Giant Cell (Reparative) Granuloma of the Orbit

Giant Cell (Reparative)

Granuloma of the Orbit PHILLIP C. HOOPES, MD, RICHARD L. ANDERSON, MD, FREDERICK C. BLODI, MD

Abstract: Clinically and histologically, there exists considerable controversy as to what constitutes a giant cell tumor. There is an increasing awareness that the term giant cell tumor should not be used indiscriminately in describing lesions of the mandible, maxilla, and facial bones which contain multinucleated giant cells. 1 ,2 This paper reports a rare case of giant cell reparative granuloma of the orbit. The differentiation between giant cell reparative granuloma and true giant cell tumor is discussed and a description of the visual symptomatology associated with these lesions of the sphenoid bone is presented. These tumors must be removed completely to prevent recurrence. [Key words: giant cell granuloma, orbit.] Ophthalmology 88:1361-1366, 1981

CASE REPORT A 12-year-old white girl was referred to the University of Iowa Hospital on December 26, 1974 for evaluation of a mass in the temporal region of the right orbit (Fig 1). On September 30, 1974, her local doctor had attempted to remove what he thought to be a subcutaneous nodule at the right brow region but found bony involvement. She was in good health with no previous medical problems. Visual acuity was 20/40 + 2 right eye and 20/20 - 3 left eye. The right eye was 2 mm prop to tic (17 mm right eye vs 15 mm left eye by Hertel). Extraocular muscle movements were full. Funduscopic examination of the right eye revealed choroidal folds with tortuous vessels in the superior temporal quadrant. External examination revealed a 4 cm x 2.5 cm hard non-mobile mass on the temporal aspect of the right orbit. Echography revealed a solid, irregularly bordered, lowreflective tumor in the upper temporal quadrant of the orbit. Polytomograms demonstrated bony destruction involving the superior, lateral, and posterior portion of the right orbit associated with some bony density reaction of the sphenoid

From the Department of Ophthalmology, University of Iowa Hospitals and Clinics, Iowa City. Reprint requests to Richard L. Anderson, MD, Department of Ophthalmology, University of Iowa Hospitals and Clinics, Iowa City, IA 52242. 0161-6420/8111200/13611$00.80

© American Academy of Ophthalmology

wing on the right side. EMI scan showed a bony defect of the lateral orbit; the right globe was compressed and pushed forward by a mass posterolateral to it. The patient underwent a lateral orbitotomy on January 2, 1975 with partial removal of the large orbital mass. Several pathologists reviewed the histology with diagnosis of giant cell tumor (osteoclastoma) versus giant cell reparative granuloma being made (Fig 2). The postoperative course was uneventful; however, the lesion recurred rapidly. On June 20, 1975, the patient underwent a second lateral orbitotomy with attempted removal of all identifiable tumor mass. The tumor mass was again examined by a number of pathologists including the Air Force Institute of Pathology with a final diagnosis of giant cell reparative granuloma being made. The patient had depression of the lateral orbital region and a small LXT and LHT following this procedure. However, she did well with no clinicalor echographic changes on yearly examinations until May 1979, at which time she presented with 6 mm of right proptosis and marked deformity of the lateral and superior orbital regions (Fig 3). Visual acuity was 6115 right eye and 6/6 left eye. Radiographs, EMI scan, and echography showed osseous distortion originating in and involving the right lateral orbital wall as well as the entire superior orbital wall with a large corresponding soft tissue mass (Figs 4-6). The mass extended into the orbit and displaced the globe anteriorly. The area of involvement extended through the superior orbital roof into the frontal lobe region. On June 13, 1979, the patient underwent a combined right frontal craniotomy and a superior orbitotomy. The large area of involvement had replaced the entire roof and lateral orbital walls and was ad-

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herent to dura. The right side of the frontal bone was also extensively involved including the superior orbital rim . The tumor was removed with a surrounding border of normal tissues . Agraft of galea was used to patch the dura . The large orbital extension of the tumor was found to have displaced rather than invade orbital tissues other than periorbita and was completely removed. A large acrylic plate (cranioplastic) was formed to rebuild the frontal region as well as the roof and lateral walls of the orbit. The patient did well postoperatively . Visual acuity was 6/6 right eye and 6/6 left eye with no evidence of recurrence with an adequate functional and cosmetic result one year after surgery (Fig 7).

COMMENT The entity of giant cell reparative granuloma was used initially by Jafk' to describe lesions found in the jaw bone and thought to represent a non-neoplastic reaction to hemorrhage. Prior to that time, the lesion had been casually referred to as a "giant cell tumor" of bone. There have been only two previously reported cases in the English literature of giant cell reparative granuloma of the sphenoid bone, 1 and one reported case involving the ethmoid and maxillary sinus. 4 However, 20 cases of giant cell tumor of the sphenoid bone have been reported. 5 - 22 Although some investigators

Fig 2. Histologic section demonstrating multinucleated giant celis and spindle-shaped fibroblasts .

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Fig 4. Radiograph showing bony erosion of superior orbit with extension superiorly.

Fig 3. Recurrence of tumor with marked inferior displacement of right eye.

claim that the giant cell reparative granuloma is not a separate entity but a variant of the giant cell tumor peculiar to the jaw, 23 the distinction between these two lesions can be very important in the management, treatment, and prognosis of the tumor.1.24 Grossly, the two tumors appear similar in that they may destroy bone and usually consist of red or reddish-blue, friable vascular tissue. 1 However, based on numerous reports, 1.~.2S-30 distinct histologic and clinical differences for diagnosis and differentiation of giant cell reparative granuloma from giant cell tumor of bone have been established. According to the criteria of HirschI and Katz,~1 the giant cells of the reparative granuloma are located in groups around hemorrhagic foci. The stroma shows, along with oval cells, an equally large number of spindle-shaped fibrohlastic cells with zones of abundant collagen formation and relatively few giant cells. There is evidence of marked hemorrhage. The giant cells are generally smaller, frequently irregular and elongated with fewer nuclei than giant cells seen in giant cell tumors of bone. Foci of osteoid and new bone formation are frequently present. In giant cell tumor of bone, the giant cells are uniformly dispersed, dominate the entire field, and are generally larger and more rounded with a greater number of nuclei. The stroma is composed of pre-

Fig 5. Caldwell view showing marked destruction of the roof of the right orbit.

Fig 6. CAT scan demonstrating hony erosion and proptosis of right eye.

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Fig 7. Patient one year postoperatively.

dominately plump, round cells. Fresh hemorrhage is slight to moderate. The tumor usually does not produce new bone formation. The histologic differentiation between giant cell reparative granuloma and giant cell tumor is not always unequivocal and should not be made without considering the clinical data. The differentiation based on clinical information is made by considering the sex, age, location of the tumor, and clinical course of the patient. Giant cell reparative granuloma is predominately a disease involving females. In a study of giant cell reparative granuloma of the jaw, 26 of 38 patients (68%) were females, a ratio of 2.1: l. 32 In the same study, the vast majority of patients were between the ages of 10 and 25 years, consistent with data published by other investigators. 3.26 ,31,33-36 In contrast, giant cell tumor of bone is seen most frequently in the third and fourth decades and is unusual in a patient less than 20 years of age. 3,31,32,37-41 Giant cell tumor of bone most commonly affects the epiphysis of the long bones. In a study of 3,987 primary bone tumors at the Mayo Clinic,42 155 (4%) were found to be of giant cell origin. Of these, 75% were found in long bones with remaining 25% occurring in the sacrum, carpals, patella, vertebra, and skull. The majority of giant cell tumors of the skull occur in the mandible and maxilla. The location of the majority of giant cell 1364

reparative granuloma is in the mandible and to a lesser extent the maxilla;3,26,32,43 it was originally thought to be peculiar to the jaw bone. 3 However, reports have demonstrated that skull bones other than the jaw can be involved. A review of the histology, history, and clinical course of five cases that were initially diagnosed as giant cell tumors of the maxilla, sphenoid, and ethmoid bones, revealed that four of the lesions were actually compatible with giant cell reparative granuloma. 1 Two of the four cases involved the sphenoid bone. HirschI and Katz 31 reported the first case of giant cell reparative granuloma of the temporal bone and then reviewed 23 reported cases of giant cell tumor of the temporal bone. These investigators concluded, on the basis of their criteria, that 18 of the 23 cases actually represented giant cell reparative granulomas. The major factors supporting their diagnoses in the 18 cases were the characteristic histologic appearance and the benign clinical outcome consistent with a non-neoplastic, self-limiting lesion. We have reviewed the 20 case reports of" giant cell tumor" of the sphenoid bone. 5- 22 When the criteria of HirschI and Katz 31 were applied to these 20 cases, 14 appeared to actually represent giant cell reparative granulomas. The occurrence of giant cell reparative granuloma in the orbit is extremely uncommon. Sood et al 4 reported what was believed to be the first case of reparative granuloma of the orbit that involved the ethmoid and maxillary sinus. One of the cases of sphenoid giant cell reparative granuloma reported by Friedberg et aP extended into the orbit. Our case demonstrated extensive orbital involvement of zygoma, sphenoid, and frontal bones. As already pointed out, the giant cell reparative granuloma is a benign lesion. However, the granuloma may demonstrate wide local destructive spread and may recur after incomplete surgical removal 10 to 15% of the time. 31 ,32,44 The lesion is self-limited and can subside spontaneously, whereas no spontaneous cure of a giant cell tumor has been reported. 31 .43 The giant cell tumor has a recurrence rate as high as 45% after surgical and radiation therapy and may give rise to metastases. 41 A history of trauma is more frequently elicited in reparative granuloma than in giant cell tumors. 26,30,31,37.45 However, in an analysis of 38 cases of giant cell granuloma ofthejaws, Waldron and Shafer32 found a history of trauma in so few of the cases that they doubted the etiologic significance of this factor. They believed that the lesion does not represent a "reparative" process. It was illogical to them that a destructive, locally invasive, and potentially enlarging lesion could represent a "reparative" reaction. We agree with their proposal to delete the word reparative and refer to this lesion as a giant cell granuloma until its true nature is more clearly established. We have used the word reparative in this paper only to conform to present terminology. Other lesions to be considered in the differential diagnosis of giant cell reparative granuloma are the

HOOPES, et al • ORBITAL GIANT CELL GRANULOMA

"brown tumor" of hyperparathyroidism and aneurysmal bone cyst. In hyperparathyroidism, there is a lytic destruction of bone with local hemorrhage. The pathologic process within the bone consists of a fibrocellular proliferation in the marrow spaces, numerous osteoclasts, and imperfect attempts at regeneration of bone. 33 The histologic picture is almost identical to reparative granuloma and differentiation is achieved only by determination of calcium and phosphorus levels. The aneurysmal bone cyst has a typical radiograph appearance of a cystic "blow-out" of bone and is characterized histologically by large vascular spaces. 46 - 47 The treatment of choice for giant cell reparative granuloma is surgical remova1 2,3,26,3o,31 with radiation therapy being reserved for cases considered to be inoperable. 2,31 However, if radiation is to be used, it should be in small dosage as reports of sarcomatous and carcinomatous transformation following radiotherapy have been reported. 2,26 Our case which recurred three times suggests that initial aggressive approach to these tumors should be made to avoid recurrences and extensive procedures such as this patient required. Of interest to ophthalmologists are the visual symptoms presented by patients with giant cell tumors of the sphenoid bone. In reviewing the 20 patients reported with giant cell lesions of the sphenoid bone,5-22 diplopia was found to be the most common eye symptom; it was present in 15 of the 20 cases. Multiple cranial nerve palsies involving the third, fourth, fifth, and sixth nerves in various combinations are frequent. Abducens nerve· palsy was the most common, occurring in 50% of cases. Decreased vision occurred in 11 patients including three cases of complete blindness. Those cases with decreased visual acuity showed varying degrees of ischemic optic neuropathy. In only one instance was there a return of visual acuity, 22 Proptosis was present in only four cases, In the two reported cases of giant cell reparative granuloma of the sphenoid,1 visual symptoms were also reported. One case disclosed a paresis of the right lateral rectus muscle with diplopia on right gaze. There was slight proptosis. The other case presented with findings of paresis of the right medial rectus muscle and proptosis. In the case of giant cell reparative granuloma of the orbit involving the ethmoid and maxillary sinus, proptosis with lateral displacement of the eye occurred. 4 Our patient presented with a palpable mass in the temporal region of the right orbit and proptosis. Visual acuity was mildly decreased and funduscopic examination revealed the presence of choroidal folds. We have presented what is believed to be the fourth reported case of giant cell reparative granuloma of the orbit. However, one should be mindful that the majority of previously reported cases of giant cell tumors involving the sphenoid bone were most likely reparative granulomas. Although it is an infrequent cause of orbital disease, the ophthalmologist should be aware of

and entertain the entity of giant cell granuloma in the differential diagnosis of orbital tumors. Our case strongly supports the concept that these tumors should be removed completely to prevent recurrence.

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