow-up patients with complete excision had tumor recurrence with 0–2 cm variance in margin widths.[
The locally aggressive nature of OF makes surgical resection the preferred treatment. Despite this, when OF involves areas around vital structures, periodic removal of the lesion and preservation of the vital structures with follow-up is warranted. Reported rates of recurrence vary from 0% to 28% after total primary resection and are higher in subtotal resections.[
The treatment of choice for DF of the skull is complete resection, with tumor-free margins due to the locally aggressive and infiltrative nature of DF.[
In our patient, an en bloc resection of the affected portion of the skull was performed, while leaving the uninvolved dura intact [Figure
Nonsurgical management of FD is not effective.[
FD, OF, and DF of the skull are rare and often present with somewhat similar clinical and radiographic features. In our case, the radiological diagnosis was that of an OF and the initial pathology was that of a fibro-osseous lesion most in keeping with a DF. Review at another institution suggested an atypical FD. Our case highlights that the difficulty in diagnosing these bony tumors of the skull. Diagnosis requires consideration of the clinical, radiographic, and pathology. The preferred treatment for all three involves en bloc surgical resection due to potential locally aggressive behavior. Regular follow up is strongly recommended.
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