monostotic fibrous dysplasia orbit: unusual lacrimal fossa mass · monostoticfibrous...

Bnrtish,Journal ofOphthalmology 1993; 77: 54-56

CASE REPORTS

Monostotic fibrous dysplasia of the orbit: an unusuallacrimal fossa mass

Peter McCluskey, Richard Wingate, Ross Benger, Stanley McCarthy

AbstractA case of monostotic fibrous dysplasia involv-ing the orbit, presenting with a short history ofpainful, progressive proptosis and a lacrimalfossa mass is reported. There were no clinicalor radiological clues to the diagnosis which wasmade on frozen section at the time of opensurgical biopsy. The features of orbital fibrousdysplasia are reviewed and a possible mecha-nism for this rare clinical presentation offibrous dysplasia is discussed.(BrJ Ophthalmol 1993; 77: 54-56)

Department ofOphthalmology, RoyalPrince Alfred Hospital,and Laboratory of OcularImmunology, Universityof NSW, Sydney,AustraliaP McCluskey

Department ofOphthalmology, RoyalPrince Alfred Hospital,Sydney, AustraliaR WingateR Benger

Department ofAnatomical Pathology,Royal Prince AlfredHospital, Sydney,AustraliaS McCarthyCorrespondence to:Dr P J McCluskey,Department ofOphthalmology, Royal PrinceAlfred Hospital,Camperdown, NSW 2050,Australia.Accepted for publication28 July 1992

Figure I Frontal view ofpatient. Note the inferiorglobe displacement.

Orbital involvement by fibrous dysplasia is wellrecognised and may present clinically in severalways, including proptosis. This case reportdetails a rare presentation consisting of a shorthistory ofpainful proptosis associated with globedisplacement and an apparent lacrimal fossamass.

Case reportA 41-year-old Egyptian female presented with a 4month history of progressive diplopia, reductionofvision in the left eye, and increasing pain in theupper temporal quadrant of the left orbit where afirm tender mass was palpable. There was nohistory of trauma. The patient was otherwise ingood health with no history ofsignificant medicalor surgical illness. She was taking no medication.A careful review of systems was non contribu-tory. Her most recent pregnancy was 5 years ago.On examination the right eye and adnexae

were normal. The left visual acuity was reducedto 6/24. There was a fullness in the uppertemporal quadrant of the orbit and a firm,slightly tender mass was palpable in the lacrimal

Figure 2 CoronalCT scan showing large mass in theposition ofthe lacrimal gland. Note the calcification in themass.

fossa region. The globe was proptosed 7 mmaxially and 2 mm inferiorly (Fig 1). There was asignificant reduction of elevation and abductionof the left eye. Examination of the globe wasnormal. General physical examination wasnormal; in particular, there was no clinicalevidence of thyroid disease, nor any abnormalskin pigmentation or bony deformity.Coronal and axial computed tomographic (CT)

scanning of the orbits was performed andrevealed an extraconal mass lesion whichinvolved the superior and lateral aspects of theleft orbit and contained some calcification (Fig2). The lesion was smoothly irregular in outlineand depressed the globe. There was evidence ofearly cortical bone erosion by the lesion in the leftupper lateral orbital margin and the frontalprocess of the zygomatic bone. The lacrimalgland could not be seen separately from the mass.Full blood count, ESR, biochemical profile, liverfunction tests, and thyroid function tests werenormal except for the serum alkaline phospha-tase which was elevated at 284 units/I (normal<160 units/l).The history and clinical findings were consid-

ered consistent with a diagnosis of lacrimal glandcarcinoma and therefore a trans-septal orbitalbiopsy was performed. At operation a bulky masswas found in the position of the lacrimal gland.

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Monostotic fibrous dysplasia ofthe orbit: an unusual lacrimalfossa mass

Its surface was thick and pale but on cutting itproved to be gritty, friable, and haemorrhagic.Histopathological examination revealed a fibro-osseous lesion containing metaplastic bone withirregular birefringence consistent with fibrousdysplasia. About one third of the tissue removedwas fibrous with foci ofmultinucleated giant cells

Figure 4 The areas offibrous dysplasia with metaplastic bone trabeculae merge with non-bony areas showing acute haemorrhage, fibrous tissue, and multinucleated giant cells. At theedge of the lesion the cortical bone was generally very thin or absent (magnification x 8).

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and prominent recent haemorrhage, featuresfound in giant cell reparative granuloma.

Five days later a modified Wright's lateralorbitotomy was performed. At surgery the masswas found to be surrounded by a capsule andsuperficial to the periorbita. The mass appearedto arise from bone and it was not possible todissect the mass free from bone (Fig 3). Anosteotome was therefore used to remove the massand curettage of the orbital bones was thenperformed to remove any residual tumour.Histopathological examination confirmed thediagnosis of fibrous dysplasia with areas of giantcell (reparative) granuloma (Figs 4 and 5). Thelatter included acute haemorrhage, fibrocytes,and multinucleated giant cells.

Following surgery, the patient's visual acuityreturned to normal and the other physical find-ings resolved. When last reviewed some 6months postoperatively, orbital and ocular exaim-ination were normal apart from the surgicalscars.

CommentFibrous dysplasia is an uncommon benign bonydisease of unknown aetiology which typicallypresents in children or young adults. First recog-nised in 1937 by Albright as a syndrome charac-terised by 'osteitis fibrosa disseminata', areas ofpigmentation, and endocrine dysfunction, itlater became clear that the bony lesions coulddevelop in the absence of the other features ofAlbright's syndrome and that the disease couldbe monostotic as well as polyostotic in distribu-tion.' The disease is not heritable and is seen withequal frequency in both sexes.Monostotic involvement usually involves the

craniofacial bones or ribs with the sphenoid andfrontal bones being the most commonly affected.Polyostotic lesions may involve any bone and bewidespread but typically the lower limbs arepredominantly affected. Up to 50% of girls withthe polyostotic form have associated macules ofabnormal skin hyperpigmentation (Coast ofMaine lesions) and precocious puberty. Abnor-mal skin pigmentation may also be seen in boys.'

Orbital involvement is common as the sphen-oid and frontal bones are sites of predilection forthe disease. The typical patient presents in thefirst three decades of life with slowly progressivepainless proptosis variably associated with non-axial globe displacement, dystopia, and facialdisfigurement. It is frequently progressive but isthought to be self limiting with a variable endpoint in the second or third decades of life.2

In contrast, our patient was aged 41 years andpresented with a short history of rapidly increas-ing proptosis, a mass lesion, globe displacement,and pain typical of an infiltrative process. Theradiological findings were consistent with a masslesion in the lacrimal fossa associated with bonedestruction. The patient had no clinical evidenceof bone disease, there was no history of pre-cocious puberty, and there were no skin lesions.Indeed until the trans-septal biopsy was per-formed the working diagnosis was lacrimal glandcarcinoma. Although it is well recognised thatfibrous dysplasia produces proptosis, it rarelypresents with the history seen in our patient. A

Figure 3 Operative viewshowing the tumour massand its origin from thefrontal bone.

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McCluskey, Wingate, Benger, McCarthy

review of the literature was able to identify onlyone similar reported case where proptosisevolved over 1 month; however this was associ-ated with dysaesthesia but no pain.3The histopathology of the mass removed at

orbitotomy revealed the features of both fibrousdysplasia and giant cell reparative granuloma.Giant cell reparative granuloma consists histo-logically of haemorrhagic masses with a spindlecell stroma containing osteoblastic giant cells andhaemosiderin.2 Giant cell reparative granuloma isa benign process, which occurs in associationwith trauma and intraosseous haemorrhage; it ispart of the spectrum of reactive bone conditionscommonly seen in a number of bone diseasesincluding fibrous dysplasia.The haemorrhage and reparative granuloma

may well explain the most unusual clinical pre-sentation of this patient. It is likely that thispatient had a small localised area of monostoticfibrous dysplasia in the zygomatic process of thefrontal bone which was asymptomatic. Bleedingthen occurred in an area of the fibrous dysplasiaeither spontaneously or following trauma. Theresultant swelling and reparative granuloma wasthen responsible for the rapidly evolving painfulproptosis.

1 Wintrobe MM, Thorn GW, Adams RD, Braunwald E, Issel-bacher KJ, Petersdorf RG. Harrison's principles of internalmedicine, 7th Ed. New York: Blakiston, 1974: 1981-3.

2 Rootman J. Diseases ofthe orbit. Philadelphia: Lippincott, 1988:chapter 12.

3 Retter RH. Unilateral proptosis due to monostotic fibrousdysplasia. Ann Ophthalmol 1976; 8: 45-9.

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