CASE REPORT

Aneurysmal bone cyst within fibrous dysplasia of the anteriorskull base: continued intracranial extension after endoscopicresections requiring craniofacial approach with free tissuetransfer reconstruction

Sunil Manjila & Chad A. Zender & John Weaver & Mark Rodgers &

Alan R Cohen

Received: 29 December 2012 /Accepted: 14 January 2013 /Published online: 26 February 2013# Springer-Verlag Berlin Heidelberg 2013

Introduction

Fibrous dysplasia (FD) is a rare pathology that represents2.5 % of all bone tumors and 7 % of benign bone tumors [1].It is most commonly found in female patients under the ageof 30 [2]. It is an idiopathic disorder and is a malformationcharacterized by an intramedullary cavity proliferation offibrous tissue with woven bony trabeculae and spindle-shaped fibroblasts [3]. The most affected craniofacial bonesare maxilla, mandible, frontal, sphenoid, and temporalbones [4].

Aneurysmal bone cysts (ABCs) are non-neoplasticlesions with benign pathology which can occur within FD.These tumors have no propensity to metastasize, but becomesymptomatic as a result of their tendency to be locallydestructive [5]. ABCs were originally described by Jaffeand Lichenstein in 1942 [6]. The name ABC is a misnomeras it is neither an aneurysm nor a true cyst. Seventy-five

percent of the patients having ABCs are under 20 years ofage. They are most commonly found in long bones and thespinal column with skull involvement being rare [7]. ABCsnormally present as primary osseous lesions; however, in30 % of cases, they occur secondary to other bone lesions.

The association of ABCs with a FD is a known but rareentity. Recently, the authors have cared for a child with ag-gressive FD of the anterior skull base with secondary ABCrefractory to multiple endoscopic endonasal resections. Thedefinitive treatment for the intracranial extension was an opencraniotomy with extensive skull base reconstruction using amyofascial free flap from the anterolateral thigh.

Case report

This 10-year-old Amish boy presented in 2009 with a scho-lastic decline over a 3-month span. Examination was re-markable for only mild left proptosis. CT showed a tumorof the paranasal sinuses that extended into the skull base.The lesion was a well-defined ovoid mass in the left ethmoidair cells. The mass extended into the superior aspect of theleft nasal cavity with the remodeling of the medial wall ofthe left orbit; although, the lamina papyraces appeared to begrossly intact. These findings were confirmed with a MRIthat revealed diffuse homogeneous enhancement followinggadolinium administration. The likelihood of a benign fibro-osseous lesion was considered (see Fig. 1a–c).

First operation

The patient underwent an endoscopic sinus operation fordiagnosis and removal of the tumor with sphenoidotomy,maxillary antrostomy, ethmoidectomy, and frontal sinusotomy.

S. Manjila : J. WeaverDivision of Pediatric Neurosurgery & Minimally InvasiveNeurosurgical Laboratory, University Hospitals Case MedicalCenter, Cleveland, OH, USA

C. A. ZenderDivision of Otolaryngology & Minimally InvasiveOtolaryngological, Laboratory, University Hospitals Case MedicalCenter, Cleveland, OH, USA

M. RodgersDepartment of Pathology, University Hospitals Case MedicalCenter, Cleveland, OH, USA

A. R. Cohen (*)Department of Neurosurgery, Children’s Hospital Boston, HarvardMedical School, 300 Longwood Avenue,Boston, MA 02115, USAe-mail: alan.cohen@childrens.harvard.edu

Childs Nerv Syst (2013) 29:1183–1192DOI 10.1007/s00381-013-2034-7

Surgery was uncomplicated, and pathology showed FD (seeFig. 2).

Second operation

Four months later, postoperative CT showed a newly devel-oped lesion in the ethmoid sinus. The patient underwent asecond endoscopic revision operation involving the leftethmoid maxillary and frontal sinus with resection of the

recurrent tumor in the left ethmoid sinus that was found tobe, again, consistent with fibrous dysplasia. The tumorappeared to be a thick fibrous tissue sac filling the left anterior,middle, and posterior ethmoidal cells with the base extendingto the frontal sinus ostium. The surgery was uncomplicated.

Third operation

After 3 months, a persistent left-sided tumor in the paranasalsinuses was diagnosed. CT scan of the sinuses showed a large,recurrent ovoid fibro-osseous lesion replacing the left ethmoidair cells and extending into the medial aspect of the left orbitwith additional cranial extension through the cribriform plateinto the left aspect of the anterior cranial fossa. There wasevidence of multiple hemorrhagic fluid levels within the lesion.The extension of the tumor into the left aspect of the nasalcavity had caused significant remodeling of the left middleturbinate and the left uncinate process (see Fig. 3a, b). MRIconfirmedmultiple hemorrhagic fluid levels within the focus offibrous dysplasia. The presence of multiple hemorrhagic fluidlevels within the lesion suggested the possible development ofa secondary aneurysmal bone cyst within the focus of fibrousdysplasia. Although there was mildly increase mass effect uponthe adjacent left frontal lobe, there was no definite white matteredema within the effaced left frontal lobe on T2 and FLAIRsequences. A repeat endoscopic procedure and attempted re-section of the recurrent tumor was performed without incident,and histopathology was reported as secondary aneurysmalbone cyst within fibrous dysplasia (see Fig. 4a, b).

Fourth operation

Six months later, the patient was found to have mild aniso-coria, left pupil larger than the right, and left proptosis of

Fig. 1 a–c CT scan of the sinuses with coronal and sagittal cutsshowing a well-defined ovoid left ethmoidal mass lesion with exten-sion into left nasal cavity, remodeling of medial left orbital wall andextension into anterior cranial fossa through cribriform plate. Thelesion measured approximately 2 cm mediolaterally×3.6 cm antero-posteriorly×3.8 cm craniocaudally

Fig. 2 Microscopic image of the lesion suggestive of fibrous dysplasiashowing small bony trabeculae without osteoblastic rimming embed-ded in a monotonous cellular stroma. Neither mitotic figures nornecrosis were identified (hematoxylin and eosin stain)

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9 mm, a 2–3-mm increase since his last examination. Histelecanthus and proptosis worsened over the next month. Arepeat CT showed a recurrent aneurysmal bone cyst withinfibrous dysplasia of the left anterior cranial base. A defini-tive operation to resect the intranasal portion, an attempt toresect the intracranial portion, was performed by the headand neck skull base team (the first operation performed bythe senior otolaryngologist on this paper). The patient un-derwent tumor resection by endoscopic endonasal approach,which included left endoscopic maxillectomy, orbital de-compression with resection of the medial orbital wall, leftsphenoidotomy, and resection of left anterior skull basetumor in its extradural location. The tumor was resected upto where a thin rib of bone lined the dura. Two weeks later, thefollow-up endoscopic exam revealed minimal left-sided nasalcrusting, which was debrided. The patient was asymptomatic,and the nasal cavity on the left appeared healthy. In another2 months, his proptosis completely resolved, and his

telecanthus was partially improved. Nasal endoscopy verifiedan adequate resection of the intranasal portion with a patentsphenoid sinus and left maxillary sinus. His anterior ethmoidsinus had been resected, and there was no evidence of anobvious recurrence of the bone cyst.

Fifth operation

A MRI at 1 and 2 months posttreatment revealed stable post-surgical changes and likely residual fibrous dysplasia, but nosigns of recurrent aneurysmal bone cyst. A scan at 5 monthsposttreatment showed a new enhancing mass at the left anteriorskull base. The mass extended intracranially, elevated the leftfrontal lobe, and extended into the frontal sinus (see Fig. 5a, b).Imaging showed fibrous dysplasia involving the roof of the leftorbit and cribriform plate (see Fig. 6a–c).

The senior authors decided that a multidisciplinary ap-proach with otolaryngology and neurosurgery would benecessary to eradicate the tumor and reconstruct the cranialvault appropriately. He underwent a bifrontal craniotomywith cranialization of both frontal sinuses and excision ofthe intracranial portion of the recurrent tumor (see Fig. 7a,b). After mobilizing a large bilateral vascularized pericranialgraft based on the supraorbital arteries bilaterally, a bifrontal

Fig. 3 a–b CT scan of sinonasal region showing recurrent left ethmoidfibrous dysplasia with multiple hemorrhagic fluid levels within thelesion indicating possible development of a secondary aneurysmalbone cyst. Note the left proptosis and heterogeneous ground glassmatrix within the area of bony expansion. The lesion measured ap-proximately 3.6 cm anteroposteriorly×2.3 cm mediolaterally×4.8 cmcraniocaudally

Fig. 4 a–b Images showing the aneurysmal bone cyst wall withfibrosis and underlying reactive new bone formation

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craniotomy was performed with supraglabellar bur holes asthe inferior limit. There was a firm, fleshy, whitish graytumor in the epidural space on the left subfrontal regiondeforming the orbit and elevating the left frontal lobe extra-durally. The tumor was also noted within both frontalsinuses, which were then exenterated along with the supra-orbital bone and a portion of the planum sphenoidale. Thedura was intact, and there was no CSF leak. The operativecavity was reconstructed with a myocutaneous free flapfrom the anterolateral thigh in the intracranial compartment.The free tissue transfer was supplied by the left superficialtemporal artery, which was microsurgically anatomosed tothe descending branch of the lateral circumflex femoralvessels. The deepithelialized flap was laid along the planum

sphenoidale and orbital apex posteriorly, along the perior-bita laterally and anteriorly up to the level of the thinned outdeep soft tissue over the nasal bone. On the right-hand side,it was then secured to the remaining anterior table of thefrontal bone. This allowed for complete separation of thecranial vault from the nasal cavity. It also allowed forseparation of the orbital contents from the dura to preventpulsatile enopthalmos. The surgery was uncomplicated, and

Fig. 5 a–b MRI showing postsurgical changes of the left nasal cavitycistern with reception of the superior and middle turbinates and leftethmoid air cells. Again noted is abnormal enhancement within the leftaspect of the cribriform plate and superior medial left frontal bonewhich was expansile and caused mass effect on the left globe and leftfront lobe.

Fig. 6 a–c Increase in size of expansile fibro-osseous lesion involvingthe left frontal bone and cribriform plate with elevation of the frontaldura and recurrence of proptosis. Suggestive of persistent fibrousdysplasia

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the patient was recovered in stable condition. Pathology wasfibrous dysplasia with no evidence of aneurysmal bone cyst.

Follow up

Two weeks later, CT showed no definite residual dysplasticbone except for a sub-centimeter segment involving the leftplanum sphenoidale. Orbital contents were displaced slightlycaudally and laterally, and a very small subdural fluid collec-tion was seen in the left frontal region. One month later, theperiorbital swelling had resolved almost completely. The pa-tient had no headaches or visual complaints, and the surgicalsite had healed well. The left-sided proptosis and supraorbitalswelling were markedly diminished. MRI showed postopera-tive changes: an intact myocutaneous flap, resolving fluid inthe paranasal sinuses, and mild enhancement of the adjacentmeninges. MR imaging 5 months post-surgery showed smallposterior thickening along the planum that has remained stableand no sign of recurrent tumor. Nine months post-surgery, thepatient remains asymptomatic (see Fig. 8a–c).

Discussion

The authors present an aggressive case of fibrous dysplasiawith secondary aneurysmal bone cyst of the anterior skullbase in a child with extensive intracranial extension follow-ing multiple endoscopic procedures. Although the intranasalportion of the tumor was removed endoscopically after a

definitive procedure was performed, the intracranial portionwas only controlled after a bifrontal craniotomy, and resection

Fig. 7 a–b The above pictures illustrate the cranial base defect afterexcision of fibrous dysplasia. The vascularized pericranial flap wasused as an initial separation of the cranial vault from the nasal cavity. Athick myofascial free flap from the anterolateral thigh was used as atissue transplant, contoured to fill the anterior cranial fossa defect,obliterating any potential space. Note the midline endotracheal tubeseen through the cranionasal defect

Fig. 8 a–c MRI scan of the brain after bifrontal craniotomy andresection of dysplastic bone involving left frontal and orbital regions.There is evidence of fat packing in the entire surgical bed outlining theresected bony defect

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of the tumor was performed. A free myofascial flap wascontoured to fill the anterior cranial fossa defect and obliterateany potential space, separating the intranasal and intracranialcontents and orbital contents from the dura. The rationale forusing free tissue transplantation was so that the bulk would bemaintained for the duration of the patient’s life unlike non-vascularized fat grafts, which atrophy over time.

There are three main clinical patterns of FD: monostoticfibrous dysplasia (MFD), polyostotic fibrous dysplasia(PFD), and McCune–Albright syndrome. MFD accountsfor about 70–75 % of cases with 20–25 % of them beinglocalized at the level of the craniofacial skeleton. PFDaccounts for around 30 % of cases with craniofacial involve-ment in 50 % of cases. PFDs with craniofacial involvementoccur at an earlier age and have a more aggressive course.McCune–Albright syndrome is characterized by PFD, skinpigmentation, and endocrine disturbances. It is more preva-lent in females [3]. In most cases, FD lesions stabilize atpuberty, while, in a few cases, they undergo malignanttransformation [8]. Thus, the clinical course is unpredict-able. Malignant transformation occurs in 0.5 % of patientswith monostotic disease and in 4 % of patients with poly-ostotic FD or McCune–Albright syndrome [9].

FD recurs in 5–44 % of cases [10–14]. Spontaneousremission has been reported [15, 16]. When FD involvesthe cranial base, neural structure compression can compli-cate the natural history of the disease. Craniofacial lesionsthrough mass effect can impinge on neurovascular struc-tures, resulting in visual changes, trigeminal neuralgia, hear-ing loss, headaches, and epiphora [17–21]. There is a verylittle potential for malignant degeneration in fibrous dyspla-sia, as illustrated by the fact that it occurs in less than 1 % ofnonirradiated patients. The potential for recurrence is lowand is possibly related to the age of patient, size of thelesion, presence of mitoses, or incompleteness of resection[22], but the recurrence rate does not appear to be alteredwhen a second primary lesion is present [10, 11, 13, 23, 24].

Pathologically, FD is characterized by replacement ofmature bone by immature woven bone, scarce osteoblastsand osteoclasts, and a varying number of fibroblasts.Irregular bony trabeculae make up the osseous component.Hemorrhagic areas associated with inflammatory and giantcell reactions are often present, and on occasion, the marrowis replaced by fibrous connective tissue, islets of spindlecells, cartilage, and metaplastic bone [25]. It should be notedthat histologically fibrous dysplasia and ossifying fibromasare very hard to differentiate, although ossifying fibromasare a true benign neoplasm with lamellar bone formationand osteoblastic rimming [26–28]. Clinically, it is charac-terized by slow growth and is typically asymptomatic butcan cause cosmetic sequelae. Pathologically, ABCs consistof a blood-filled space with few endothelial cells. Septaseparate the cysts and are composed of fibrous tissue with

osteoclast-like giant cells and osteoid tissue. The surround-ing bone cortex appears as a very thin bony shell [29]. In2010, Docquier et al. determined that histologic markers canbe prognostic in terms of the aggressiveness of ABCs [30].They found that the two groups, aggressive versus nonag-gressive, differed significantly in the proportion of theircellular content and their healing index. The ratio ofCD68-negative to CD68-positive cells was also significant-ly different between the two groups; however, this studyincluded exclusively primary ABCs. The lytic radiographicappearance of an aneurysmal bone cyst can simulate atelangiectatic osteosarcoma; however, in contrast to thecystic spaces within aneurysmal bone cysts, the cysticspaces in telangiectatic osteosarcoma are sites in whichtumor cells are in direct contact with areas of hemorrhageand with the cystic space [31, 32].

ABCs normally present as primary osseous lesions; how-ever, in 30 % of cases, they present as secondary to otherbone lesions such as giant cell tumors, chondroblastoma,osteoblastoma, chondromyxoid fibroma, osteosarcoma, andFD [5]. Oliveira et al. found primary ABCs to be a mesen-chymal neoplastic disease that, in two thirds of cases, pos-sesses USP6 or CDH11 rearrangements, while secondaryABCs often do not possess these rearrangements and aresimply a morphologic mimic of primary ABC and possiblyrepresent a common endpoint of differentiation in variousnon-ABC bone tumors [33].

The associated primary preexisting lesions usually deter-mine the clinical presentation of ABC. The primary osseousdisease process most commonly associated with ABC is agiant cell tumor. It has been reported to occur in 19–39 % ofcases [5]. FD presenting with ABC is extremely rare. Levy etal. in a report of 57 cases of ABC with other bony lesions didnot find a single case of ABC with FD [24]. A comprehensivereview study conducted by Martinez et al. [5] found only onecase of ABC in the subgroup of 42 patients with FD.

Fries described the radiographic characteristics of FD in1957 [34]. These characteristics are classified into threepatterns. The most common is bone expansion with alter-nating areas of radiolucency and radiopacity, while the othertwo patterns are sclerotic and pagetoid lesions. The mostreliable technique used to evaluate FD is high-resolutionCT. Characteristic findings are the widening of diploic spaceexpansion, narrowing of vascular and neural foramina andopacity, and contrast enhancement [35]. MR images aremost useful in assessing the impact of bony lesions onadjacent soft tissue structures.

When occurring in the same patient, FD and ABC presentan especially difficult diagnostic challenge. FD can be con-fused with malignant tumors, and occasionally so can anABC [5]. FD and ABCs can grow in an insidious manner, orthey can be aggressive and increase in size rapidly. Thelatter example results in more intense symptoms and skeletal

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Table 1 Published cases of secondary aneurysmal bone cysts with fibrous dysplasia of the skull

Reference Age Gender Site Symptoms Radiology Treatment Follow-up

Branch etal. [47],1986

19 M Right parietal Expandingmass

CT: cysticexpansion of theskull

Surgical excision No recorded follow-up

Branch etal. [47],1986

9 F Left parietalandfrontotemporal

Parietaltendermass;frontotemporal firm,nontendermass

CT: large area ofbone lysis withexpansion of thediploic space inthe parietal lesion

Surgical excision No recorded follow-up

Rappaport[48],1989

25 M Left occipitalbone andclivus

Tender mass CT: pagetoidchanges in theleft orbital area;with intradiploichypodense lesion

Surgical excision No recorded follow-up

WojnoandMcCathy[14],1994

14 F Righttemporalextendinginto middlecranialfossa

Painless rightfacialswelling

CT:nonhomogeneouscystic mass

Radical resection Disease free at 2-yearfollow-up

Wojno andMcCathy[14],1994

40 M Left frontal Expandingmasscausingexophthalmos anddiplopia

CT: diffusethickening of thecalvarium, leftfrontal cyst withseptation

Resection withcranioplasty andsubsequent additionalresection of fibrousdysplasia fromethmoid sinus

No recorded follow-up

Haddad etal. [49],1998

6 M Righttemporal

Painless,expandingmass

CT:nonhomogeneouscystic mass; MRI:two componentssolid and cystic

Frontotemporalcraniotomy withcomplete excision

Disease free at 4-yearfollow-up

Saito et al.[26],1998

11 M Nasal cavity,sphenoidbone, andskull base

Nasalobstructionandheadache

CT and MRI:irregularmultilobulatedtumor

Bifrontal craniotomyand frontonasalorbitaland cribriformosteotomies weremade. Completeexcision andreconstruction usingbipedicledtemporoparietal galealflap.

Disease free at 3.5-yearfollow-up

Itshayek etal. [29],2002

19 M Left occipitalbone andclivus

Painless,slightlytendermass

CT and MRI:fibrous dysplasiaof the clivus, leftpetrous apex, andoccipital bonewith an occipitalcyst lesion

Embolization of lesionwith Ivalon particlesfollowed by surgicalexcision withcranioplasty

Disease free at 1-yearfollow-up

Pasquini etal. [7],2002

5 M Rightmaxillarysinus

Progressiveright-sidedepiphoraandrhinosinusi-tis

CT: cyst-like lesion Transnasal endoscopicapproach with rightinferior turbinectomyand complete excision

Disease free less than1 year post-surgery

Lin et al.[50],2004

18 M Left frontalbone

Severeheadache

CT: severalexpansile cysticspaces

Craniotomy No recorded follow-up

Iseri et al.[51],2005

35 F Left occipitalbone andclivus

Progressivesevereheadache

CT and MRI:fibrous dysplasiaof clivus and

Unresectable; patientdenied posteriordecompression.Alendronat

No progression at timeof publication

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deformity [7]. An aneurysmal bone cyst secondary to FDmay simulate a sarcoma [5, 11, 36, 37]. It must be noted thatmalignant transformation of fibrous dysplasia is rare, ac-counting for 0.5 % of cases with osteosarcoma being themost common secondary malignancy [38].

Treatment for FD with secondary ABC can include totalexcision or a more conservative excisional approach thatfocuses on alleviating symptoms and preventing furtherskeletal deformity. Some authors have divided the craniofa-cial skeleton into four zones based on surgical, aesthetic,and functional consideration [39, 40]. They adopted conser-vative treatment for the alveolar part of the maxilla, mandi-ble, and cranial base and the radical approach for the fronto-orbital area and the maxillozygomatic complex. In fact,Ozek et al. asserted that orbital hypertelorism, dystopia, orproptosis can only be corrected by radical excision andreconstruction [41].

The conventional therapeutic strategy also involves cu-rettage, cryotherapy, radiation therapy, and direct sclerother-apy during surgery [43]. Direct intratumoral sclerotherapy

makes it suitable for devascularization but carries the risk ofintracranial bleeding postoperatively. Preoperative arterialembolization has been performed by Minteguiaga et al. tocontrol vascularity of the lesion. ABCs can present withsubarachnoid hemorrhage in which case, repeated aspirationof the cyst may be needed [18, 42]. Three patients whoseABCs were treated with radiotherapy subsequently devel-oped a sarcoma, and consequently, this treatment has nolonger been recommended [13]. Radiation for ABC of theskull base or concomitant ABC with FD residual tumors hasnot been reported [43]. Regrowth of FD is far more likely ifa patient undergoes incomplete resection as opposed tocomplete resection. However, complete resection in thehead and neck is rarely possible due to the frequency ofmultiple bone involvement and the morbidity of radicalresection. The only other reported case of recurrence in apatient with FD and an ABC of the skull was in a patient inwhich only 95 % of the tumor was resected [44]. Serumalkaline phosphatase (ALP) is significantly high in patientswith FD. Park et al. found that ALP can be used as a simple

Table 1 (continued)

Reference Age Gender Site Symptoms Radiology Treatment Follow-up

temporal andoccipital bones

monosodium trihidratewas given.

Mattei etal. [18],2005

19 M Occipitalbone

Severeheadacheand nuchalrigidity

CT and MRI:hemorrhageand cyst

Craniotomy withpartial resectionleaving portionassociated withcerebellum and clivus

No recorded follow-up

Skladzienet al.[52],2008

16 M Rightmaxillary,orbital

Epistaxis andchronicrhinosinusi-tis

CT: largecystic lesion

En bloc excision Disease free at 9-monthfollow-up

Salmasi etal. [46],2011

16 M Nasal cavity,paranasalsinuses,andskull base

Vision loss MRI: Large skullbase tumor

Expanded endonasalendoscopic approach

Disease free at 6-monthfollow-up with residualABC not addressedat the time of surgicalintervention showingdecrease in size

Terkawi etal. [27],2011

7 F Sphenoidalandethmoidalbones

Left eyevision lossand leftnasalobstruction

CT: cysticexpansible lesion

Endonasal-cranialapproach withextradural bicoronalcraniotomy and medialbilateral orbitotomy

Recurrence 5 monthsafter removal

Our case 10 M Paranasalsinusesextendinginto theskull base

Change incognitivefunction inschool

CT and MRI: tumorof the paranasalsinuses,extending into theskull base

Three simple debridingendoscopic resectionsfollowed by oneaggressive endonasalresection, followed byone bifrontalcraniotomy forresection withextensivereconstructionof anterior cranialfossa floor

Recurrence followingfirst three endoscopicresections. Cranialextension withoutintranasal recurrencefollowing radicalendoscopic resection.No recurrencefollowing bifrontalcraniotomy at 9-monthfollow-up

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and reliable postoperative marker to determine if recurrenceis likely in a patient [45]. Endoscopic endonasal approachhas been described for anterior skull base FDs. There aretwo previous reports of fibrous dysplasia presenting with asecondary aneurysmal bone cyst in which an endoscopicresection technique was utilized. The first was in 2000, andat the time of publication in 2002, the patient remainedrecurrence-free [7]. The second reported case was in 2010,and at the patient’s 6-month follow-up, there was no evi-dence of signs of recurrence [46]. An endonasal endoscopicapproach has the advantages of no external incision, de-creased blood loss, lower morbidity, shorter hospital stay,and possible repetition in cases of recurrence when com-pared to traditional craniofacial approaches [7]. However,there can be postoperative complications such as rhinosinu-sitis and secondary mucocele from the obstruction of theosteomeatal complex. In refractory cases, open skull baseapproaches are performed, often requiring use of microvas-cular free tissue transfer. As we noted in the present case, aminimally invasive surgery does have limits and was unableto clear the extensive intracranial portion of the ABC andaggressive FD. Table 1 illustrates all of the published casesof secondary aneurysmal bone cysts with fibrous dysplasiaof the skull to this point.

Endonasal endoscopic tumor resection is an excellent ap-proach for removing select benign and malignant tumors ofthe anterior skull base. The limits of the endonasal approachare illustrated well in this case as the tumor began to extendsuperior and lateral to the orbit. This has been described byothers and typically necessitates a combined approach [53]. Acraniofacial approach utilizing the expertise of both specialtiesis often necessary to eradicate tumors with significantsuperior-lateral extension. The endoscopic endonasal ap-proach allows for removal of the intranasal portion of thetumor with no incisions on the face and minimal tissue dis-ruption of the lower facial skeleton. The bifrontal craniotomyallows for better access to the intracranial disease extendingsuperior and lateral for a safer and more complete resection.

Conclusion

Endoscopic techniques have been commonly employed forresection of anterior skull base tumors including benignlesions such as fibrous dysplasia. We report an aggressivetumor treated with multiple endoscopic resections and ulti-mately required a bifrontal craniotomy and extensive skullbase reconstruction to address the extensive intracranial dis-ease. We highlight the utility, feasibility, and advantages ofendoscopic resection while also portraying the shortcoming ofthis technique. The efficacy of endonasal endoscopic resectionfor fibrous dysplasia, particularly with a secondary aneurys-mal bone cyst, is dependant largely on the superior and lateral

extent of the disease, aggressiveness of the lesion, and com-pleteness of the initial operation. A multidisciplinary teamwith a clear understanding on the limits and benefits of theseapproaches allows for the most accurate surgical planningnecessary for safe and complete tumor removal.

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