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

370 TUMORS OF THE EYE AND OCULAR ADNEXA

6.Siatokowski RM, Capo H, Byrne SF, et al. Clinical and echographic findings in idiopathic orbital myositis. Am J Ophthalmol 1994;118:343–50.

7.Wan WL, Cano MR, Green RL. Orbital myositis involving the oblique muscles: an echographic study. Ophthalmology 1988;95:1522–8.

8.Patrinely CR, Osborn AG, Anderson RL, Whiting AS. Computed tomographic features of nonthyroid extraocular muscle enlargement. Ophthalmology 1989;96:1038–47.

9.Hornblass A, Jakobiec FA, Reifler DM, Mines J. Orbital lymphoid tumors located predominantly within extraocular muscles. Ophthalmology 1987; 94:688–97.

10.Dabbs CB, Kline LB. Big muscles and big nerves. Surv Ophthalmol 1997;42:247–54.

11.DiBernardo C, Pacheco EM, Hughes JR, et al. Echographic evaluation and findings in metastatic melanoma to extraocular muscles. Ophthalmology 1996;103:1794–7.

12.Simpson JL, Alford MA, Carter KD, Keech RV. Metastatic rhabdomyosarcoma presenting as an isolated lateral rectus restriction. J Pediatr Ophthalmol Strabismus 1999;36:90–1.

13.Bedford PD, Daniel PM. Discrete carcinomatous metastases in the extrinsic ocular muscles: a case of carcinoma of the breast with exophthalmic ophthalmoplegia. Am J Ophthalmol 1960;49:723–6.

14.Ashton N, Morgan G. Discrete carcinomatous metastases in the extraocular muscles. Br J Ophthalmol 1974;58:112–7.

15.Jiang N-S, Fairbanks VF, Hay ID. Assay for thyroidstimulating immunoglobulin. Mayo Clin Proc 1986;61:753–5.

16.Halbach VV, Higashida RT, Hieshima GB, et al. Dural fistulas involving the cavernous sinus: results of treatment in 30 patients. Radiology 1987;163:437–42.

17.Howard GR, Nerad JS, Bonavolonta G, Tranfa F. Orbital dermoid cysts located within the lateral rectus muscle. Ophthalmology 1994;101:767–71.

18.Hakim KN, McNab AA, Sullivan TJ. Spontaneous hemorrhage within the rectus muscle. Ophthalmology 1994;101:1631–4.

19.Sekhar GC, Lemke BN. Orbital cysticercosis. Ophthalmology 1997;14:1599–604.

20.Sekhar GC, Lemke BN, Singh SK. Cystic lesions of the extraocular muscles. Ophthal Plast Reconstr Surg 1996;12:199–205.

Perhaps the greatest degree of orbital neoplastic diversity occurs in the region of the lacrimal gland. The marked variation in tumor patterns and their behavior makes the evaluation and treatment of lacrimal gland masses a challenge. In adults, infection, inflammation, benign and malignant epithelial or lymphoid tumors, and metastatic disease can all present in the lacrimal fossa.1–4 A number of other less common processes can also involve the lacrimal fossa.5–10

A number of symptoms and both clinical and radiographic signs are helpful to delineate the nature of different lacrimal processes. Any enlarging lacrimal mass can produce proptosis, and often the eye is displaced both medially and inferiorly. As a lacrimal gland mass enlarges, it often produces a characteristic “S-shaped” lid (Figure 20–1).

Age is not a useful parameter to differentiate benign from malignant processes. Malignancies of the lacrimal gland can occur at any age, except that they are very rare in children under 10 years old. Laterality, chronicity, dry eye symptoms, and pain are clinical findings that are sometimes helpful in differentiating disease processes. Bilateral involvement excludes an epithelial lacrimal gland neoplasm. Involvement of both lacrimal glands is most consistent with infection, inflammation, or lymphoma. As Gass and Blodi noted, a significant percentage of patients with bilateral lacrimal gland enlargement have an associated systemic process (see chapter on orbital pseudotumors).11 Esoteric systemic processes can present first to the ophthalmologist with lacrimal fossa symptoms. A magnetic resonance imaging (MRI) scan from a patient with bilateral lacrimal gland enlargement secondary to syphilis is shown in Figure 20–2. Lacrimal gland enlargement in a patient with systemic lupus

erythematosus is shown in Figure 20–3. The coronal MRI scan in Figure 20–4 shows bilateral lacrimal gland enlargement in Wegner’s granulomatosis.

Epithelial malignancies of the lacrimal gland most commonly have pain and more rapid progression than benign epithelial neoplasms. Usually, patients with benign mixed tumors (pleomorphic adenomas) have

A

B

Figure 20–1. A, Lacrimal gland lymphoma as a presenting sign of systemic disease. The “S-shaped eyelid” is typical for lacrimal gland enlargement. B, Direct coronal MRI scan of biopsy-proven lesion. Bone marrow biopsies were also positive for lymphoma.

Figure 20–2. Coronal MRI scan of syphilitic involvement of the lacrimal glands.

an insidious onset and chronic symptoms; but these findings are neither specific nor diagnostic.1 This author has never seen a patient with a lacrimal gland malignancy present with dry eye symptoms, although some have absent tear function on testing.

The presence and pattern of bone involvement noted on plain radiography, computed tomography (CT), or MRI are often useful in determining the nature of a lacrimal fossa lesion. Most benign

Figure 20–4. T1-weighted coronal MRI shows bilateral lacrimal gland enlargement in Wegner’s granulomatosis.

processes either do not involve the bones of the lacrimal fossa, or if they do, there is diffuse noninvasive fossa enlargement, without sclerosis. Generalized expansion of the orbit can occur in young patients with chronic lesions.12 In adults, longstanding processes can result in localized, smooth enlargement of the fossa, and this pattern has been noted with benign epithelial lesions (mixed tumors or pleomorphic adenoma), pseudotumors, and rarely lymphomas. Dermoid cysts have a propensity to involve the lacrimal fossa in adults, and usually, they produce a rounded, nonsclerotic bone defect (Figure 20–5). Rarely, a dumbbell-shaped dermoid will break through the lateral orbital wall, as shown in Figure 20–6. Lacrimal fossa bone changes can occur in histiocytosis syndromes, as discussed in the chapter on pediatric orbital tumors. Rarely, traumatic hematic cysts (organized hematomas) can involve the lacrimal fossa (Figure 20–7). Sometimes these post-traumatic

Figure 20–3. T1-weighted MRI scan demonstrates bilateral lacrimal gland enlargement in a patient with systemic lupus erythematosus. FNAB was positive for benign nfiltrate.

Figure 20–5. Axial CT scan demonstrating evenly rounded bone defect in the lacrimal fossa typical of dermoid cyst.

hematic cysts can simulate an epithelial malignancy with apparent bone invasion as shown in Figure 20–8.13–15 Frank bone invasion is diagnostic of either a primary or metastatic malignancy. An adenoid cystic carcinoma of the lacrimal gland with bone involvement is shown in Figure 20–9A. This lesion was diagnosed by fine-needle aspiration biopsy (FNAB) (Figure 20–9B). Both the CT and MRI scans reveal a jagged, serrated invasion of the bone. Often, there is

A

B

Figure 20–7. A, Axial MRI scan shows a biopsy-proven hematic cyst (organized hematoma) that mimics a dermoid. B, Computer reformatted parasagittal CT shows lesion shown in Figure 20–7A.

A

B

Figure 20–6. A and B, Axial CT scan with computed reformation of a dumbbell-shaped dermoid that involved both the orbit and temporalis fossa.

contiguous spread to adjacent structures as shown in Figures 20–9A, C, and 20–10B. These malignancies may be calcified and can also cause bone sclerosis.16 Adjacent bone sclerosis is usually a sign of malignancy, although we have intragland calcification with amyeloid involvement of the lacrimal gland (Figure 20–11).17 Bone sclerosis can occur in irradiated histiocytosis patients, and those with fibrous dysplasia, meningioma, or rarely sarcoid.18

Epithelial tumors of the lacrimal gland are a major diagnostic and clinical problem for the orbital

Figure 20–8. Direct coronal CT scan with coronal reconstruction demonstrating bone alterations in the lacrimal fossa. The lesion simulated an epithelial neoplasm on both CT and ultrasonography but was actually a hematic cyst secondary to trauma

374 TUMORS OF THE EYE AND OCULAR ADNEXA

A

B

C

Figure 20–9. A, Invasion of bone by an adenoid cystic carcinoma of the lacrimal gland demonstrated on CT. B, Fine-needle aspiration biopsy reveals typical adenoid cystic carcinoma. C, MRI scan demonstrates invasion of bone, manifested as loss of normal bone signal void.

surgeon for three major reasons: (1) histologic studies have demonstrated that approximately 50 percent of lacrimal masses are epithelial, and one-half of these are malignant. Despite some clinical and radiographic signs it can often be difficult to distinguish benign from malignant epithelial tumors.19 Some authors believe that calcification or a dense surround with a more lucent center is typical for a malignancy, but others have not found that these patterns are reliable;20 (2) the malignant epithelial tumors (adenoid cystic carcinoma, malignant mixed tumor, mucoepidermoid carcinoma, adenocarcinoma, and undifferentiated carcinomas) have 10year tumor-related mortality of > 80 percent.19,21,22 Incomplete excision of a potentially resectable epithelial malignancy has a fatal prognosis. All such lesions must be recognized and operated on in a manner to minimize potential tumor seeding; and

(3) benign epithelial mixed tumor (pleomorphic

A

B

Figure 20–10. A, This adenoid cystic carcinoma patient’s CT scan demonstrated contiguous involvement of the central nervous system. B, Follow-up CT scan 5 years after radiation treatment; usually, this fatal disease takes many years to kill the patient.

Figure 20–11. Intragland calcification in amyeloid.

adenoma) has a 20 percent malignant transformation rate, and recurrence occurs in at least 25 percent of incompletely resected lesions.19

DIAGNOSIS

A schema for diagnostic decisions based on the clinical presentation of patients with lacrimal gland

lesions is shown in Table 20–1. Patients can be separated into those with and those without inflammatory symptoms and signs. Patients with acute swelling, periorbital pain, scleritis, painful eye movements, or an erythematous, indurated lid most likely have an inflammatory process. The evaluation of orbital inflammatory disease is a multidisciplinary problem. Infectious diseases can involve the lacrimal gland, and a review of systems with emphasis on recent respiratory or generalized infections should be sought. In children and teenagers, there is a history of a prodromal viral syndrome (see chapter on pediatric orbital tumors). As shown in Figure 20–1 lacrimal gland inflammation often gives an S-shaped, swollen, red lid. A number of acute and chronic infectious diseases can involve the lacrimal gland.23 Sarcoidosis is one of many inflammatory lesions that can involve the lacrimal gland.24 Figure 20–12 shows a bilateral case of sarcoidosis, while Figure 20–13 shows a patient who also had biopsy confirmation but who presented with a unilateral lacrimal gland mass with a negative systemic work-up for sarcoid.13 These patients may have a history of systemic

Figure 20–13. Patient had biopsy-proven sarcoid involving only one lacrimal gland with a negative systemic evaluation. Axial

T1–weighted MRI scan shows unilateral involvement.

Figure 20–14. Hilar adenopathy and lacrimal gland uptake on a limited gallium scan are helpful to confirm the diagnosis of sarcoid in a patient not receiving corticosteroids.

covered in the chapter on orbital pseudotumors. Usually, lymphomas occur in a slightly older population than do epithelial lacrimal gland malignancies. Lloyd noted the mean age for lymphoma patients to be 63 years and for pseudotumor patients to be 43 years.16 Benign or malignant lymphoid tumors can expand the lacrimal fossa (Figure 20–17), and can often be bilateral (see Figure 20–1B).16 They usually contiguously involve muscle and fat, with irregular margins (Figure 20–18). Neither benign nor malignant lymphoid lacrimal gland masses usually invade bone.30

As discussed above, patients with bilateral lacrimal gland lesions most commonly have a systemic disease associated with inflammation (sarcoid, collagen vascular disease), infection (tuberculosis, lues, mumps), or lymphoma.11 These patients should have an extensive medical evaluation prior to or simultaneous with the ocular evaluation. Depending on the patient’s findings, complete blood count, urine analysis and urine sediment, urine for Bence-

Jones protein, sedimentation rate, luetic serologies, serum ACE, lysozyme, serum protein electrophoresis, antinuclear antibody, anti-DNA antibodies, Sjögren antibodies, C3, blood urea nitrogen (BUN), creatinine, gallium scan, chest radiography, antineutrophil cytoplasmic antibody (ANCA) and skin tests may be indicated. A patient with Waldenstrom’s macroglobulinemia with lacrimal gland involvement is shown in Figure 20–19.

The most difficult patients to correctly diagnose are those without inflammatory signs who present with a unilateral lacrimal gland mass. Unfortunately, the differential diagnosis includes many of the above mentioned entities, especially lymphoid

378 TUMORS OF THE EYE AND OCULAR ADNEXA

lesions, plus epithelial tumors, mesenchymal neoplasms, and dermoid cysts. Rarely, some other simulating lesions, such as a lacrimal dacryops (Figure 20–20), can be diagnosed correctly on clinical examination alone, and if they are symptomatic, they may be excised.31 In one case, this finding was noted in association with a benign mixed tumor.32 A number of rarer causes of lacrimal fossa tumors will only be diagnosed histologically. Reported lesions included myoepithelium, fibrous histiocytoma, Warthin tumor, solitary fibrous tumor, epithelioid hemangoma, hemangiopericytoma, and Kimura’s disease.

Usually, patients with benign mixed tumors (pleomorphic adenomas) have a long history and minimal pain. In a series reported from London, 13 of 14 benign tumors were present for 1 year prior to surgery and only 1 patient had pain. Rarely, this tumor can present in children, as shown in a 5-year-old boy (Figure 20–21). On axial MRI scans, these are usually relatively discrete; commonly, they enhance with gadolinium (Figure 20–22). On CT, MRI, and plain film radiography, often there is smooth molding of the bone in patients with benign mixed tumors (Figure 20–23).16 The diagnosis of a benign pleomorphic adenoma can be confusing. Wharton and O’Donnell reported a case where there was a hemorrhagic cyst that developed in association with this tumor, leading to diagnostic confusion.40 Rarely, these tumors can develop in the palpebral lobe of the lacrimal gland.41,42

Figure 20–20. Lacrimal dacryops.

In contrast to benign epithelial lacrimal gland tumors, 12 of 17 patients with malignant epithelial tumors had pain.43 In another large retrospective pathology series, neuronal involvement was noted in 11 percent of benign and 31 percent of malignant epithelial lacrimal gland neoplasms.19 The patient shown in Figure 20–10A presented with pain and upper lateral quadrant swelling. At diagnosis, CT demonstrated extraorbital disease (see Figure 20–10B). While most lacrimal gland epithelial

A

B

Figure 20–22. A and B, Benign mixed tumors are relatively discrete (A) and enhance with gadolinium (B).

malignancies arise in the main body, 17 percent in one series arose from the palpebral lobe.42

Dermoid cysts most often present as painless proptosis or patient awareness of a mass along the lateral orbital rim. Sathananthan reported 70 patients with dermoid cysts in the lateral canthus. Thirtyseven percent were mainly intraorbital, and 20 percent were mainly extraorbital. The bone in the lateral wall was found to be abnormal and bony damage is common.44 Rarely, these patients initially present when the cyst breaks, and an inflammatory reaction has developed.45 Even less frequently, dermoids can appear to invade the lateral rectus muscle.46 Metastatic disease to the lacrimal gland, like metastases elsewhere in the orbit, can be the initial presenting sign of a primary tumor.47 Rarely, a metastasis can simulate a malignant lacrimal epithelial tumor.48 Sebaceous neoplasia from the conjunctiva surface can on rare occasions invade the lacrimal gland.49

There are no pathognomonic signs that separate a small adenoid cystic carcinoma from benign mixed tumors. Figure 20–24A demonstrates a small adenoid cystic carcinoma without bone involvement in

a 14-year-old female. As such lesions enlarge, they may only demonstrate bone molding without invasion, as in the 17-year-old shown in Figure 20–25. Figure 20–26 shows a 9-year-old girl who presented to me with what we felt was a solitary benign tumor, which was an adenoid cystic carcinoma. At the time of diagnosis, larger adenoid cystic carcinomas have an irregular posterior extension, usually with bone involvement and frequently with central nervous system (CNS) extension (see Figures 20–9 and 20–10). CT evaluation, using bone window programs, is probably the most sensitive method to detect bone involvement associated with a lacrimal gland tumor.50,51 We recently reviewed our results with MRI in lacrimal lesions and did not find that the use of this technique led to more accurate diagnosis than that with CT.51–53 Some early studies demonstrated that ultrasonography was more accurate than early generation, thick-section CT. In the author’s experience, this is not the case with high-resolution, thinsection (1.5 or 2 mm) CT or MRI.53,54 Further, while ultrasonography has been listed in other reports as being quite accurate, the broad diagnostic groups used in that study were sufficiently diffuse to limit their value.55 As an example, knowing that a lesion is

Figure 20–23. CT demonstrating expansion of the lacrimal fossa with molding of bone, secondary to a benign mixed tumor of the lacrimal gland.