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

B

Figures 20–24. A and B, Small adenoid cystic carcinoma without evidence of bone involvement on axial CT (A). Another small tumor of the same histiologic cell type is shown on axial MRI in another young patient (B).

in the sarcoma/lymphoma/metastatic group is not extremely helpful.55 In one series, a benign mixed tumor was incorrectly diagnosed with echography as a hemangioma, and a dermoid was labeled as a benign mixed tumor.55 A diagnosis based on CT appearance can also be deceiving, since a traumatic blood cyst, both radiographically and on quantitative echography, can be consistent with an epithelial tumor. The MRI pattern of these degenerative blood lesions is usually typical.

We have used clinical examination combined with thin-section, high-resolution CT or MRI to establish a preliminary diagnosis in all lacrimal cases. As discussed in the chapter on diagnosis and management of orbital tumors, we do not use ultrasonography in the diagnosis of orbital tumors, since it does not add to the information available from CT or MRI, and it is neither as effective in delineating extraorbital spread nor as useful as CT or MRI in

Figure 20–26. Adenoid cystic carcinoma in a 9-year-old child, with obvious invasion. This child later developed widespread disease.

treatment planning. If there is a unilateral lacrimal gland mass that has invaded bone, FNAB (see Chapter 15) is an excellent method to diagnose the nature of the lesion. Figure 20–9B shows the diagnostic FNAB of an adenoid cystic carcinoma. There are fewer than five cases reported in the medical literature of any human tumor spread by FNAB using a 23-gauge or smaller needle.

In unilateral lacrimal fossa lesions that do not have inflammatory signs associated with them, we assume (often a false-positive assumption) that the lesion may be an epithelial tumor and plan to remove it en bloc.

As discussed above, while the CT or MRI pattern of dermoid cysts is almost pathognomonic, with the rounded, scalloped bone loss, and a cystic mass (Figures 20–4 and 20–27), it is often difficult to differentiate small malignant neoplasms, benign mixed tumors, and less common lacrimal gland malignancies with noninvasive techniques. The surgical approach to these lesions is discussed in the chapter on orbital therapy.

THERAPY

The management of nonepithelial neoplasms of the lacrimal gland is relatively straightforward. Prior to any form of therapy, we obtain a Schirmer test to document tear production by the lacrimal gland. Dermoid cysts are removed surgically (see chapter on orbital surgery). If the capsule is ruptured prior to or during surgery, the lesion is removed piecemeal, the bony fossa is curetted, and 40 mg of long-acting steroid is injected into the area to decrease the inflammatory response.

The management of both benign and malignant lymphoid lesions is discussed in the chapter on lymphoid lesions. All these patients require a medical oncologic evaluation to rule out systemic lymphoma (physical examination, chest-abdominal CT, urine for Bence-Jones proteins, serum protein electrophoresis, complete blood count [CBC], and bone marrow biopsies). Benign lesions can often be effectively treated with short-term steroids or low-dose irradiation. Lymphomas require 35 to 40 gray (Gy) of photon irradiation, if the orbit is the only site of tumor involvement.

Figure 20–27. Typical axial T1-weighted MRI pattern of a dermoid cyst in the lacrimal fossa.

The treatment for localized epithelial neoplasms is surgical.56 Benign mixed tumors are usually encapsulated; if the entire neoplasm is not removed, there is a 20 to 28 percent recurrence rate, and approximately 20 percent of reported pleomorphic adenomas have undergone malignant degeneration.19,20 If a benign mixed tumor is inadvertently incised, contiguous spread can occur in the orbit, bone, and adjacent areas. Completely resected pleomorphic adenomas have less than a 3 percent recurrence rate.19 We attempt to resect any tumor localized in the lacrimal fossa area using careful dissection and isolation of both the orbital periosteum and periorbita. Sometimes, this is not possible. Wright noted that 6 of 26 benign mixed tumors had erosion of orbital bone through to the dura.57

It is difficult to make definitive statements regarding the management of malignant epithelial tumors of the lacrimal gland for three reasons: (1) there is a paucity of reported cases, and no controlled treatment trials; (2) most of the reported irradiated patients did not receive what is now felt to be optimal radiation;58–60 and (3) while this group of tumors has a very high rate of metastases, often the interval between treatment and metastatic disease is very prolonged.19

Adenoid cystic carcinoma is the most frequent epithelial lacrimal gland malignancy, and it accounts for approximately 1.6 percent of all orbital tumors, and 29 percent of epithelial neoplasms of the lacrimal gland.61,62 Usually, these patients present with proptosis and often have pain. Most patients are

382 TUMORS OF THE EYE AND OCULAR ADNEXA

not candidates for local resection. Wright noted that only 4 of 24 adenoid cystic carcinoma patients had apparently localized intraorbital tumors at the time of presurgical evaluation. At orbitotomy, only 2 of these 4 lesions (8%) were confined to the orbit.57 The Mayo Clinic experience with 26 adenoid cystic carcinomas was similarly dismal, and neither radiation nor surgery appeared to markedly affect the disease course.61 In a retrospective uncontrolled study, survival in cases that were not treated with exenteration was 9 years, while the exenterated cases had a 6.7- year life span.21 Only 3 of 26 remained alive at the completion of that study, most having died from contiguous spread to the CNS, lung (5 of 13), or lymph node (3 of 13) metastases.61 Other sites of metastatic adenoid cystic carcinoma include the skeletal system and liver; widespread dissemination can become apparent even in the presence of local disease control.63 Overall, 15-year survival with this tumor is < 10 percent.64 Six long-term survivors have been reported; we have two additional patients.65 In a histologic study, Gamel and Font noted that the presence of a basaloid histologic pattern was correlated with poor prognosis. If this pattern was present, 5-year survival was 21 percent; and if it was absent, it was 71 percent.66

Prognosis in patients 18 years of age and younger seems to be somewhat better. Eleven cases that met that inclusion criterion were reported from the Armed Forces Institute of Pathology. Eight with long-term follow-up survived. Probably, this reflected that the tumors had less aggressive histologic features.67,68

There have been no large series using radiation therapy to treat adenoid cystic carcinomas of the lacrimal gland. Brada and Henk reported their results with irradiation of 33 malignant epithelial lacrimal gland tumors.69 They noted that adjunctive radiation after incomplete tumor excision was effective in only 1 of 13 patients with adenocarcinoma, undifferentiated carcinoma, or malignant mixed tumors. In 7 of 13 adenoid cystic carcinoma patients with residual local disease, they achieved good control and felt that > 60 Gy was best for tumor control.70 Unfortunately, it is not clear in their study what surgical procedures were done to the patients before irradiation. It is therefore difficult to deter-

mine the exact effect of radiation in contrast to surgery on tumor-related mortality.71,72

Similarly, while our follow-up is < 10 years, we have obtained good palliation in lesions that have local disease and contiguous extraorbital spread with 60 Gy of photon radiation. Examples of pretreatment clinical photographs (see Figure 20–10A) and 5-year follow-up CTs (see Figure 20–10B) of a patient who initially presented with CNS extension of an orbital adenoid cystic carcinoma are shown. As Simpson and colleagues stressed, this tumor requires a higher radiation dose than previously thought.58 Our limited experience with charged particle beam irradiation in this malignancy has emphasized the point that often tumor spread is underestimated, and we have observed late marginal recurrences with that modality. Probably, optimal treatment is with wide-field photon irradiation to approximately 70 Gy intensity-modulated conformal. The adjunct use of a charged particle beam may also allow more dose to the tumor with less morbidity to contiguous structures. As discussed in other chapters, the use of intensity-modulated conformal therapy may allow us to deliver more dose with less morbidity than currently possible. Other workers have used brachytherapy, but there are minimal data to support that approach.

Meldrun and colleagues reported the use of intracarotid chemotherapy for advanced disease in addition to radiation in a few patients with shortterm follow-up.70 Unfortunately, in the author’s experience, often such patients will survive for up to 10 years, with locally extensive neoplasms before developing tumor-related mortality.

Gormley and colleagues presented 16 cases with intracranial extension of adenoid cystic carcinoma that involve the salivary glands. Their report was optimistic, but the caveat is that one really needs 15 years of follow-up to be certain of a cure.71

The optimal management of localized adenoid cystic carcinoma that has not invaded the orbital bones is uncertain. Surgical resection is usually not possible, as most of these carcinomas have bone and CNS involvement at presentation. Most long-term survivors underwent radical exenteration. The patient shown in Figure 20–25 is such a case.57,61,64 The author would still advocate radical orbitectomy in a

young patient, since some irradiated patients have been seen to have good tumor control for up to 7 years but then relapse. In an older patient with this disease, irradiation may be a reasonable primary therapy option.

Too few cases of other types of primary epithelial malignancies of the lacrimal gland have been reported to draw significant conclusions regarding treatment, and we treat all such patients in the same way as for adenoid cystic carcinomas.72–78 In the largest report of primary adenocarcinomas of the lacrimal gland from several centers, the authors concluded, on the basis of 13 cases, that exenteration plus radiation was the most efficacious treatment.79 In some cases of adenoid cystic carcinomas elsewhere, combination chemotherapy has had up to a 25 percent response rate.80

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22.Bloche C, Lloyd DM, Izbicki JR, et al. Successful surgical treatment of liver and kidney metastases 25 years after a primary lacrimal gland tumor. Eur J Surg Oncol 1993;19:569–72.

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24.Collison JMT, Miller NR, Green WR. Involvement of orbital tissues by sarcoid. Am J Ophthalmol 1986;102:302–7.

25.Sacher M, Lanzieri CF, Sobel LI, Som PM. Computed tomography of bilateral lacrimal gland sarcoidosis. J Comp Assist Tomogr 1984;8:213–5.

26.Lowder CY, Char DH. Uveitis: a review. Western Med 1984;140:421–32.

27.Khan JA, Hoover DL, Giangiacomo J, Singsen BH. Orbital and childhood sarcoidosis. J Pediatr Ophthalmol Strabismus 1986;23:190–4.

28.Peterson EA, Hymas DC, Pratt DV, et al. Sarcoidosis with orbital tumor outside the lacrimal gland: initial

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manifestation in two elderly white women. Arch Ophthalmol 1998;116:804–6.

29.Harris GJ, Dixon TA, Haughton VM. Expansion of the lacrimal gland fossa by a lymphoid tumor. Am J Ophthalmol 1983;96:546–7.

30.Monbaerts I, Schlingeman RO, Goldschmeding RN, et al. The surgical management of lacrimal gland pseudotumors. Ophthalmology 1996;103:1619–27.

31.Brownstein S, Belin MW, Krohel GB, et al. Orbital dacryops. Ophthalmology 1984;91:1424–8.

32.Christie DB, Woog JJ, Lahav M. Combined dacryops with underlying benign mixed cell tumor of the lacrimal gland. Am J Ophthalmol 1995;119:97–9.

33.Grossniklaus HE, Wojno TH, Wilson ME, Someren AO. Myoepithelioma of the lacrimal gland. Arch Ophthalmol 1997;115:1588–90.

34.al-Hazzaa SA, Specht CS, McLean IW, et al. Benign orbital fibrous histiocytoma simulating a lacrimal gland tumor. Ophthal Surg Lasers 1996;27:140–2.

35.Bonavolonta G, Tranfa F, Staibano S, et al. Warthin tumor of the lacrimal gland. Am J Ophthalmol 1997;124:857–8.

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37.Coombes AG, Manners RM, Ellison DW, Evans BT. Lacrimal gland epithelioid haemangioma. Br J Ophthalmol 1997;81:1020.

38.Kodama T, Kawamoto K. Kimura’s disease of the lacrimal gland. Acta Ophthalmol Scand 1998; 76:374–7.

39.Burnsteine MA, Morton AB, Font MB, et al. Lacrimal gland hemangiopericytoma. Orbit 1998;17.

40.Wharton JA, O’Donnell BA. Unusual presentations of pleomorphic adenoma and adenoid cystic carcinoma of the lacrimal gland. Aust NZ J Ophthalmol 1999;27:145–8.

41.Yamada T, Kato T, Hayasaka S, et al. Benign pleomorphic adenoma arising from the palpebral lobe of the lacrimal gland associated with elevated intraocular pressure. Ophthalmologica 1999;213:269–72.

42.Vangveeravong S, Katz SE, Rootman J, White V. Tumors arising in the palpebral lobe of the lacrimal gland. Ophthalmology 1996;103:1606–12.

43.Stewart WB, Krohel GB, Wright JE. Lacrimal gland and fossa lesions: an approach to diagnosis and management. Ophthalmology 1979;86:886–95.

44.Sathananthan N, Moseley IF, Rose GE, Wright JE. The frequency and clinical significance of bone involvement in outer canthus dermoid cysts. Br J Ophthalmol 1993;77:789–94.

45.Sherman RP, Rootman J, Lapointe JS. Orbital dermoids. Clinical presentation and management. Br J Ophthalmol 1984;68:642–52.

46.Howard GR, Narad JS, Bonabolonta G, Tranfa F. Orbital dermoid cysts located within the lateral rectus muscle. Ophthalmology 1994;101:760–71.

47.Font RL, Ferry AP. Carcinoma metastatic to the eye and orbit. Cancer 1976;38:1326–35.

48.Bernstein-Lipschitz L, Lahav M, Chen V, et al. Metastatic thyroid carcinoma masquerading as lacrimal gland tumor. Graefes Arch Clin Exp Ophthalmol 1990;228:112–5.

49.Mooy CM. Intraepithelial sebaceous neoplasia invading the lacrimal gland. Br J Ophthalmol 1997;81: 612–3.

50.Norman D, Char DH, Newton TH. Imaging techniques in orbital disease. In: Littleton JT, Durizsch MC, editors. Sectional imaging methods: a comparison. Baltimore, MD: University Park Press; 1983.

51.Char DH, Sobel D, Kelly WM, et al. Magnetic resonance scanning in orbital and intraocular tumor diagnosis. Ophthalmology 1985;92:1305–10.

52.Jakobiec FA, Yeo JH, Trokel SL, et al. Combined clinical and computed tomographic diagnosis of primary lacrimal fossa lesions. Am J Ophthalmol 1982;94:785–807.

53.Polito E, Leccisotti A. CT and MRI features of lacrimal fossa tumors. Orbit 1993; 25–38.

54.Hesselink JR, Davis KR, Dallow RL, et al. Computed tomography of masses in the lacrimal gland region. Radiology 1979;131:143–7.

55.Balchunas WR, Quencer RM, Byrne SF. Lacrimal gland fossa masses: evaluation by computed tomography and A-mode echography. Radiology 1983; 149:751–8.

56.Janecka I, Housepian E, Trokel S, et al. Surgical management of malignant tumors of the lacrimal gland. Am J Surg 1984;148:539–41.

57.Wright JE. Factors affecting the survival of patients with lacrimal gland tumours. Can J Ophthalmol 1982;17:3–9.

58.Simpson JR, Thawley SE, Matsuba HM. Adenoid cystic salivary gland carcinoma: treatment with irradiation and surgery. Radiology 1984;151:509–12.

59.Sidrys LA, Fritz KJ, Variakojis D. Fast neutron therapy for orbital adenoid cystic carcinoma. Ann Ophthalmol 1982;14:42–5.

60.Sealy R, Buret E, Cleminshaw H, et al. Progress in the use of iodine therapy for tumors of the eye. Br J Radiol 1980;635:1052–60.

61.Lee DA, Campbell RJ, Waller RR, Ilstrup DM. A clinical pathologic study of primary adenoid cystic carcinoma of the lacrimal gland. Ophthalmology 1985;92:128–34.

62.Font RL, Smith SL, Bryan RG. Malignant epithelial tumors of the lacrimal gland: a clincopathologic study of 21 cases. Arch Ophthalmol 1998;116:613–6.

63.Byers RM, Berkeley RG, Luna M, Jesse RH. Combined therapeutic approach to malignant lacrimal gland tumors. Am J Ophthalmol 1975;79:53–5.

64.Marsh JL, Wise DM, Smith M, Schwartz H. Lacrimal gland adenoid cystic carcinoma: intracranial and extracranial en bloc resection. Plast Reconstr Surg 1981;68:577–85.

65.Naugle T Jr, Tepper DJ, Haik BG. Adenoid cystic carcinoma of the lacrimal gland: a case report. Ophthal Reconstr Surg 1994;10:45–8.

66.Gamel JW, Font RL. Adenoid cystic carcinoma of the lacrimal gland: the clinical significance of a basaloid histologic pattern. Hum Pathol 1982;13:219–25.

67.Tellado MV, McLean IW, Specht CS, Varga J. Adenoid cystic carcinomas of the lacrimal gland in childhood and adolescence. Ophthalmology 1997;104:1622–5.

68.Shields JA, Shields CL, Eagle RC, et al. Adenoid cystic carcinoma of the lacrimal gland simulating a dermoid cyst in a nine year old boy. Arch Ophthalmol 1998;116:1673–6.

69.Brada M, Henk JM. Radiotherapy for lacrimal gland tumours. Radiother Oncol 1987;9:175–83.

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Tumors of the lacrimal sac are uncommon. Approximately 400 cases have been reported in the literature, and a little more than one-half were malignant.1–4 As evidence of the rarity of this neoplasm, only 10 cases were observed at the Institute of Ophthalmology in London between 1948 and 1967.2 The clinical presentation of a lacrimal sac neoplasm is similar to that of chronic dacryocystitis; however, lacrimal sac tumors often involve the area both below and above the medial canthal tendon, whereas in chronic dacryocystitis, the swelling is usually limited to the area inferior to the tendon. Patients develop epiphora and a small medial canthus mass as the lesion expands (Figure 21–1). Spontaneous retrocanalicular bleeding associated with epiphora is a classic finding with a malignant lacrimal sac tumor, although it can also occur with inflammation. In a series of 117 cases from the Armed Forces Institute of Pathology, approximately 50 percent of lacrimal sac neoplasms presented with epiphora, 38 percent with dacryocystitis, and 36 percent with a mass.5

A number of different malignant processes can involve the lacrimal sac, and polyps in this location can undergo malignant degeneration.6 Carcinoma generally occurs in older patients and is associated with pain or epiphora or both. Deaths from metastasis of lacrimal sac tumors are uncommon and are usually due to local extension.

Squamous cell carcinoma can involve the lacrimal sac or the canaliculi as either a primary tumor or as a result of contiguous spread from the eyelids, conjunctiva, or sinuses.7,8 There are a few reports, similar to conjunctival squamous cell carcinoma, of human papilloma virus (HPV) positivity.9 Mucoepidermoid carcinomas can involve the conjunctiva, lacrimal

gland, or the sac.10–12 Approximately 29 cases of lacrimal sac malignant melanomas have been reported; as with other malignancies in this area, symptoms are consistent with a chronic dacryocysti- tis.4–13 As discussed in Chapter 4, in less than 4 percent of conjunctival melanomas, involvement of the lacrimal drainage system can occur.14 A number of less common neoplasms have involved the lacrimal sac, including oxyphil cell adenoma, leukemia, sinus histiocytosis, hemangiopericytoma, mucoepidermoid

A

B

Figure 21–1. A, Lacrimal sac plasmacytoma that has produced a mass both above and below the medial canthal tendon. Growth superior to the medial canthal tendon is more consistent with a tumor than with dacryocystitis. This is its clinical appearance. B, Axial CT scan of the same case demonstrates a mass with bone molding.

carcinoma, cavernous hemangioma, adenoid cystic carcinoma, inverted papilloma (Figure 21–2), fibrous histiocytoma, fibroma, solitary fibrous tumors, angiofibroma, metastasis, contiguous extension of sinus carcinoma, juvenile xanthogranuloma, oncocytic hyperplasia, oncolytic adenocarcinomas, and lymphoid lesions including lymphoma and leukemia. Figure 21–2A shows a lymphoid lesion that involved the lacrimal sac.

Sarcoidosis has also been reported in the lacrimal sac. Chest radiographs can be negative; either serum angiotensin converting enzyme (ACE) assays or limited gallium scans are usually positive.45 Rarely, inflammatory sinus disease, such as Wegener’s granulomatosis, can also involve the lacrimal drainage system.46 Usually, Wegener’s granulomatosis patients have positive serum antineutrophil cytoplasmic antibody (ANCA) levels and cutaneous ulceration.

The treatment of lacrimal sac neoplasms depends on the time of diagnosis (preoperative, intraoperative, or postoperative) and the nature of the tumor. Most cases are not suspected prior to surgery and are discovered either during or after performing a dacryocystohinostomy (DCR). Patients with a bloody canalicular discharge should have an evaluation with computed tomography (CT), using bone windows, and magnetic resonance imaging (MRI) prior to

surgery (Figure 21–3). Some patients may have regional adenopathy involving the preauricular, submaxillary or cervical nodes. These later suspicious areas should be evaluated with fine-needle aspiration biopsy (FNAB) prior to surgery. Unlike chronic dacryocystitis, lacrimal sac malignancies often extend downward, with swelling developing above the medial canthal tendon. If a suspicious mass is noted at the time of a DCR, frozen-section biopsy should be performed.

The management of lacrimal sac tumor depends on its histology. While some benign tumors, such as fibrous histiocytomas, can recur, wide local excision, followed by intubation of the canalicular remnants with silicone tubes to retain patency of the nasal lacrimal drainage system, is often adequate treatment.

Only rarely has death by local extension of primary lacrimal sac neoplasms been reported. If a primary epithelial malignancy or melanoma is present and appropriate evaluations for contiguous extension (orbit, brain, and sinus imaging studies) and metastatic disease (chest and abdominal CT, complete blood count, lactate dehydrogenase, glutamyl

A

B

Figure 21–2. MR of an inverted papilloma of the lacrimal sac.

Figure 21–3. A, Lymphoid lesion that involved the lacrimal sac. B, Demonstrates the lesion from the patient shown in 3A on axial

T1–weighted MRI scan.

388 TUMORS OF THE EYE AND OCULAR ADNEXA

transpeptidase, carcinoembryonic antigen [CEA], alkaline phosphatase) are negative, exenteration is sometimes indicated. Some tumors, however, can be controlled with wide excision and wide-field photon irradiation.8,30 Occasionally, radical combined orbitalsinus exenteration with postoperative irradiation has been performed.20

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