Ординатура / Офтальмология / Английские материалы / Orbital Tumors Diagnosis and Treatment_Karcioglu_2005

The term neoplasia literally means “new growth,” and the tissue that results from this process is called a neoplasm. The word tumor originally

designated the edema caused by inflammation but is now widely used as a synonym for neoplasm.

Although patients understand that a tumor is an abnormal and harmful lesion, a precise medical definition of what characterizes a neoplasm is difficult. According to Cotran et al., a neoplasm is an abnormal mass of tissue whose growth is uncoordinated with that of the normal tissues; the mass is purposeless and preys on the host.1 From the functional standpoint, neoplasms can be divided into two basic categories: benign and malignant. Rapid growth, local invasion, and metastasis are typical characteristics of malignancy. Since most cancers grow rapidly with infiltration and destruction of the surrounding tissues, most clinicians intuitively consider local invasiveness to be the hallmark of neoplasms.

Inflammation is a complex tissue reaction that occurs in response to specific endogenous or exogenous stimuli. The inflammatory process is characterized by increased vascular permeability that allows plasma proteins and circulating cells to leave the circulation and infiltrate the injured area. Depending on the nature of the stimulus that initiates the inflammation, the cells involved in the inflammatory process will change. Infection, for instance, is characterized by a predominance of neutrophils and polymorphonuclear leukocytes. Other infiltrates are formed by collection of epithelioid histiocytes and granuloma formation or polyclonal lymphocytes. Sometimes the cell contingent is sparse and the tissue changes are dominated by fibrous proliferation. The ultimate goal of the inflammatory reaction is to protect the organism from the initial cause of injury. However, the process of tissue repair that is closely related to inflammation can be harmful, and severe sequelae may be the final result of the inflammatory response.

Although neoplasia and inflammation are different biological processes, the clinical differentiation between the two can be difficult in the orbit. Neoplasia can induce inflammation, and some inflammatory responses may mimic malignant neoplasms.

INFLAMMATION

MIMICKING NEOPLASIA

Idiopathic orbital inflammation (IOI) is a condition of unknown etiology characterized by the involvement of the orbital tissues by a nonspecific inflammatory process. Histologically, this infiltration is composed of lymphocytes, plasma cells, eosinophils, macrophages, and varying amounts of collagen deposition.2 By definition, IOI is restricted to the orbital contents, and several patterns of tissue involvement have been described.3 However, the same types of inflammatory change found in the orbit have been reported in other locations of the head and neck region, including larynx, mouth, tonsils, paranasal sinuses, pterygomaxillary fossa, thyroid, and parotid.4

Whenever there is inflammation within the boundaries of the orbit, it is not uncommon to find inflammatory changes outside the orbit as well. Table 27.1, which summarizes the clinical characteristics of 11 cases of idiopathic inflammation diagnosed around the orbits, indicates that 53.8% of the inflammatory changes are found simultaneously inside and outside the orbital limits, and bone erosion was a common finding (69.2%).5–11 From the clinical point of view, bone erosion is a disturbing feature because it indicates an aggressive lesion with high capacity of local invasion and destruction, typical of neoplasias. Figure 27.1 shows how similar to neoplasia an idiopathic inflammation can be: the patient, a 35-year-old man, presented with a 1-month history of proptosis, diplopia, and pain in the right eye (OD). Visual acuity was normal. Hertel exophthalmometry measurements were OD 19 mm, and for the left eye (OS), 14 mm with elevation and marked inferior restriction of the right eye (Figure 27.1A). A computed tomography (CT) scan of the orbits showed an extensive infiltration along the floor involving the inferior, lateral, and medial recti. The intraconal fat between the optic nerve and the inferior rectus was also affected. The infiltration extended outside the orbit into the pterygopalatine and infratemporal fossae. The lateral wall was partially destroyed (Figure 27.1C,D). The results of the biopsy revealed mature lymphocytes associated with dense col-

lagen deposition (Figure 27.1E,F). The patient was treated with radiation (2000 cGy) and 1 mg/kg/day of prednisone for one month. The steroid therapy was maintained for an additional month and tapered over 8 weeks. There was a gradual improvement in ocular motility and relief of the symptoms (Figure 27.1G,H). The patient’s clinical picture was indistinguishable from a malignant neoplasm of the paranasal sinuses and the pterygopalatine and infratemporal fossae; a correct diagnosis was possible only after a biopsy.

Large areas of infiltration beyond orbital limits are also suspect and can easily lead the physician to the conclusion that the orbit is being invaded by a malignant process. The patient shown in Figure 27.2A presented with a 5-month history of diplopia, pain, and proptosis in the right eye. On examination, a painful mass was palpable in the right temporal fossa. Visual acuity for both eyes was 20/40; ocular fundi and visual fields were normal. Hertel measurements were 20 mm (OD) and 13 mm (OS) with a significant limitation of right abduction. Computed tomography and magnetic resonance imaging (MRI) of the orbits showed a large mass infiltrating soft tissue structures well beyond the orbital limits. As shown in Figure 27.2B,C, the infratemporal and temporal fossae as well the lateral aspect of the right orbit were extensively involved. The infiltration was into the lateral and inferior recti muscles but did not cause bone erosion. Biopsy samples from intraorbital and temporal fossa contents showed the same histology: a chronic inflammatory process composed mainly of mature lymphocytes associated with collagen deposition, and occasional lymphoid follicles. The patient was treated with prednisone (1 mg/kg/day) tapered over 4 weeks. The clinical response to treatment was good, with pain reduction and improvement of ocular motility. A repeat CT scan showed that infiltration was less dense (Figure 27.2D).

Orbital myositis is a subgroup of IOI characterized by inflammation of the extraocular muscles (EOMs).12

Lacey et al., in their comprehensive review of EOM disease, classified orbital myositis into acute, chronic or recurrent, and atypical forms.13 The isolated form is characterized by a short history (days to weeks) of pain, swelling, and/or diplopia. Pain exacerbated by eye movement is the most common clinical presentation (62.5%). The recurrent form (32%) is typified by repeated acute episodes; less frequently, it has a progressive course. Rarely (5%), patients may present with features such as optic nerve dysfunction and minimal pain in atypical cases.

The differential diagnosis of myositis includes other causes of specific EOM inflammation such as sarcoidosis,14–16 systemic lupus erythematosus,17,18 rheumatoid arthritis,19 Wegener’s granulomatosis,20 scleroderma,21 infections (trichinosis, cysticercosis, Lyme disease),22,33 Crohn’s disease, celiac disease, Wipple’s disease,13,24 and neoplasias such as lymphoma25 and metastatic tumors.26

The diagnosis of myositis can be straightforward. On imaging, increased EOM with muscle tendon thickening is a common finding. Orbital fat is generally not infiltrated. Because orbital myositis is usually localized to the extraocular muscles, the coexistence of myositis and infiltration of other structures not related to the EDMs creates a diagnostic dilemma that is resolved only with a biopsy. The patient described next exemplifies a nonspecific myositis with atypical findings, similar to neoplasia.

A 66-year-old woman presented with redness, foreign body sensation, and proptosis in the right eye. She had a hard nodule in the lateral aspect of the right upper lid of 3-month duration that was painful to palpation. She also complained of diplopia in right and superior gazes. Extraocular motility was decreased in abduction and infraduction of the right eye. Pupillary examination was within normal limits. Intraocular pressures were 15 mm OD and 13 mm OS. Biomicroscopy revealed a mass in the superior conjunctival fornix of the right upper lid. Arteriolar attenuation and

FIGURE 27.1. (A) The right eye is proptotic and displaced upward.

(B) Marked restriction on downgaze. (C) Axial CT scan showing extensive infiltration along the floor involving the inferior, lateral, and medial recti. (D) Axial CT scan showing infiltration of the infratemporal fossa with bone destruction. (E) Dense collagen depo-

sition replacing orbital fat. Sparse mature lymphocytes can be seen among the collagen bundles (hematoxylin and eosin, original magnification 40). (F,G) Improvement on the ocular motility and relief of the symptoms after anti-inflammatory therapy.43

mild tortuosity of the vessels were the only significant findings on funduscopy. Hertel exophthalmometry revealed 21 mm OD and 14 mm OS.

A CT scan showed diffuse enlargement of the right lateral rectus muscle with a hyperdense lesion. The op-

tic nerve was dislocated to the right but normal in appearance, and the right infraorbital nerve was somewhat enlarged at the level of the pterygopalatine fossa (Figure 27.3B). Thyroid function tests were normal. C-reactive protein, anti-streptolysin titers, and results of a com-

FIGURE 27.2. (A) A 56-year-old woman who had diplopia, pain, and proptosis in the right eye. (B) Coronal CT scan showing infiltration involving the lateral and inferior recti and temporal fossa (arrow) without bone erosion. (C) Coronal CT scan showing infil-

tration in the infratemporal fossa (arrow). (D) Coronal CT scan after anti-inflammatory therapy. The infiltration in temporal and infratemporal fossa disappeared. The inferior and lateral recti are still enlarged.43

plete blood cell count were normal; erythrocyte sedimentation rate was 22. Results were negative for antinuclear factor and antineutrophil cytoplasm antibody.

A diagnosis of myositis of the lateral rectus was made, and treatment was initiated with three daily oral doses of prednisone totaling 1 mg/kg/day and tapered over 3 weeks. The nodule in the right upper lid improved after just a few days of therapy. However, there was no change in diplopia or motility restriction. There was only 1 mm of residual proptosis of the right globe from her initial presentation after 4 months. The patient presented again 2 years later with a firm nodule in the right lower eyelid. Visual acuity was 20/25 with correction. Goldmann visual fields were normal in both eyes. A CT scan at this time showed enlargement of medial, lateral, and inferior recti, with the latter two being most involved (Figure 27.3C,D). The enlargement of the infraorbital nerve had significantly increased. Prednisone was again given with a good response and reduction in proptosis.

The patient was lost to subsequent follow-up for 4 years. When she presented again there was about 6 to 7 mm proptosis of the right globe with a pattern of

motility restriction similar to the one she had at presentation, as well as a firm nodule in the right lower eyelid. She had been self-medicating herself with 20 mg/day of prednisone for 2 months. A CT scan performed at this time showed involvement of all extraocular muscles including the levator–superior rectus complex. There was enlargement of the infraorbital canal circumferentially from the pterygopalatine fossa to about midway along the floor of the orbit (Figure 27.3C).

The patient was again treated empirically with high doses of oral prednisone during the next 2 years, with good results. An MRI scan was also performed 3 years after treatment, which showed diffuse enlargement of all rectus muscles. The right infraorbital nerve was markedly enlarged posteriorly, near its origin and along the pterygopalatine fossa (Figure 27.3E,F).

Biopsy of the inferior oblique muscle and the infraorbital canal was performed. The infraorbital canal was so enlarged that there was no difficulty in obtaining a piece of the nerve, which showed a nonspecific chronic inflammatory infiltrate with follicle formation interspersed among the nerve

FIGURE 27.3. (A) Axial CT scan showing diffuse infiltration of the right lateral rectus muscle. The optic nerve is moved medially. (B) Axial CT scan showing enlargement of the right infraorbital nerve at the level of the pterygopalatine fossa (arrow). (C) Coronal CT scan showing enlargement of the right medial, lateral, and inferior recti muscles as well as of the right infraorbital nerve. (D) Axial CT scan: significant increment of the infraorbital nerve enlargement (arrow).

(E) T1-weighted, contrast-enhanced MR image showing enlarge-

ment of all extraocular muscles and of the infraorbital nerve. (F) Unenhanced T2-weighted MR image showing the diffuse enlargement of the right infraorbital nerve and also reveals that the size of the left infraorbital nerve is increased in the pterygopalatine fossa.

(G) Histopathology from biopsy of right inferior oblique muscle: lymphoid follicle in a dense collagen matrix (hematoxylin and eosin, original magnification 1000).44

bundles. The muscle biopsy showed similar histology with a greater degree of fibrosis (Figure 27.3G). Immunohistochemistry revealed this infiltrate to be polyclonal and showed B and in lymphocytes as well as T lymphocytes.

NEOPLASIA MIMICKING

INFLAMMATION

Patients with typical inflammatory signs may prove to be suffering from orbital neoplasia. A classic ex-

ample is rhabdomysarcoma, which grows rapidly and frequently presents with infection and swelling of the eyelids. Usually, imaging and histopathologic studies disclose the true nature of these pseudoinflammatory features, and a correct diagnosis can be suggested, but the ultimate diagnosis should always be based on histopathologic examination.

In rare instances, the malignancy of an acutely inflamed orbit can be elusive and extremely difficult to prove. For instance, in childhood the classic signs of orbital inflammation such as proptosis, eyelid edema and erythema, pain, limitation of extraocular movements, and loss of vision are most often related to cellulitis due to sinusitis.27 Less frequently, this clinical picture can be the expression of IOI or represent extremely rare cases of Tor T/natural killer cell (T/NK) sinonasal lymphomas.28 The case presented in Figure 27.4 highlights the difficulty of diagnosing this type of lymphoma.

The patient, an 11-year-old boy, was admitted with a 2-month history of right ethmoid–maxillary sinusi-

tis and cellulitis of the right eye. The patient had initially been managed at another hospital with intravenous antibiotics and sinus drainage without success. During the 2 weeks before admission to our hospital, the proptosis of the right eye worsened and the child lost his vision in both eyes. He presented with proptosis, eyelid edema and erythema, limitation of eye movement, and excruciating pain on the right side of the face (Figure 27.4A). There was bilateral mydriasis and no light perception in either eye. Fundoscopy was unremarkable. The boy had fever (38°C) and was extremely anxious. Orbital CT scans from his initial treatment were available and showed opacification of the right maxillary and ethmoid sinuses and progressive right EOM (Figure 27.4B). The initial CT scan after admission showed massive right extraocular muscle infiltration; EOMs were almost indistinct. There was also an infiltrate in the frontal lobe (Figure 27.4C). Biopsies were done on the right orbit and the inferior turbinate immediately. Histopathologic examination was reported to be consistent with IOI. Cultures for

bacteria and fungi were negative. Since the clinical picture was not consistent with IOI, another biopsy was performed, but the same results were obtained. Only on the third biopsy, tissue samples from the medial rectus muscle, did orbital soft tissue and maxillary sinus show extensive tissue necrosis and an infiltrate of atypical cells, with pleomorphic nuclei and no architectural pattern permeating the nonnecrotic areas and the walls of blood vessels. The immunohistochemical study showed that these cells were positive for leukocyte common antigen (CD45), terminal deoxynucleotidyltransferase (TdT), CD3, and CD45RO, and negative for CD56, CD20, CD57, CD99, and Epstein–Barr virus. Both the morphologic and the immunohistochemical findings supported the diagnosis of T-cell lymphoma.

During the investigation period the disease progressed rapidly with development of scalp and palatal ulcers, fever, and deterioration of patient’s consciousness. Blood cultures and lumbar puncture results were negative. MRI disclosed a frontal lobe area of edema and diffuse meningeal infiltration. Chemotherapy was instituted, but the child’s status continued to deteriorate and he died of respiratory insufficiency.

It is interesting to note three biopsies were necessary to demonstrate the malignant cells. The scarcity of neoplastic cells in the biopsies is a typical finding in T or T/NK sinonasal lymphomas.29 It is well to remember that the biopsy should be repeated if results are not consistent with the clinical picture. In sinonasal T/NK-cell lymphomas, the cytologic atypia is quite variable, with cells of different sizes. The immunophenotype is positive for T-cell markers (CD2, CD3, CD5, CD45RO) and/ or NK-cell markers, especially CD56. In almost all cases, Epstein–Barr virus infection can be demonstrated by in situ hybridization. The difficulty in recognizing such cases as malignancies is exemplified by the fact that only recently has this entity been delineated as a lymphoma. In the past, the clinical presentation characterized by palatal ulcers, orbital swelling, and erythema was described as “lethal midline granuloma,” “idiopathic midline destructive disease,” “malignant granuloma,” “midline malignant reticulosis,” “rhinitis gangrenosa progressiva,” and “polymorphous reticulo- sis.”30–34 Because this neoplasm typically shows an angiocentric and angiodestructive pattern of growth, usually there is extensive necrosis admixture with inflammatory cells. In this context, identification of atypical malignant cells can be difficult, especially because these cells tend to be scarce and the diagnosis of lymphoma is seldom suspected clinically. In addition, surface crusting and secondary changes due to local infection explain the need for abundant biopsy material to establish a correct diagnosis. With respect to the patient discussed in

connection with Figure 27.4, positivity for T-cell markers (CD3, CD45RO) and negativity of CD56 confirmed that the case was a pure T-cell lymphoma.

“MALIGNANT” INFECTIONS

Most patients interpret cancer as an extremely harmful disease that carries a somber prognosis; a rapidly fatal course is widely seen as a hallmark of such an illness. However, some infections, even in our era of antibiotic eras, can behave like aggressive tumors and destroy the host at an astonishing pace. Sino-orbital mucormycosis is an example of an infection that can mimic an aggressive cancer. This type of infection is caused by fungi that belong to the class Zygomycetes, order Mucorales. The genera that account for most cases of mucormycosis (Rhizopus, Absidia, Rhizomucor, Mucor, and Apophysomyces) belong to the Mucoraceae family. Agents from other families of the Mucorales order (Cunninghamellaceae, Saksenaceae, Syncephalastraceae, and Thamnidiaceae) have also been implicated in this disease.25

These fungi are widely distributed in nature and are harmless in immunocompetent individuals. The disease represents an opportunistic infection that occurs in debilitated patients, especially those with diabetes and ketoacidosis.36 Spores inhaled from the air may invade and grow in the paranasal sinuses. From this site, hyphae invade the blood vessels, provoking thrombosis and necrosis. Spread of the organisms to the orbit, face, and brain gives rise to rhino-facial-or- bital-cerebral mucormycosis. The clinical course of the disease may range from rare chronic and indolent presentations37 to fulminant fatal forms.38 These polar extremes of the presentation spectrum of mucormycosis are illustrated in two cases presented next.

The first patient was a 65-year-old male who was seen at another hospital with a history of pain in the infraorbital region of the left eye. He had presented with edema on the left side of his face that appeared to be paretic. A CT scan demonstrated left ethmoid and maxillary sinus opacification. Serum glucose was 307 mg. The following day the local physician noted that the left side of the patient’s face was clearly paretic and erythematous. A new CT scan revealed that the sinus opacification extended into the right ethmoid sinus and nasal cavity. Ceftriaxone was prescribed, but on the third day of admission the patient’s status deteriorated. The left soft palate was paretic, and there was left ptosis with mydriasis in both eyes. An otolaryngologic examination disclosed an area of necrosis in the nasal mucosa, and a biopsy sample was taken. Histopathologic examination of this material revealed the presence of nonseptate hyphae typical of mucormycosis. Intravenous amphotericin B was ini-

FIGURE 27.5. Extensive necrosis involving almost the entire left side of patient’s face.

tiated. The following day the patient developed respiratory distress, and the entire left side of his face became necrotic (Figure 27.5). An aggressive surgical debridement was performed without success, and the patient died one day after surgery.

The clinical presentation of the next patient with rhino-orbital mucormycosis was completely different. The course of the infection was chronic and difficult to eradicate. The patient was a 70-year-old diabetic male who had a history of 10-day treatment for cellulitis at another hospital. Twenty-five days after discharge he developed ophthalmoplegia and lost vision in his left eye. On admission to our hospital, visual acuity was 20/30 in the right eye, but there was no light perception in the left eye, which showed proptotic edema and erythema in the upper eyelid. There was a large amount of secretion in the nasal cavity. The middle turbinate was necrotic. An orbital CT scan disclosed opacification of the ethmoid and sphenoid sinuses and an area of atypical infiltration along the medial wall (Figure 27.6A). The orbit and the sinuses

were simultaneously operated. A combined endoscopic and transconjunctival approach was employed. A large amount of necrotic tissue was removed from both the sinuses and the orbit. Histopathologic examination of these samples revealed the nonseptate hyphae characteristic of mucormycosis. Four days after surgery the sinuses were clean and the patient was transferred to the infectious disease unit. After 2 months of treatment with amphotericin B, a new CT scan revealed an infiltration in the pterygopalatine fossa with bone destruction of the lateral wall of the maxillary sinus and erosion of the deep portions of the lateral wall of the orbit (greater wing of the sphenoid bone) (Figure 27.6B). Exenteration, plus a combined transcranial approach to the sphenoid bone, was performed. Six months after the initial presentation, the patient was being carefully followed, with no certainty that the infection was under control.

These two cases illustrate different presentations of orbital mucormycosis. In the first case, the fulminating course of the disease is an example of aggressiveness rarely encountered in cancers. The second case, in contrast, shows a disease that tends to be locally invasive with an indolent but progressive destructive pattern.

In a previously healthy child, particularly between the ages of 1 and 7 years, rapidly developing proptosis and eyelid edema, often mimicking orbital cellulitis, should always arouse suspicion of rhabdomyosarcoma, which needs to be ruled out by biopsy results. However, the masquerade syndrome may present in reversed order: namely, a child who presents with signs and symptoms of rhabdomyosarcoma may be proven by biopsy results to have infectious etiology. The boy shown in Figure 27.7 provides a good example of the reverse masquerade syndrome. This patient presented with a rapidly enlarging, well-delineated mass in the left orbit, which was worked up with the clinical diagnosis of rhabdomyosarcoma; on biopsy, however, the condition proved to be mucormycosis.

FIGURE 27.7. (A) Eyelid edema and proptosis secondary to rapidly developing medial orbital mass causing bone erosion. (B) Axial scan showing bone erosion due to orbital mass.

The upper respiratory tract involvement of mucormycosis was not obvious at the time of admission, but sinus lesions and pulmonary involvement developed after the biopsy, causing death, despite exenteration and amphotericin B treatment.

Another inflammatory condition that may mimic invasive neoplasm is the allergic fungal disease of the nose and paranasal sinuses (Table 27.2).39,40 In this disease, the infection starts in the nasal cavity or in the lumen of a paranasal sinus and may extend into the orbit. In most instances, an Aspergillus species is the causative organism; other fungal species, including Fusarium and Rhizomucor, have also been incrimi-

nated.41 Although this entity is considered to be confined to the lumina, without mucosal and submucosal invasion, it nevertheless is known to spread from one paranasal sinus cavity to the other and to the orbit.42 Imaging studies show bony expansion and remodeling of the involved cavity and focal bony erosion. The mucoid content of the paranasal sinuses mixed with fungus balls produce low signal intensity in MR images. The extensive bony expansion and irregular remodeling, coupled with bony erosion, may simulate an invasive tumor of the nose or the sinus, with secondary orbital invasion, such as esthesioneuroblastoma or leukemia/lymphoma.

The 9-year-old girl shown in Figure 27.8A presented with right-sided proptosis and diplopia; her visual acuity was 20/40 OD, 20/20 OS. She had an initial working diagnosis of a malignant tumor, based on clinical findings. Orbital CT revealed high attenuation within the maxillary ethmoid and the sphenoid sinus. Bone destruction suggested an invasive tumor, but the diffuse nature of the process in paranasal sinuses, coupled with eosinophilia and elevated IgE levels, brought up the possibility of allergic fungal sinusitis. During ethmoidectomy/ medial orbitotomy, extensively necrotic, whitishgray, toothpastelike material was removed from the sinuses. The histopathologic examination of this material revealed a mixture of necrotic mucosal cells, eosinophils, and clusters of septate-fungal hyphae. Fungal cultures revealed the causative organism as Aspergillus flavus. The patient’s orbital symptoms were resolved, and the visual acuity went back to normal after treatment with surgical debridement and systemic corticosteroids.

CONCLUSIONS

Inflammation and neoplasia may be extremely difficult to differentiate in the orbit. In some cases, a correct diagnosis is made only after consideration of the whole context of the pathology, including clinical presentation, pattern of disease evolution, imaging studies, and histopathologic examination.

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FIGURE 27.8. (A) A 9-year-old girl whose right eye showed inferior lateral proptosis. (B) Axial scan shows enlargement and remodeling of right ethmoidal sinus; bone erosion and orbital invasion are seen medially. Biopsy results revealed numerous eosinophils

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