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

and infectious cellulitis as well as lymphoma and metastatic carcinoma.

Advances in the management of WG over the past 20 years have improved the survival with this disease, which in its classic form is rapidly fatal if not treated.106 It is extremely important to establish the diagnosis of WG as early as possible, since early treatment may prevent renal failure, which is usually the cause of death. The mainstay of treatment is systemic immunosuppression with cytotoxic therapy; usually with the combination of corticosteroids and cyclophosphamide.103 Although definitive treatment of any ophthalmic involvement is systemic immunosuppression, orbital inflammation may respond poorly to systemic cytotoxic therapy and may remain active despite the remission of the systemic disease.103,107

Amyloid Deposits

Amyloid deposits in orbital inflammation are a common occurrence; however, a localized mass formation of “amyloid tumor” is a very uncommon disor- der.108–110 Involvement of the lacrimal gland with amyloid deposits may mimic localized orbital pseudotumor or a neoplasm (Figure 29.13).111 Clinically, these lesions present with painless proptosis. On imaging studies, the gland shows enlargement and molding to adjacent bones, frequently with punctate calcification. The appearance of calcification on the CT is a helpful feature, since the MRI findings are nonspecific. With MRI, amyloid deposits show hypointensity on T2-weighted images without any enhancement with contrast.110 Lacrimal gland tumors and extramedullary plasmacytoma may simulate amyloid formations.112,113

Plasmacytoma is a rare type of non-Hodgkins lymphoma that primarily attacks patients after the age of 40. Diagnosis is based on the documentation of clonal plasma cell proliferation. Immunohistochemical stains should be utilized to document the light chain types (Figure 29.14). These tumors may be confused with lymphoma, localized pseudotumor, SS, and epithelial tumors of the lacrimal gland. The management of plasmacytoma is combined treatment with radiation, surgery, and chemotherapy.114,115

Many other vasculitides and collagen tissue disorders rarely involve the orbit and occasionally may form localized masses. These include angiolymphoid hyperplasia with eosinophilia (Kimura disease),116–118 polyarteritis nodosa,119,120 Churg–Strauss syndrome,121–123 lupus erythematosus,124–127 Behçet disease,128,129 der- matomyositis,130–132 scleroderma,133–136 and rheumatoid arthritis.137–139 Differential diagnosis of these lesions from neoplasms is usually not difficult because the disorders present with other ocular and systemic manifestations and laboratory findings. Some of these entities were summarized in Table 29.2.

FIGURE 29.14. (A,B) Lateral view of the skull and coronal CT scan of the same patient, showing osteolytic lesions of localized plasmacytoma. The patient presented with rapidly developing proptosis and an inferior dislocation of the left eye. (C) Biopsy of the mass revealed extensive proliferation of atypical plasma cells, which were stained with light chains.

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Surgery is the most common modality of tumor treatment in the orbit, whether it is performed for total excision or for debulking or biopsy pur-

poses.1–4 Even in today’s multimodality cancer treatment milieu, surgery remains very effective not only for diagnosis but also for treatment.4,5 Surgical treatment is most effective in benign lesions and/or welldelineated malignant lesions. It also provides extremely useful initial information about the nature of the tumor as well as about staging, an appropriate management plan, and prognosis. In many orbital tumors, such as rhabdomyosarcoma, lymphoma, and secondary and metastatic tumors, radiation therapy alone or in combination with chemotherapy offers promising results. However, it is extremely rare that an orbital tumor patient’s care does not include an initial surgical component.

Whether the objective of the therapy is cure or palliation, the management plan depends on the specific typing and staging of the neoplasm.6,7 If the cancer is considered to be localized without evidence of spread, the tumor can be excised and the patient is cured.8 If the cancer has spread beyond local cure, the objective is to control symptoms and maintain the maximum function of the eye and the adnexa with the best quality of life for as long as possible. Patients are generally considered to be incurable if they have distant metastasis or evidence of diffuse extension of the tumor into the cranium.2

The selection of the single or multimodality therapy depends on the type and the extent of the neoplasm, which is determined by staging.1,2 For example, in general, if a neoplasm is limited to the orbit without extension into the globe, cranial cavity, and/or regional lymph nodes, the malignancy is considered curable by surgery and/or radiation. Extension of a given orbital tumor into neighboring structures including the globe, optic nerve, nose and perinasal sinuses, central nervous system, or regional lymph nodes is of great clinical importance; these features affect the staging and, therefore, the treatment of the tumor.

Three significant events determine the biological behavior of a malignant neoplasm: local tumor (T)

growth, extension to lymph nodes (N), and distant metastasis (M).6,9 These events determine the anatomic extent of the malignancy, which is more commonly known as the stage of the disease at a given time in its progression. Therefore, the letters T, N, and M represent the indicators of prognosis of the patient harboring a particular type of tumor. The type of tumor as well as the staging are determined by histopathologic examination of tissues. The TNM classification by stage grouping is possible only when based on examination of surgically excised specimens from the primary tumor, from its margins, from the regional lymph node, and from distant metastatic sites.

Clinical experience has proven that the definition of the anatomic staging varies depending on the histologic type and the anatomic site of origin. Therefore, the American Joint Committee on Cancer (AJCC) utilizes the TNM classification scheme for particular tumors of each anatomic site (Table 30.1).9 The system is intended to provide a common language by which patient information can be shared among physicians to establish therapeutic plans and to estimate prognosis. The long-range benefit of the staging system is that it provides easy comparison of similar groups of patients when therapeutic regimens are evaluated.

In addition to the TNM classification, the histopathologic type and the grade of a given neoplasm are important therapeutic and prognostic determinants (Box 30.1). Numerical subsets that may be used for each letter, such as, T0, T1, N2, M1, etc., indicate the progression of the malignant disease, creating a shorthand notation for describing the clinical and histopathologic extent of a given malignancy. Four classifications are designed for each anatomic site:

1.Clinical classification (cTNM)

2.Pathologic classification (pTNM)

3.Retreatment classification (rTNM)

4.Autopsy classification (aTNM)

Clinical (c) classification is based on physical findings, imaging, endoscopy, and biopsy. The clinical staging is essential to make therapeutic decisions and evaluate the patient’s response to that treatment.

TABLE 30.1. Definitions of TNM.

Primary tumor (T)

BOX 30.1. Summary of Descriptors

Utilized in TNM Classification

G: Histopathology grade (GX, G1–G4)

L: Lymphatic vessel invasion (LX, L0, L1) V: Venous invasion (VX, V0, V1, V2)

R: Residual tumor (RX, R0, R1, R2)

m suffix: Presence of multiple primary tumors [e.g., pT(m)NM]

y prefix: Patients classified during or following initial therapy (e.g., ycTNM)

r prefix: Recurrent tumor after following diseasefree interval (e.g., rTNM)

Histopathology grade (G) is expressed with numbers and represents the qualitative assessment of the differentiation of a given neoplasm. It ranges from most differentiated (G1) to least differentiated (G4); GX stands for a grade that cannot be assessed.

Pathologic (p) classification is based on histopathologic examination of the tissues removed by biopsy or exploratory surgery. Histopathologic evaluation provides additional data to make a management plan and estimate the prognosis.

Retreatment (r) classification is helpful to provide

further management plan for a neoplasm that recurs after a disease-free interval.

Autopsy (a) classification is done in the staging of a given neoplasm not evident prior to death that is discovered by postmortem examination.

Recently, the AJCC upgraded the TNM classification for cancers of all types, including orbital neoplasms. During this upgrading of the system, the staging forms for each anatomic site were modified. The following staging forms for the malignancies of the orbit are borrowed from the current (2002) manual of the AJCC, with the written consent of the committee.

Acknowledgment Staging forms are used with the permission of the American Joint Committee on Cancer (AJCC), Chicago. The authors are grateful to AJCC and Dr. James C. Fleming, who was primarily instrumental in the development of the staging forms. The original source for this material is the AJCC Cancer Staging Manual (6th edition, 2002, published by Springer-Verlag New York, Inc., New York).

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