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

400 TUMORS OF THE EYE AND OCULAR ADNEXA

43.Schoub L, Timme AH, Uys CJ. A well differentiated inverted papilloma of the nasal space associated with lymphnode metastasis. SAFR Med J 1973;47: 1663–5.

44.Elner VM, Burnstine MA, Goodman ML, Dortzbach RK. Inverted papillomas that invade the orbit. Arch Ophthalmol 1995;113:1178–83.

45.Lawton AW, Karesh JW, Gray WC. Proptosis from maxillary sinus inverted papilloma with malignant transformation. Arch Ophthalmol 1986;104:874–7.

46.Karcioglu ZA, Wesley RE, Greenidge KC, McCord CD Jr. Proptosis and pseudocyst formation from inverted papilloma. Ann Ophthalmol 1982;14:443–8.

47.Cohn HC, MacPherson TA, Barnes L, Kennerdell JS. Cemento-ossifying fibroma of the ethmoidal sinus manifesting as proptosis. Ann Ophthalmol 1982;14: 173–5.

48.Fields JN, Haverson KJ, Devineni VR, et al. Juvenile nasopharyngeal angiofibroma: efficacy of radiation therapy. Radiology 1990;176:263–5.

49.Font RL, Laucirica R, Patrinely JR. Immunoblastic B-cell malignant lymphoma involving the orbit and maxillary sinus in acquired immune deficiency syndrome. Ophthalmology 1993;100:966–70.

50.Som PM, Brandwein MS, Maldjian C, et al. Inflammatory pseudotumor of the maxillary sinus: CT and MR findings in six cases. AJR Am J Roentgenol 1994;163:689–92.

51.Coutu RE, Klein M, Lessell S, et al. Limited form of Wegener granulomatosis. JAMA 1975;233:868–71.

52.Fauci AS, Haynes BF, Katz P, Wolff SM. Wegener’s granulomatosis: prospective clinical and therapeutic experience with 85 patients for 21 years. Ann Intern Med 1983;98:76–85.

53.Wolff SM, Fauci AS, Horn RG, Dale DC. Wegener’s granulomatosis. Ann Intern Med 1974;81:513–25.

54.Spalton DJ, Graham EM, Page NGR, Sanders MD. Ocular changes in limited forms of Wegener’s granulomatosis. Br J Ophthalmol 1981;65:553–63.

55.Stavrou P, Deutsch J, Rene D, et al. Ocular manifestations of classical and limited Wegener’s granulomatosis. Quart J Med 1993;86:719–25.

56.Kalina PH, Lie JT, Campbell RJ, Garrity JA. Diagnostic value limitations of orbital biopsy in Wegener’s granulomatosis. Ophthalmology 1992;99:120–4.

57.Hardwig PW, Bartley GB, Garrity JA. Surgical management of nasolacrimal duct obstruction in patients

with Wegener’s granulomatosis. Ophthalmology 1992;99:133–9.

58.Janknecht P, Mittelviefhaaus H, Loffler KU. Sclerochoroidal granuloma in Wegener’s granulomatosis simulating an uveal melanoma. Retina 1995;15(2): 150–3.

59.Soukiasian SH, Foster CS, Niles JL, Raizman MB. Diagnostic value of anti-neutrophil cytoplasmic antibodies in scleritis associated with Wegener’s granulomatosis. Ophthalmology 1992;99:125–32.

60.Kay MC, McCrary JA. Multiple cranial nerve palsies in late metastases of midline malignant reticulosis. Am J Ophthalmol 1979;88:1087–90.

61.Simonis-Blumenfrucht S, Mestdagh C, Lustman F, et al. Le granulome malin centro-facial. Dermatologica 1979;158:153–62.

62.Spalton DJ, O’Donnell PJ, Graham EM. Lethal midline lymphoma causing acute dacryocystitis. Br J Ophthalmol 1981;65:503–6.

63.Lubin JR, Jallow SE, Wilson WR, et al. Rhinoscleroma exophthalmos: a case report. Br J Ophthalmol 1981; 65:14–7.

64.Mansour AM, Salti H, Uwaydat S, et al. Ethmoid sinus osteoma presenting as ipiphora and orbital cellulitis: case report and literature review. Surv Ophthalmol 1999;43:413–26.

65.Teed RW. Primary osteoma of the frontal sinus. Arch Otolaryngol 1941;33:255–92.

66.Arnold J. Zwei Osteome der Stirnhohlen. Virchow’s Arch Pathol Anat 1873;57:145.

67.Ersner MS, Saltzman M. Osteoma of the sinuses. Laryngoscope 1938;48:29.

68.Rawlins AG. Osteoma of the maxillary sinus. Ann Otol Rhinol Laryngol 1938;47:735.

69.Wilkes SR, Traumann JC, DeSanto LW, Campbell RJ. Osteoma: an unusual cause of amaurosis fugax. Mayo Clin Proc 1979;54:258–60.

70.Whitson WE, Orcutt JC, Walkinshaw MD. Orbital osteoma in Gardner’s syndrome. Am J Ophthalmol 1986;101:236–41.

71.Ciappetta P, Delfini R, Inanneti G, et al. Surgical strategies in the treatment of symptomatic osteomas of the orbital walls. Neurosurgery 1992;31:628–35.

72.Senior BA, Lanza DC, Kennedy DW, Weinstein GS. Computer-assisted resection of benign sinonasal tumors with skull base and orbital extension. Arch Otolaryngol Head Neck Surg 1997;123:706–11.

Bony orbital involvement can be due to infection, reparative processes, benign neoplasms, malignant primary tumors, and metastases. Metastases to the orbit are the most common tumors that involve the orbital bones, but unlike primary bone lesions, they almost always also invade adjacent structures. As discussed in the chapter on orbital metastases, breast, lung, renal, and gastrointestinal carcinomas can often invade the orbit with secondary bony involvement. The pattern of orbital metastases that involve bone is fairly typical, and as shown in Figure 23–1, these lesions may also invade contiguous structures. In prostate metastases, they may either have that pattern or predominantly produce an osteoblastic lesion (Figure 23–2). In children, neuroblastoma metastases are the most common malignancy to affect the bony orbit, although Wilms’ tumor and Ewing’s sarcoma can also produce this pattern (see chapter on pediatric orbital cancer). Histiocytosis syndromes and infantile myofibromatosis can also involve the orbit.1

A number of other lesions can involve the bony orbital walls. Dermoid cysts in the lacrimal fossa (Figure 23–3) typically create a smooth, scalloped bone defect. Similarly, the pattern of sclerotic bone involvement in unifocal eosinophilic granuloma, part of the Langerhan’s cell histiocytosis syndrome is fairly characteristic (see Figures 16–41A, B and 16–42).

Malignant epithelial lacrimal gland tumors, most commonly adenoid cystic carcinomas, invade bone and have a pattern similar to bony metastases, except that the location in the lacrimal fossa region makes the former diagnosis much more likely (see Figure 20–24). Metastases with bone invasion of the orbit rarely involve the lacrimal fossa.

Hematic orbital cysts, especially those in a subperiosteal position, can simulate a bony lesion (Figure 23–4). The six orbital cases that the author treated all occurred in the superior orbit, although some have been reported in other locations.2–11 The magnetic resonance imaging (MRI) pattern of a chronic hematoma is hyperintense on both T1- and

402 TUMORS OF THE EYE AND OCULAR ADNEXA

Figure 23–3. Axial CT scan showing smooth bone defect secondary to an enlarging dermoid cyst.

T2-weighted images, and this is virtually diagnostic of this process.12

A number of expansile benign orbital tumefactions can mold the orbital bone but do not invade it. In very young children with diffuse orbital tumors, the orbital cavity can show generalized enlargement (see Figure 16–26).

Approximately 200 cases of fibrous dysplasia of the orbit have been described.12 A number of synonyms have been used for this disease including juvenile Paget’s fibrocystic disease and osteogenesis imperfecta tarda. Most patients present with orbital involvement with decreased vision, diplopia or proptosis. Usually, the proptosis is axial, and the globe is displaced downward.8 The bone on plain x-ray films has a ground-glass appearance. The computed tomography (CT) pattern is more typical, with an amorphous ground-glass texture and thickening (Figure 23–5).13,14 The MRI of these lesions is neither diagnostic nor more helpful than CT data. Occasionally, there can be optic nerve compression from fibrous dysplasia, and in such cases, decompression has been done.14 Molecular biologic studies have demonstrated that an oncogene, c-fos, may be important in the development of these bone lesions.15–17 Rarely, malignant degeneration can occur, as was documented in a recent case report of a 78-year-old woman.18

Osteomas involve the orbit secondarily (see chapter on orbital sinus lesions). Most arise as a result of developmental defects, trauma, or infection in the ethmoid or frontal sinuses.17 Occasionally, orbital osteomas can occur in association with the autosomal dominant Gardner’s syndrome and osteoma patients require a complete medical evaluation (physical examination, blood studies, gastrointestinal radiologic studies) to rule out this possibility.19–21 Orbital osteomas are associated with more operative compli-

cations than these tumors in other body sites.22 Rarely, these tumors can provide confusion, such as a recent case report of a patient with a Ewing’s cell sarcoma of the fibula who was noted on bone scan to have an orbital lesion which was due to an osteoma.20

Meningiomas that secondarily involve the orbit can be difficult to diagnose (Figure 23–6). They involve the sphenoid wing in the posterior lateral orbit predominantly, are usually not palpable, and can grow en plaque with few signs or symptoms, until either a vessel or nerve is compromised.21 These tumors are much more common than optic nerve sheath meningiomas.22 Some meningiomas have an early effect on bone, and the presence of hyperostosis on radiologic studies aids in making the correct diagnosis (Figure 23–7). They may have orbital, bone, and central nervous system (CNS) changes at presentation (Figure 23–8). Advanced CNS meningiomas that invade the orbit may produce proptosis, lid swelling, conjunctival chemosis, or a temporal fossa mass (Figure 23–9). Lid and conjunctival edema is probably due to obstruction of venous return. Usually, these meningiomas have an indolent course and present with either proptosis or decreased vision. Occasionally, compression by the tumor may produce central retinal artery occlusion.22 We have diagnosed some of these lesions with CT-directed fine-needle biopsy (Figure 23–10). Historically, most patients developed some evidence of optic nerve dysfunction, with impaired direct and consensual pupillary reaction, a positive Marcus-

Gunn pupil, unilaterally decreased color vision and perception of brightness, and central or paracentral visual field defects. As discussed in Chapter 18, survival with meningioma is excellent.23,24

The management of orbital meningiomas is in flux.25–26 We currently try to grossly remove the entire neoplasm and irradiate the area; with reasonable fol- low-up, we have had > 95 percent control using this approach.27 A skull-based surgical approach using a team of neurosurgical and ophthalmological surgeons yields good results.28 In some cases, the author has found the CO2 laser quite useful to ablate the intraor-

Figure 23–8. Axial CT demonstrates a sphenoid wing meningioma that invaded both the orbit and CNS at the time of presentation.

Figure 23–9. Obstruction of venous return by a large CNS meningioma invading the orbit. The patient has lid swelling and chemosis.

bital and orbital bone meningioma component of this lesion (see Chapter 27). Figures 23–11 and 23–12 show a lesion prior to and after its removal with a CO2 laser. The patient was then treated with photon radiation. Intraoperative CT localization can be useful in minimizing morbidity in lesions of the sphenoid wing. In the case shown in Figure 23–13, we used this approach to safely remove gross tumor, without damaging the intraorbital contents.29

Giant cell granulomas of the orbit can occur after surgery. When they become a problem, they can be curetted.30 Usually, these are expansive lytic lesions, but are quite rare.31 Similarly, subperiosteal orbital abscesses can simulate a tumor in bone and often require drainage.32 These lesions are usually a response to acute sinus inflammation and require management on an emergency basis. In one case, blindness occurred within a few hours of the onset

Figure 23–11. Axial CT shows sphenoid wing meningioma prior to surgery.

of symptoms. Most commonly, there is a history of a recent upper respiratory tract infection with the rapid development of eye edema, proptosis, and pain. Usually, it is difficult to distinguish subperiosteal abscess from other forms of sinusitis with contiguous orbital involvement.

Rarely, hemangiomas may arise in bone; they account for approximately 1 percent of primary bone tumors in other areas of the body (Figure 23–14).33 There have been fewer than 20 case reports of intraosseous hemangiomas of the orbit. Occasionally, hemangiomas can simulate meningioma, since they have areas of bone density on CT, but an area of round or oval rarefaction of bone is almost pathognomonic.34 Unlike meningioma, the

striations and stippled effect are vertical and parallel. These lesions are usually encapsulated and are best treated surgically.35,36 Some will not recur even after a partial excision.34 Rarely, multiple lesions have been reported.37

Brown tumors are secondary tumors of orbital bone that represent focal bone lesions of hyperparathyroidism.38 The bone in these cases is replaced by proliferating fibrous tissue. This lesion can be occasionally confused with a fibrous dysplasia or a mucocele on imaging studies.39 However, these patients usually have a history of either primary hyperparathyroidism or hyperparathyroidism in association with renal failure. Sometimes these tumors are quite vascular, and some clinicians embolize them prior to surgery.40

Osteoblastoma, another rare condition affecting the bony orbit, usually arises from the orbital portion of the frontal bone. It may involve the sinuses, and most commonly occurs in children.41 It is managed by neurosurgery.42

Osteogenic sarcoma can also rarely involve the orbit either as a primary neoplasm or following treatment of bilateral retinoblastoma patients.43–45 In any child who has survived bilateral retinoblastoma, the

incidence of secondary osteogenic sarcoma is approximately 400-fold that in normals. These children should be evaluated with yearly bone scans, and any sign of an orbital tumor should mandate prompt

Figure 23–14. T1-weighted axial MRI scan of an intradiploic hemangioma.

406 TUMORS OF THE EYE AND OCULAR ADNEXA

Figure 23–15. Chordoma metastatic to an extraocular muscle on T1-weighted axial MRI scan.

biopsy. The management of orbital osteogenic sarcoma is local surgical debulking and chemotherapy.

Chondrosarcoma is a similar neoplasm arising from cartilage46 (see Figure 17–38). Approximately 10 orbital cases have been reported.47,48 The preferred treatment is surgical excision. The tumor is characterized by progressive slow recurrence without distant metastases. Death can occur as a result of contiguous spread into the CNS.

Chordomas, usually arising either in the CNS in the region of the dorsum sellae or clivus or arising from the sinuses, can secondarily involve the orbit. They are quite rare and the author has not managed such a case.49,50 We did manage a metastatic chordoma that secondarily involved extraocular muscle (Figure 23–15).

Aneurysmal bone cysts are discussed in the chapter on pediatric orbital tumors. These usually are multisystem vascular lesions, and often patients have other bone abnormalities.51,52

Several other benign and malignant processes can involve the orbital bones. Approximately 30 cases of epidermoid cysts of the orbital bones have been On MRI scans, these are usually

hypointense on T1-weighted and hyperintense on T2- weighted scans and do not enhance with gadollinium.55 The histologic patterns of these lesions is distinct from an orbital dermoid, and these epidermoid lesions usually are in the intradiploic space.53–56 Rarely, a necrotizing vasculitis has been reported to destroy a portion of the orbital wall.57 A benign myxoma of the orbital wall can also destroy orbital bone.58

Giant cell tumors are approximately twice as frequent as aneurysmal bone cysts.59,60 The management of the giant cell bone tumors is excision. Postoperative radiation in one series resulted in a local control rate in 19 of 21 cases in all body sites. The two failures were ultimately salvaged.59

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Symptomatic fibrous orbital tumefactions are relatively uncommon; they occur either as a result of a primary fibrous tumor or as a secondary reactive process. These lesions most frequently involve extraconal areas of the orbit. Most commonly, fibrous orbital processes occur as part of a spectrum of changes associated with orbital pseudotumors (see Chapter 26) or thyroid orbitopathy.1 A computed tomography (CT) scan of a patient with Graves’ disease, thyroid orbitopathy, and orbital fibrosis is shown in Figure 24–1. On CT, orbital detail is obliterated by this fibrous process.

For most orbital tumefactions, imaging studies are quite diagnostic; for fibrous lesions, their accuracy is limited.2,3 There is a great deal of overlap, which can result in misdiagnoses. Generally, benign fibrous tumors tend to be well circumscribed, while aggressive malignant lesions infiltrate and destroy

Figure 24–1. “Orbital wipeout syndrome”; secondary fibrosis as a result of thyroid orbitopathy.

bone. Similarly, on magnetic resonance imaging (MRI) the benign lesions are more likely to be homogeneous, while the malignant tumors can change their pattern of homogeneity between T1- and T2-weighted images.2

Both benign and malignant fibrous histiocytomas can involve all areas of the orbit.4–8 In the author’s experience they have a predilection for the intraconal space, and they are therefore discussed in Chapter 17. Fibrous histiocytomas appear to have a loss of chromosome 13 as their most frequent genomic alteration, suggesting there may be a tumor suppressor gene in that location.9 Neurofibromas, either multiple or plexiform, as part of the neurofibromatosis syndrome, as an isolated finding, or after enucleation, can occur in an intraor extraconal location (Figure 24–2).10 Fibroma can also occur as a consequence of trauma or surgery.11

Nodular fasciitis was first described in 1955, and there have been over 1,000 cases reported.12 It can often be difficult to histologically differentiate this lesion from other tumors, especially sarcomas.12,13 One case of an ophthalmic nodular fasciitis, diagnosed with fine-needle aspiration biopsy, has been reported.13 Font and Zimmerman described 10 patients with ophthalmic findings: 5 involving the lids, 2 the periorbita, 1 the brow, 1 subconjunctivally, and 1 the limbus.14 Generally, these lesions were nonencapsulated, round, oval tumors. I have seen one such orbital case. As shown in Figure 24–3, this patient had a lesion of the orbital floor with elevation of the globe. Both clinically and histologically, it is difficult to differentiate such a lesion from a fibrosarcoma, myxoma, neurofibroma, neurilemmoma, or sarcoma.13,15