CASE REPORT

Giant Cell Tumor of the Frontal Bone Presentingas an Orbital Mass

Peter H. Tang . Pradeep Mettu . Amanda C. Maltry . Andrew R. Harrison .

Ali Mokhtarzadeh

Received: January 14, 2017 / Published online: February 16, 2017� The Author(s) 2017. This article is published with open access at Springerlink.com

ABSTRACT

A 10-year-old male was referred for evaluation ofa right orbital mass present for 3 weeks withassociated tenderness to palpation. Magneticresonance imaging (MRI) and computedtomography imaging (CT) revealed a solid masscentered in the frontal bone with extension intothe orbit. Surgical excision and histologic anal-ysis of the lesion was consistent with a diagnosisof a Giant Cell Tumor (GCT) of the frontal bone.The patient tolerated the procedure withoutcomplication and is doing well upon follow-up.

Keywords: Frontal bone; Giant cell tumor;Orbital tumor; Osteoclastoma

INTRODUCTION

Primary orbital bone tumors compose less than2% of all orbital tumors [1]. Of these, GCT is anextremely rare osseous neoplasm that can causesignificant bone destruction and has a highpropensity for recurrence if not treated appro-priately. While GCTs are commonly believed tobe benign, there are rare case reports of possiblemalignant transformation [2–4]. Due to its rar-ity, much uncertainty and debate exist as to theproper management of these and other primaryorbital bone tumors. We describe our experi-ence treating a 10-year-old male who presentedwith a rare GCT of the frontal bone that wastreated with surgical excision. This report is infull compliance with the Declaration of Helsinkiand the current Health Insurance Portabilityand Accountability Act regulations. Informedconsent was obtained from the patient for beingincluded in the study.

CASE REPORT

A 10-year-old male was referred for evaluationof a 3-week history of an enlarging right orbitalmass (Fig. 1). Past ocular history was notable fora long-standing exotropia (XT). The patientendorsed mild pain to palpation of the lesionbut denied vision changes. There was no recentor remote history of periorbital trauma, and hewas otherwise healthy. His visual acuity was

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P. H. Tang � P. Mettu � A. C. Maltry �A. R. Harrison � A. Mokhtarzadeh (&)Department of Ophthalmology and VisualNeurosciences, University of Minnesota,Minneapolis, MN, USAe-mail: mokh0003@umn.edu

P. H. Tange-mail: tangx520@umn.edu

Ophthalmol Ther (2017) 6:215–220

DOI 10.1007/s40123-017-0081-y

measured to be 20/25 without correction ineach eye. Intraocular pressures were 19 and20 mmHg in the right (OD) and left (OS) eyes,respectively. Extraocular movements were full,and alternate cover testing revealed a 15-prismdiopter XT. Hertel exophthalmometry wassymmetric without relative proptosis. Heexhibited 2–3 mm of hypoglobus. Cranialnerves V1, V2, V3, and VII were found to benormal. The mass was firm and fixed to thesuperolateral orbital rim, and was tender topalpation. The remainder of the anterior andposterior segment examinations of both eyeswas unremarkable.

The patient underwent magnetic resonanceimaging (MRI) with and without contrast as well

as computed tomography (CT) without contrastfor further evaluation and surgical planning. OnMRI, therewas a 2.7 cmmass centered in theboneof the superolateral right orbit with associatedbone destruction and extension into the supero-lateral right extraconal orbit and abnormal signalin the right frontal bone and sphenotemporalbuttress (Fig. 2). CT revealed bone destructionwith only thin bone remaining between thetumor bed and the intracranial space. The patientunderwent an uncomplicated right orbitotomywith excision of the mass. Given his lack of aneyelid crease, a sub-brow incision was used toaccess the mass, which was found to be firmlyadherent to the bone. The surrounding perios-teum was incised around the mass

Fig. 1 External photographs show right brow lesion from before (top), 1-month (middle) and 4-months (bottom) aftersurgical excision

216 Ophthalmol Ther (2017) 6:215–220

circumferentially with cutting cautery, and themass was then dissected free using a Freer perios-teal elevator. There was a residual defect in thesuperolateral orbital rim. The incision was closedin layers.Histopathology revealed the lesion to becomposed of osteoclast-type giant cells evenlydistributed among mononuclear cells with ill-de-fined cell borders and associated fragments ofreactive woven bone rimmed by osteoblasts(Fig. 3). Both the mononuclear and giant cellsstained positively for CD68 on immunohisto-chemistry. Only rare scattered cells stained withCD1a. Ki-67 demonstrated a high proliferationindex with 70% of the mononuclear cells beingpositive. Further analysis of the lesion revealed aloss of onenormal copyof chromosomes 5 and 12

with additional material of unknown originadded to the long arm of chromosome 16, andgain of a chromosome 21 and of a marker chro-mosome of unknown origin. The chromosomalfindings indicate a neoplastic process which, inthe setting of the imaging and histopathologyfeatures, was consistent with giant cell tumor ofthe bone. Given that the mass was grossly resec-ted, and further curettage would necessitate acraniotomywith reconstruction, observation waselected.

At the 1-month follow-up, MRI with andwithout contrast revealed residual enhancementthat reduced dramatically by his 4-month fol-low-up scan. Examination at 4 months revealed apalpable defect in the superolateral bony rim;

Fig. 2 Post-contrast T1 sequence with fat suppression MR images before (top), 1 month (middle) and 4 months (bottom)after surgical resection

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otherwise, he is asymptomatic and continues tohave his longstandingwell-controlledXT (Figs. 1,2). He is healing appropriately and denies pain orother symptoms of concern.

DISCUSSION

As the incidence of GCT of the frontal bone isextremely rare, the majority of studies in the

literature have been case reports [5–7]. TheWorld Health Organization recognizes threedistinct types of GCTs arising from the bone,tendon sheath, or soft tissue [8]. GCT mostfrequently occurs in the epiphyses of long bonesand rarely occur in the orbit [9]. Radiographicfeatures of GCT include an osteolytic lesion thatis radiolucent and results in bony erosions withsharp margins on CT scan. MRI shows a

Fig. 3 Intraoperative photographs (a) highlight appear-ance of lesion (top) and the extent of surrounding bonedestruction after excision (middle), and gross pathology ofexcised lesion is shown (bottom). Histologic analysis (b) atlow magnification (top) reveals areas of reactive woven

bone (star), and high magnification (bottom) showsmultinucleated giant cells (arrows) scattered amongmononuclear cells (H&E staining; scale bars are 200 lmtop and 50 lm for bottom)

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well-circumscribed lesion that exhibits isoin-tensity on T1-weighted images and hypointen-sity on T2-weighted images. Typically, thelesion enhances with contrast on both imagingmodalities [10, 11]. Histologically, GCT arisingfrom bone are composed of stromal mononu-clear cells and giant cells whose histogenesis iscontroversial. The mononuclear cell representsthe true neoplastic component, while themultinucleated giant cells have an osteo-clast-like phenotype and express histocytic lin-eage markers. The histologic differentialdiagnosis of GCT include other processes inwhich multinucleated giant cells can be found,such as giant cell granuloma, ‘‘brown tumor’’ ofhyperparathyroidism, non-ossifying fibroma,osteoblastoma, aneurysmal bone cyst, andLangerhans cell histiocytosis [12, 13]. WhileGCT is generally considered a benign tumor, ithas the potential for malignant transformation[3, 4, 11]; therefore, careful observation isessential. The lesion often grows slowly and cancause significant bone destruction. The currenttreatment goal is for total surgical resection ofthe tumor without adjuvant radiation therapy,which has been shown to have the lowestrecurrence rate. Indeed, Kamoshima et al.described a 2-year-old female patient withrecurrent GCT of the frontal bone that under-went two partial surgical removals of the tumorwith recurrence before total resection of thelesion, surrounding bone, and frontal base duramater was curative [14]. The reported incidencein the literature of non-recurrence after totalresection of a frontal bone lesion has been up to30 months [15]. If total resection cannot beachieved, the combination of subtotal resectionand radiation therapy shows a similar lowrecurrence. Other therapeutic strategies maylead to increased recurrence rates [16]. Contin-ued long-term follow-up will be important inthe ongoing management of our patient.

CONCLUSION

In summary, we present a rare case of arapidly enlarging superotemporal orbital massin a ten-year-old boy, which proved to be GCTof the frontal bone. While total surgical

resection could not be achieved without a riskof added morbidity, gross resection, followedby close surveillance has resulted in norecurrence with 4 months of follow-up.Although rare, the ophthalmologist should beaware that GCT might present with orbitalinvolvement.

ACKNOWLEDGEMENTS

No funding or sponsorship was received for thisstudy or publication of this article. All namedauthors meet the International Committee ofMedical Journal Editors (ICMJE) criteria forauthorship for this manuscript, take responsi-bility for the integrity of the work as a whole,and have given final approval for the version tobe published.

Disclosures. Peter H. Tang, Pradeep Mettu,Amanda C. Maltry, Andrew R. Harrison and AliMokhtarzadeh have nothing to disclose.

Compliance with Ethics Guidelines. Thisreport is in full compliance with the Declarationof Helsinki and current Health InsurancePortability and Accountability Act regulations.Informed consent was obtained from thepatient for being included in the study.

Open Access. This article is distributedunder the terms of the Creative CommonsAttribution-NonCommercial 4.0 InternationalLicense (http://creativecommons.org/licenses/by-nc/4.0/), which permits any noncommer-cial use, distribution, and reproduction in anymedium, provided you give appropriate creditto the original author(s) and the source, providea link to the Creative Commons license, andindicate if changes were made.

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