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

A number of benign and malignant sinus processes can contiguously involve the adult orbit. These include infections and their sequelae, immunologically mediated diseases, idiopathic isolated inflammations, and both benign and malignant neoplasms.1 Sinusitis with contiguous infectious extension into the orbit is less common in adults than in children, unless the patient has an acquired immunodeficiency.

MUCOCELE

In adults, the most common orbital sinus problem is mucocele, generally arising from either the ethmoid or frontal sinus.2–5 In the current era, most patients with mucoceles have a history of either midface bone trauma or severe sinusitis since childhood. Usually, the patients present with a history of recurrent nose and sinus disease and the onset of unilateral proptosis (Figure 22–1). Figure 22–2A shows a typical ethmoid mucocele on axial computed tomography (CT) and coronal T1-weighted magnetic resonance imaging (MRI) (Figure 22–2B). The scans show a cystic lesion arising from the sinus which has destroyed the lamina papyracea and invaded the orbit. A dermoid

Figure 22–1. Patient with slight anterior and lateral proptosis due to sinus mucocele.

cyst arising in the medial orbit can appear clinically similar to a mucocele (Figure 22–3), but dermoid cysts leave the medial orbital wall intact (Figure

A

B

Figure 22–2. A, Axial CT demonstrating mucocele arising from the ethmoid sinus. B, T1-weighted direct coronal MRI scan.

Figure 22–4. Axial CT with parasagittal re-formation of orbital dermoid cyst. Note that in contrast to Figure 22–2, the medial wall of the orbit is intact.

The second most common sinus process that involves the adult orbit is malignant squamous cell

A

B

Figure 22–6. A, Coronal reformatted CT scan demonstrating extradural extension of a mucocele. B, Direct coronal MRI demonstrates a process similar to the one shown in Figure 22–10A and in other patients.

carcinoma with contiguous spread.10–14 Epidemiologic studies have demonstrated that a number of environmental toxins, including nickel, chrome pigments, mustard gas, radium, and dust are associated with an increased risk of sinus cancer.15 Squamous or undifferentiated carcinomas account for more than 70 percent of all sinus cancer.15,16 Most of these malignancies are not localized to the sinuses at the time of diagnosis.17 Approximately 20 to 45 percent have orbital invasion, and 80 percent have cranial nerve involvement when they are first seen by a physician.10,11,18 The patient shown in Figure 22–8

A

B

C

Figure 22–7. A, Coronal CT of large multiple sinus, orbit, and CNS mucocele. B, Preoperative photographs of Figure 22–7A. C, Postoperative photographs of Figure 22–7A.

Figure 22–8. Large sinus carcinoma in a patient whose initial symptoms were diplopia and visual loss.

year survival is less than 2 percent.22 Treatment consists of surgery, radiotherapy, and chemotherapy.22–25 Newer approaches have improved survival and decreased morbidity. Itami and colleagues reported on 37 patients with large maxillary sinus tumors that had a local recurrence-free survival of 59 percent at 5 years. Unfortunately, historically there was a significant incidence of visually destructive radiation retinopathy in survivors.26–33 In one series from Japan, 8 of 25 patients who were irradiated had significant ocular complications within 2 years of treatment.34 The use of intensity-modulated conformal therapy should probably diminish the incidence of radiation damage to the eyes and orbits.35 Figure

22–10A and B demonstrate the conformal treatment plan, which shows that while the sinuses have received high-dose radiation, there is relative sparing of the eye and visually important structures.

MISCELLANEOUS LESIONS

A variety of other malignant sinus tumors can involve the orbit, including osteosarcoma, adenocarcinoma, adenoid cystic carcinoma, chondrosarcoma, mucoepidermoid carcinoma, rhabdomyosarcoma, esthesioneuroblastoma, and fibrosarcoma. The MRI

A

Figure 22–9. Axial CT shows sinus carcinoma invasion of the orbit.

B

Figure 22–10. A and B, Intensity-modulated conformal therapy demonstrating a marked reduction of radiation to the eye, nerve, and visual pathways using this newer technique. This lateral approach should markedly diminish radiation complications associated with high-dose treatment for sinus carcinoma.

394 TUMORS OF THE EYE AND OCULAR ADNEXA

patterns of both orbital and sinus lymphomas is typical; usually, these lesions are hypointense on T1 and hyperintense with gadolinium enhancement.36 Rarely, a CNS tumor can invade both the sinuses and the orbit.37 Figure 22–11 demonstrates a young woman with an apical involvement of the orbit and painful ophthalmoplegia secondary to an inapparent adenoid cystic carcinoma arising from the sinus. A fine needle is visible in the apical tumor; the fineneedle aspiration biopsy (FNAB) was used to establish a diagnosis. Retrospective analysis of the CT shows a small focus of sinus origin. Figure 22–12 shows a nasopharyngeal carcinoma with orbital involvement in a 25-year-old Asian male. Orbital invasion from a nasopharyngeal carcinoma is relatively rare, and in a series of 562 cases, only 18 had orbital involvement, 4 being bilateral.38 We have also rarely managed lymphomas that arose in the sinuses and secondarily involved the orbit as shown in Figure 22–13.

Figure 22–14 shows a poorly differentiated sarcoma arising from a maxillary antrum and involving the orbit. This patient had a history of Wegener’s granulomatosis that was quiescent at that time.

Esthesioneuroblastoma is a variant of neuroblastoma that arises in the sinuses, most commonly in young men (Figure 16–4). Approximately 250 cases have been reported. In a series of 38 patients from the Mayo Clinic, 28 had ophthalmic signs at the time of presentation with 5 of 38 (13%) having ocular symp-

Figure 22–12. Nasopharyngeal carcinoma is more common in Asians. On direct coronal T1-weighted MRI scan, this lesion involved the sinuses and orbit.

toms, such as pain, proptosis, or epiphora as their initial complaint.39 In one case report, the initial presentation of this tumor was with unilateral blindness.40 Approximately 50 percent of patients survive.

Occasionally, an infectious process can simulate a sinus carcinoma with apical involvement.41 Figure 22–15 shows a clinical photograph and axial CT of an 80-year-old otherwise healthy woman with rapid

Figure 22–11. Apical involvement from an adenoid cystic carcinoma of the sinus. Fine-needle aspiration biopsy scout film demonstrates the needle in correct position.

Figure 22–13. Sinus lymphoma with secondary orbital involvement. Patient presented with an initial complaint of epiphora. On axial T1-weighted MRI scans, an ethmoidal mass was seen; on biopsy, this tumor was a large cell lymphoma.

onset of ophthalmoplegia and vision loss secondary to an apparent sinus carcinoma with secondary involvement of the orbital apex. At biopsy Aspergillus was found in the ethmoid and sphenoid sinuses, without underlying tumor.

Inverted sinus papillomas are uncommon benign tumors that account for < 5 percent of sinus neoplasms.42 Most commonly they occur in middle-aged males and are locally quite aggressive.43 Such a case is shown in Chapter 21. In a recent report of 10 cases that invaded the orbit, 8 had recurrences after their initial resection, and 3 had spread into the brain.44 Rarely, they can involve the orbit or metastasize.43–46 Incomplete excision usually results in a high recurrence rate, estimated between 30 and 70 percent.46

Cemento-ossifying fibromas of the ethmoid sinuses can involve either the pediatric or adult orbit.47 Juvenile nasopharyngeal angiofibromas usually occur in male teenagers and can cause death by either CNS extension or hemorrhage. These occasionally involve the orbit and may recur with or without radiation. It is uncertain whether surgery with embolization and/or radiation is more efficacious.48

Benign or malignant lymphoid lesions of the sinuses (see Figure 22–13) can involve the orbit.49 While orbital pseudotumor involving the sinuses is rare, it can occur.50 Wegener’s granulomatosis will often present with sinus disease and secondarily produce either ocular or orbital findings (Figure 22–16).51–54 Occasionally, the disease can be limited to just sinus and lung without renal involvement.54 Ophthalmic involvement has the same frequency in both the limited and complete forms of Wegener’s granulomatosis.55

B

C

Figure 22–15. A, Patient who presented with ophthalmoplegia, pain, and loss of vision due to aspergillosis of the ethmoid sinus. The clinical appearance is suggestive of a sinus carcinoma. B, Axial CT scan showing apical involvement with bone loss secondary to aspergillosis. This pattern simulated a sinus carcinoma with secondary spread. A similar case of an actual sinus carcinoma is shown in Figure 22–15C. C, Axial CT scan demonstrating sinus carcinoma with secondary orbital involvement.

396 TUMORS OF THE EYE AND OCULAR ADNEXA

Figure 22–16. Wegener’s granulomatosis with orbital involvement. ANCA tests were positive.

Historically, establishing the correct diagnosis in Wegener’s granulomatosis was difficult. In one series, only 7 of 13 orbital biopsies had the classic triad of vasculitis, granuloma, and necrosis.56 Occasionally, this disease can produce unusual ophthalmic findings, such as a pseudomelanoma or nasolacrimal duct obstruction. As discussed under orbital pseudotumor, the commercial availability of the antineutrophil cytoplasmic antibody (ANCA) test has made the diagnosis of Wegener’s granulomatosis much easier, even in the limited form of the disease.59 Most cases of ophthalmic involvement have had positive antibodies.

Midline lethal granuloma may clinically appear histologically similar to Wegener’s granulomatosis but it does not involve tissues below the neck.60 It is often difficult to differentiate histologically from other types of lymphoma. Another variant of this process is midline malignant reticulosis, a lymphoma often difficult to differentiate from a benign inflammatory process.60–63 An example of a midline lethal granuloma eventually diagnosed as a lymphoma is shown in Figure 22–17. Rhinoscleroma is another rare benign granulomatous disease. This is endemic in the developing countries but is quite rare in the United States.63

Occasionally, an osteoma arising from the sinus involves the orbit and produces proptosis, limitation of ductions, and pain.64 A patient with an orbital osteoma is shown in Figure 22–18A. Either a plain x-ray film (Figure 22–18B) or a CT scan (Figure 22–18C) is diagnostic.

Orbital osteoma was first described by Veiga in 1856.65 There are three theories of origin of osteomas: developmental, infection, or trauma.

Twelve cases of orbital osteomas were reported from the Mayo Clinic. Nine of the 12 had proptosis, and 3 had limitation of extraocular movement. There has been 1 case of amaurosis fugax as a result of a secondary osteoma.69 Occasionally, these tumors are seen in association with Gardner’s syndrome, an autosomal-dominant condition characterized by intestinal polyposis, skin and soft tissue tumors, and osteomas.70 In the 5 cases that the author managed, all presented with proptosis, ocular pain, and motil-

A

B

Figure 22–17. A, Clinical appearance of lethal midline granuloma of the orbit (Courtesy of D. Goodman, MD, San Francisco, CA). B, CT scan of patient shown in Figure 22–17A.

ity disturbances. The clinical pattern is not diagnostic, although usually, the eye is pushed laterally and downward (see Figure 22–18A). As pointed out by Ciappetta and co-workers, orbital involvement occurs in < 5 percent of cranial osteomas, and there is a higher incidence of surgical complications in this location.71

If removal of an osteoma is planned, CT with bone windows is useful. A coronal reformatted CT scan (see Figure 22–18C) shows that the tumor, which, on axial CT, appeared to arise from the medial orbital wall, actually arose in the area of the cribriform plate. The author removed this lesion with a dental burr (Figure 22–18D); lesions in less strategic areas can be broken off with a towel clip, or a dental burr may be used. Osteomas almost never recur or undergo malignant degeneration.

More recently, the use of computer assisted resection techniques using a three-dimensional CT technology can be an important adjuvant.72 While the author has used the former technique for sphenoid wing mengiomas, there has not yet been a need to use it for an osteoma. Unfortunately, in cases in which the surgeon is not familiar with orbital osteomas, significant morbidity can occur. The patient shown in Figure 22–19 had a large osteoma with a preoperative vision of 20/20 and a postoperative vision of light perception after an incomplete resection. I was able to remove the tumor and restore vision to 20/50, but with only a small central island of vision.

Finally, we have examined a few patients from the airline industry who presented with enophthalmos as a result of barotrauma when a cabin depres-

surized. Figures 22–20A and B show the normal (see Figure 22–20A) and imploded (see Figure 22–20B) maxillary antrums.

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