Ординатура / Офтальмология / Английские материалы / Tumors of the Eye and Ocular Adnexa_Char_2001

350 TUMORS OF THE EYE AND OCULAR ADNEXA

17–32 demonstrates such an intraconal lesion. On MRI, this was hypointense to brain on T1 and hyperintense on T2 (not shown). The management of these lesions is surgical removal.

NEUROFIBROMA

Isolated neurofibromas often occur in the intraconal space. In contrast to orbital cavernous hemangiomas, which are usually smaller and do not involve the orbital apex, neurofibromas (like neurilemmomas) commonly expand through the superior orbital fissure. Krohel and colleagues reported on 9 patients with localized orbital neurofibromas; 1 case was associated with neurofibromatosis.60 In 5 patients, there were multiple tumors, and 4 had a pedicle extending from the superior orbital fissure. In 3 of 5 cases, neither CT nor ultrasonography demonstrated the multiple tumors that were present.

Usually, neurofibromas involve the superior orbit. The patient in Figure 17–33A demonstrates such a case. The eye has been displaced downward. On both axial reconstructed CT (Figures 17–33B to D) and direct coronal MRI (Figures 17–33E and F), a large mass going into the superior orbital fissure was found. We were able to resect this mass, using a lateral orbitotomy, as discussed in the chapter on orbital management.

HEMANGIOPERICYTOMA

Hemangiopericytoma can simulate the MRI, CT or ultrasonographic pattern of an intraconal cavernous hemangioma. Like cavernous hemangiomas, the T1 pattern on MRI is often hypointense to CNS tissue with a variable T2 pattern.61 Both benign and malignant hemangiopericytomas can occur, and unfortunately, the histologic findings are not a good predictor of clinical behavior.62 In a study by Croxatto and Font, 16 appeared benign, 5 borderline, and 9 malignant.62 Four of 27 cases with long-term follow-up died of widespread metastases. Others have noted a similar disease course.61 Mortality did not correlate with histologic type; 5-year actuarial survival was 89 percent.62 Henderson and Farrow described 13 cases, which represented 1.7 percent of the orbital tumors

Most patients were middle-aged; no

hemangiopericytomas occurred in children. There was progressive proptosis. In almost all the patients, the tumor involved the superior orbit but could clinically simulate cavernous hemangioma almost perfectly.62,63 If the tumors were not excised, recurrences were common.63,65 The role of radiation in the management of hemangiopericytoma is uncertain. Positive results have been observed in some other body sites.61,66,67 In our experience, with postoperative irradiation in a few cases, we have obtained tumor control, when the lesion has not been completely excised.

INFILTRATIVE INTRACONAL LESIONS

Pseudotumors occasionally involve the intraconal space but are not discrete. Twenty percent of orbital metastases involve the intraconal space. Usually, the intraconal orbital metastases either are diffuse, simulating pseudotumor or involve the contiguous muscle (Figure 17–34). The diffuse quality of both the pseudotumor and metastases in this area makes their delineation from cavernous hemangioma, hemangiopericytoma, or isolated neurofibromas relatively straightforward. Rarely, metastic tumors can be quite focal, however, as shown in Figure 17–35. Lymphoid tumors rarely occur as discrete intraconal masses (see Figure 26–11). Metastases and lymphoid lesions are discussed in separate chapters. Intraocular tumors can also spread contiguously to involve the intraconal area, but this is rare in the United States. Figure 17–36 shows a direct parasagittal MR image of a patient with an intraocular-orbital metastatic carcinoid tumor. Similarly, both melanoma and retinoblastoma can either contiguously involve the ipsilateral intraconal space or, occasionally, the contralateral orbit (see Figure 17–12). Adult optic nerve neoplasms are described separately. Childhood orbital neoplasms are described in the chapter on pediatric orbital tumors.

A number of rare intraconal neoplasms can occur. A liposarcoma is shown clinically in Figure 17–37A and the CT with reconstruction and MRI are shown in Figures 17–37B to D.28,30,68 Similarly, we have seen one case of a chondrosarcoma which involved the intraconal space (Figure 17–38).

As discussed under orbital surgery, the optimal approach for tumors in the anterior or midintraconal space is via a lateral orbitotomy. Precise visualiza-

Figure 17–36. Carcinoid metastatic to the eye and intraconal space shown on direct parasagittal T1-weighted MRI scan.

E

Figure 17–37. A, Clinical photograph of a patient with liposarcoma. B, Orbital CT with computer re-formation, showing a liposarcoma with an apparent cystic component. C and D, Parasagittal and coronal reformation of the orbital CT scan. E, Axial T1-weighted MRI scan.

Figure 17–38. Axial CT scan of biopsy-proven intraconal chondrosarcoma.

tion during surgery is vital to avoid damage to contiguous vessels and nerves, and in most of these cases, surgery is carried out using hypotensive anesthesia and a microscope. Rarely, conjunctival cysts can produce an intraconal tumor, and we have seen two of those.69,70 These lesions can occur without a history of trauma, although in the author’s experience, it is more common after a sharp instrument has gone through the eyelid.

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18.Bednar MM, Trainer TD, Aitken PA, et al. Orbital paraganglioma: case report and review of the literature. Br J Ophthalmol 1992;76:83–5.

19.Moss HM. Expanding lesions of the orbit. A clinical study of 230 cases. Am J Ophthalmol 1962;54: 761–70.

20.Kopelow SM, Foos RY, Straatsma BR, et al. Cavernous hemangioma of the orbit. Int Ophthalmol Clin 1971; 11:113–25.

21.Unsold R, Hoyt WF, Newton TH. Criteria of orbital hemangiomas and their importance in differential diagnosis of intraconal tumors. Klin Monatsbl Augenheilkd 1979;175:773–85.

22.Brown GC, Shields JA. Amaurosis fugax secondary to presumed cavernous hemangioma of the orbit. Ann Ophthalmol 1981;13:1205–9.

23.Ossoinig KC, Keenan TP, Bigar F. Cavernous hemangioma of the orbit: Ultrasonography in Ophthalmology. Bibl Ophthal 1975;83:236–44.

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25.Cappaert WE, Kiprov RV, Frank KE. Sector B-scan ultrasonographic hemangioma-like pattern. Arch Ophthalmol 1983;1983:101.

26.Char DH, Stone RD, Irvine AR, Unsold R. Orbital melanoma. Ann Ophthalmol 1981;13:911–3.

27.Shen WC, Yang DY, Ho WL, Lee SK. Neurilemmoma of the oculomotor nerve presenting as an orbital

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28.Nasr AM, Ossoinig KC, Kersten RF, Blodi FC. Standardized echographic-histopathologic correlations in liposarcoma. Am J Ophthalmol 1985;99:193–200.

29.Sklar EL, Quencer RM, Byrne SF, Sklar VEF. Correlative study of the computed tomographic, ultrasonographic, and pathological characteristics of cavernous versus capillary hemangiomas of the orbit. J Clin Neuroophthalmol 1986;6:14–21.

30.Jakobiec FA, Rini F, Char DH, et al. Primary liposarcoma of the orbit: problems in the diagnosis and management of five cases. Ophthalmology 1989; 96:180–91.

31.Cohen JA, Char DH, Norman D. Bilateral orbital varices associated with habitual bending. Arch Ophthalmol 1995;113:1360–2.

32.Bullock JD, Goldberg SH, Connelly PJ. Orbital varix thrombosis. Ophthalmology 1990;97:251–6.

33.Fourman S. Acute closed-angle glaucoma after arteriovenous fistulas. Am J Ophthalmol 1989;107:156–9.

34.Leib WE, Merton DA, Shields JA, et al. Colour doppler imaging in the demonstration of an orbital varix. Br J Ophthalmol 1990;74:305–8.

35.Hanneken AM, Miller NR, Debrun GM, Nauta HJW. Treatment of carotid-cavernous sinus fistulas using a detachable balloon catheter through the superior ophthalmic vein. Arch Ophthalmol 1989;107:87–92.

36.Christi DB, Kwon YH, Choi I, et al. Sequential embolization and excision of an orbital arteriovenous malformation. Arch Ophthalmol 1994;112:1377–9.

37.Lacey B, Rootman J, Marotta TR. Distensible venous malformations of the orbit: clinical and hemodynamic features and a new technique of management. Ophthalmology 1999;106:1197–209.

38.Mojon D, Odel JG, Rios R, Hirano M. Presumed orbital hemangioma associated with blue rubber bleb nevus syndrome. Arch Ophthalmol 1996;114:618–9.

39.Wolter JR. No need for cutting of the lateral rectus muscle: in the transconjunctival approach to the muscle cone of the orbit. J Pediatr Ophthalmol Strabismus 1980;17:40–3.

40.Hassler W, Schaller C, Farghaly F, Rohde V. Transconjunctival approach to a large cavernoma of the orbit. Neurosurgery 1994;34:859–62.

41.Henderson JW, Farro GM, Garrity JA. Clinical course of an incompletely removed cavernous hemangioma of the orbit. Ophthalmology 1990;97:625–8.

42.Carta F, Siccardi D, Cossu M, et al. Removal of tumors of the orbital apex via a postero-lateral orbitotomy. J Neurosurg Sci 1998;42:185–8.

43.Sethi DS, Lau DP. Endoscopic management of orbital apex lesions. Am J Rhinol 1997;11:449–55.

44.Kennerdell JS, Maroon JC, Celin SE. The posterior inferior orbitotomy. Ophthalmol Plast Reconstr Surg 1998;14:277–80.

45.Goldberg RA, Shorr N, Arnold AC, Garcia GH. Deep transorbital approach to the apex and cavernous sinus. Ophthal Plast Reconstr Surg 1998;14:336–41.

46.Maus M, Goldman HW. Removal of orbital apex hemangioma using new transorbital craniotomy through suprabrow approach. Ophthal Plast Reconstr Surg 1999;15:166–70.

47.Dahl I, Hagmar B, Idvall I. Benign solitary neurilemmoma (schwannoma). Acta Pathol Microbiol Immunol Scan Sec A 1984;92:91–101.

48.Konrad EA, Thiel HJ. Schwannoma of the orbit. Ophthalmologica 1984;188:118–27.

49.Schatz H. Benign orbital neurilemmoma: sarcomatous transformation in Von Recklinghausen’s disease. Arch Ophthalmol 1971;86:268–73.

50.Rootman J, Goldberg C, Robertson W. Primary orbital schwannomas. Br J Ophthalmol 1982;66:194–204.

51.Lloyd GAS. CT scanning in the diagnosis of orbital disease. J Comput Assist Tomography 1979;3:227–39.

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53.Chisholm LA, Polyzoidis K. Recurrence of benign orbital neurilemmoma (schwannoma) after 22 years. Can J Ophthalmol 1982;17:271–3.

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55.Verity MA, Ebert JT, Hepler RS. Atypical fibrous histiocytoma of the orbit: an electromicroscopic study. Ophthalmologica 1977;175:73–9.

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57.Rodrigues MM, Furgiuele FP, Weinreb S. Malignant fibrous histiocytoma of the orbit. Arch Ophthalmol 1977;95:2025–8.

58.Weiss SW, Enzinger FM. Malignant fibrous histiocytoma. an analysis of 200 cases. Cancer 1978;41: 2250–66.

59.Char DH, Weidner N, Ahn J, Harbour J. Solitary fibrous tumor of the orbit. Orbit 1997;6:1–5.

60.Krohel JB, Rosenberg PN, Wright JE, Smith RS. Localized orbital neurofibromas. Am J Ophthalmol 1985;100:458–64.

61.Darcioglu ZA, Nsr AM, Haik BG. Orbital hemangiopericytoma: clinical and morphologic features. Am J Ophthalmol 1997;124:661–72.

62.Croxatto JO, Font RL. Hemangiopericytoma of the orbit: a clinicopathologic study of 30 cases. Hum Pathol 1982;13:1210–8.

63.Henderson JW, Farrow GM. Primary orbital hemangiopericytoma: an aggressive and potential malignant neoplasm. Arch Ophthalmol 1978;986:666–73.

64.Jakobiec FA, Howard GM, Jones IS. Hemangiopericytoma of the orbit. Am J Ophthalmol 1974;78:816–34.

65.Rice CD, Kersten RC, Mrak RE. An orbital hemangiopericytoma recurrent after 33 years. Arch Ophthalmol 1989;107:552–6.

66.Stetzkorn RK, Lee DJ. Hemangiopericytoma of the orbit treated with conservative surgery and radiotherapy. Arch Ophthalmol 1987;105:1103–5.

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68.Naeser P, Mostrom U. Liposarcomas of the orbit: a clinicopathological case report. Br J Ophthalmol 1982;66:190–3.

69.Schlote T, Nagel G, Vecsei PV, et al. Conjunctival cysts of the orbits: clinical aspects and histology in 4 patients. Klin Monatsbl Augenheilkd 1998;213: 117–20.

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There are no clinical findings that are pathognomonic for optic nerve tumors. In adults without intraocular or central nervous system (CNS) pathology, mild proptosis and the presence of an afferent pupillary defect, marked visual loss with minimal proptosis, optic atrophy (with or without optociliary shunt vessels), chronic disc edema, or unexplained central visual field loss are consistent with the presence of an intraorbital optic nerve tumor.

A number of entities can produce enlargement of the adult optic nerve, including optic nerve sheath meningioma, optic nerve glioma, neurofibroma, hemangioblastoma, vascular insults, metastases, leukemia, lymphoma (Figure 18–1), neuritis, sarcoid (Figure 18–2), trauma, thyroid compressive neuropathy (Figure 18–3), idiopathic inflammation (Figure 18–4), contiguous infection, and drusen.1–4 History and review of systems is usually sufficient to correctly categorize many of these lesions. Most of the

patients with optic nerve infiltration by leukemias, lymphomas, or metastases have known systemic malignancy, although ocular metastases can be the first sign of a primary neoplasm (see Chapter 5). Similarly, it is usually straightforward to distinguish sarcoid, trauma, and hemangioblastoma from other optic nerve tumefactions on the basis of history, physical findings, fluorescein angiography, or laboratory tests.5 The differentiation of a small optic canal tumor from retrobulbar optic neuritis, AION contiguous apical inflammation, infection or a cyst can be difficult.6

MENINGIOMA

The most common adult primary optic nerve sheath tumors are meningiomas; they account for approxi-

Figure 18–3. Thyroid optic neuropathy on axial MRI scan; usually the nerve is compressed.

mately 2 to 5 percent of orbital tumors. Meningiomas are responsible for approximately one-fifth of CNS tumors, and < 15 percent secondarily involve the orbit. Orbital involvement by meningiomas can occur as a result of a primary intraorbital neoplasm, almost always arising from the optic nerve sheath, or secondarily from a meningioma that involves the orbit by contiguous spread from the CNS often in the sphenoid wing. Rarely, a solitary orbital, nonoptic nerve sheath meningioma is found in association with neurofibromatosis.7 Orbital involvement, either primarily or secondarily by meningiomas occur more frequently in females.8 In five series of orbital meningiomas, 140 of 181 patients were women (77%).9–13 In a series from the Mayo Clinic, 139 of 1,376 orbital biopsies (10%) were shown to be meningiomas, and over 66 percent initially arose

from the sphenoid ridge; in less than one-third (42 of 139 cases), the meningioma arose in the orbits.10

The etiology of meningiomas is uncertain. Loss of heterozygosity at chromosome 22a due to mutations of the NF2 gene (and secondary loss of merlin expression) occur in about one-half of the cases.14 A number of other multistep genomic changes occur, especially in more aggressive meningioma tumors.15,16

The vast majority of primary orbital meningiomas arise either from the optic nerve sheath or the bony foramen of the canal. Very rarely, intraorbital, nonbony meningiomas have been reported.7,17–19 Hyperplastic meningothelial cells are often associated with optic nerve gliomas, and some gliomas have been histologically misdiagnosed as meningiomas on the basis of this finding.20 Most optic nerve meningiomas that have been reported in children without neurofibromatosis were probably optic nerve gliomas. In patients with neurofibromatosis (probably < 10% of total cases), bilateral optic nerve sheath meningiomas rarely occur.21–23

The location of an optic nerve sheath meningioma is a major factor in duration of symptoms, amount of visual loss, and degree of proptosis at the time of diagnosis. As examples, a very small, enlarging meningioma in the optic nerve canal will produce rapid visual loss without significant proptosis; rarely, a large optic nerve sheath meningioma grows through the dura and produces proptosis before visual loss (Figure 18–5). If the meningioma arises just posterior to the globe, the first symptoms may be the onset of unilateral hyperopia due to compression of the globe.

It is difficult to accurately assess the frequency of primary orbital meningiomas in various locations.

358 TUMORS OF THE EYE AND OCULAR ADNEXA

Intracanalicular meningiomas are approximately one-third as frequent as those involving the intraorbital optic nerve sheath. Most commonly, optic nerve sheath meningiomas are not entirely confined to the orbit even when they clinically appear to be.

Most frequently, optic nerve sheath meningiomas present in middle-aged women with < 3 mm of axial proptosis and markedly decreased vision (Figure 18–6A). Other signs and symptoms include optic disc pallor, disc edema, headache, diplopia, loss of color vision, afferent pupil abnormality, opticociliary shunt vessels (Figures 18–6B and C) or intraocular invasion.30 Usually, optic disc edema precedes the development of opticociliary shunt vessels by several years, and patients with greater amounts of proptosis have a longer history prior to the discovery of the optic nerve tumor.31 In a middle-aged woman with chronic, profound unilateral “optic neuritis,” subsequent optic atrophy, and minimal proptosis, this tumor should be ruled out with appropriate imaging studies (see below).

The pattern of an intrinsic orbital optic nerve tumor is usually different from a non-neoplastic simulating lesion on either computed tomography (CT) or magnetic resonance imaging (MRI).5,32 There is some overlap in the CT imaging characteristics of the two most common primary optic nerve tumors: optic nerve sheath meningioma and optic nerve glioma.33 The most useful characteristics that distinguish these two processes are the older age of presentation with meningiomas, calcifications in 25 percent of meningiomas versus almost none in gliomas, occasional outgrowth of meningiomas through the optic nerve sheath to involve other orbital structures, and the different patterns of posterior CNS extension. Posterior involvement of the optic nerve glioma usually involves visual pathways, while CNS extension of meningiomas involves the sphenoid bone. Meningiomas frequently produce pneumosinus dilation of the anterior clinoid and ethmoid air cells.

The most common radiologic finding associated with any type of meningioma is alteration of the

contiguous bone, often producing hyperostotic changes. If the optic canal is involved, usually the foramen is enlarged with irregular demineralization and hyperostosis.

The CT pattern of optic nerve sheath meningioma reflects the various growth patterns and calcification of the tumor. The nerve sheath can be enlarged in a tubular pattern (approximately twothirds of cases, (Figure 18–7), a fusiform pattern (approximately one-third of cases, Figure 18–8), or, less commonly, an excrescent pattern (Figure 18–9). The tumors typically enhance with intravenous contrast. An excrescent tumor enlargement is relatively specific for a nerve sheath meningioma.34 While central lucency (tram-track sign) is characteristic of an optic nerve sheath meningioma, it is also associated with other lesions.32,33 This finding is noted in about 25 percent of cases.37 Intratumor calcification can be diffuse, punctate, or psammomatous.38

MRI is the optimal technique to detect optic nerve sheath meningiomas.39 CT has limited sensitivity for small posterior orbital meningiomas or those with subtle intracranial extension.40 The use of fat saturation with gadolinium-DTPA significantly increases the sensitivity of detection of these lesions and their extent (Figures 18–10 and 18–11).41,42 In some cases, fine-needle aspiration biopsies (FNABs) have been diagnostic.43,44 While we have used this diagnostic modality in many orbital diseases (see Chapter 15), we have not found it necessary in most primary optic nerve tumors except atypical apical lesions.

Figure 18–8. Optic nerve sheath meningioma with a fusiform enlargement. On a contrast-enhanced CT scan, the optic nerve (arrows) appears to be encased by tumor. (From Char et al.,70 with permission)

Treatment of CNS meningiomas is the realm of the neurosurgeon.45,46 Early treatment of CNS meningiomas that involve the anterior visual pathways may improve visual prognosis.37,47 Most patients with optic nerve sheath meningiomas have poor vision at the time of presentation, and the neoplasm completely surrounds the optic nerve. Historically, we have followed most optic nerve sheath meningioma patients, if the tumor is confined to the orbit and proptosis was minimal. Often, visual acuity remained stationary for long periods, and there are reports of spontaneous visual improvements.48 If the proptosis associated with an isolated optic nerve sheath meningioma becomes cosmetically unacceptable and the eye is blind, we have resected the orbital