Ординатура / Офтальмология / Английские материалы / Ocular Pathology_6th edition_Yanoff, Sassani_2009
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Neoplasms and other tumors 581
2). The percentage of CDR+, CD7− cells is elevated in the peripheral blood of patients who have Sézary’s syndrome.
3). Although mycosis fungoides rarely involves the orbit, it does so more commonly than the other T-cell lymphomas.
Very rarely, mycosis fungoides can involve the vitreous.
4). Altered forms of the retrovirus HTLV-I and
HTLV-II have been incriminated in the causation of some cases of mycosis fungoides and Sézary’s syndrome.
E.Hodgkin’s disease‡
1.Hodgkin’s disease very rarely presents initially with orbital involvement, and orbital involvement is rare in any stage of the disease.
2.Hodgkin’s disease may be a direct consequence of a bcl-2 translocation through additional genomic events to be clarified in the future.
The bcl-2 oncogene acts mainly on the pathways of apoptosis (programmed death) and plays a crucial role in the control of cellular growth of lymphoid and nonlymphoid cells. Two other types of oncogenes are recognized: oncogenes such as myc, ras, and abl act as growth and proliferative regulatory genes; and oncogenes such as Rb and p53 inhibit growth and proliferation.
3.Di erentiation of Hodgkin’s disease from non-
Hodgkin’s lymphoma is based on finding Reed–
Sternberg cells in the former. Also helpful is the presence of markers for the Reed–Sternberg cells
[e.g., CD30 (Ki-1), and CD15], and the absence of
CD45.
In the absence of any Reed–Sternberg cells and the presence of CD30 positivity, the diagnosis of anaplastic Ki-1+
(CD30) large cell lymphoma should be considered. Four types of Ki-1+ large cell lymphoma may exist: common,
Hodgkin’s-related, giant cell-rich, and lymphohistiocytic. The tumor cells stain intensely positive with CD30. Approximately 70% of cases are of T-cell type, 15% are of B-cell type, 5% are mixed B- and T-cell types, and 10% are null type.
4.Epstein–Barr virus is found in a high percentage of cases of Hodgkin’s disease (see p. 63 in Chapter 3)
5.The origin of the neoplastic cells in Hodgkin’s disease remains an enigma in spite of the advances in immunology, cytogenetics, and molecular biology.
IX. Leukemia† (Fig. 14.47; see p. 353 in Chapter 9)
A.Orbital leukemic infiltrates most commonly occur late in the disease.
B.Occasionally, acute leukemia, usually granulocytic (myeloid or myelogenous) or stem cell, may present
initially with exophthalmos. Acute angle closure glaucoma has accompanied bilateral orbital infiltration with acute myeloid leukemia. Ultrasonography revealed uveoscleral thickening and anterior rotation of the ciliary body.
Rarely, the first sign of granulocytic leukemia relapse is ocular adnexal involvement. Occasionally, granulocytic sarcoma of the orbit has been reported as an isolated lesion.
1.The initial blood count may be normal or low with no circulating leukemic cells.
2.The bone marrow initially may be normal or hypocellular.
3.Exophthalmos is caused by an infiltrate of leukemic cells, called myeloid (granulocytic) sarcoma.
Because the pigment myeloperoxidase is sometimes present, the tumors appear greenish; hence the term chloroma.
The World Health Organization recommends the term myeloid sarcoma for a localized extramedullary tumor composed of immature myeloid cells; other terms are chloroma and granulocytic sarcoma.
4.Approximately two-thirds of patients who have myeloid leukemia present with some blurring of vision and accompanying fundus changes.
C.Histologically, leukemic cells infiltrate the orbit (see p. 353 in Chapter 9).
1.Auer rods (splinter-shaped, azurophilic, cytoplasmic crystalline inclusions) may be found in the blast cells of acute myeloid leukemia (see Fig.
14.47D).
2.The myeloid nature of the tumor is identified by chloroacetate esterase staining of the neoplastic cells.
The cells are CD43, CD45, Leder, and antilysozyme positive.
X.Monoclonal and polyclonal gammopathies‡
A.Monoclonal (single species of antibody) and polyclonal (multiple species of antibodies) macroglobulinemia may be seen in a variety of lymphoproliferative disorders such as nodular lymphoid hyperplasia, immunoblastic lymphadenopathy,nodular malignant lymphoma, and the plasma cell dyscrasias multiple myeloma, Waldenström’s macroglobulinemia, and the rare entities such as light-chain deposition disease and heavy-chain disease.
1.Monoclonal gammopathies are immunoglobulin products of single clones of plasma cells and B lymphocytes; polyclonal gammopathies are produced by more than one clone.
2.Most monoclonal gammopathies do not evolve into a malignant condition (all polyclonal gammopathies do not so evolve) and are termed monoclonal gammopathies of undetermined significance.
582 Ch. 14: Orbit
l |
l |
|
s
s 
A B
b 
b
r
r
C D
Fig. 14.47 Leukemia. A, A 9-year-old boy who presented with a left, painless exophthalmos died approximately 2 years after diagnosis. Initially, the work-up, including a complete blood count, showed normal results. Orbital biopsy was performed. B, Histologic section shows a diffuse cellular infiltratae of primitive granulocytic leukemic cells (l, large blast cells; s, small blast cells). C, Bone marrow smear shows blast cells (b, blast cells; r, red blood cells). Acute granulocytic leukemia diagnosed. D, A touch preparation of another case of granulocyte sarcoma shows Auer rods. (Case in
A–C reported by Brooks HW et al.: Arch Otolaryngol 100:304, 1974; D, courtesy of Dr. RC Eagle, Jr., who presented case to the meeting of the Verhoeff Society, 1994.)
B.Multiple myeloma‡ (Fig. 14.48)
1.Multiple myeloma shows evidence of bone marrow plasmacytosis, monoclonal gammopathy in serum or urine (Bence Jones protein), and lytic bone lesions.
2.Direct orbital infiltration by myeloma cells, mimicking a primary orbital tumor, is uncommon.
a.The orbital involvement, however, may be the initial manifestation of the systemic disease.
b.Solitary orbital extramedullary plasmocytoma is a rare tumor; fewer than 15 cases have been described.
Some cases show necrobiotic xanthogranuloma of the eyelid. The eyelids may also show characteristic hemorrhagic lesions (see Fig. 7.13).
C.Waldenström’s macroglobulinemia‡
1.Waldenström’s macroglobulinemia is a small B-cell lymphocytic lymphoma that produces monoclonal
IgM, a pentameric immunoglobulin of high molecular weight.
2.Clinical symptoms are mainly related to anemia, bleeding, or symptoms of hyperviscosity.
A progressive macroglobulinemia-associated retinopathy may develop with associated antibodies against the connecting cilia of the photoreceptors.
D.In vitro immunologic and immunohistochemical techniques can aid in the diagnosis of plasma cell infiltrates.
E.Corneal, and even iris, crystals can be found in some patients who have monoclonal gammopathy.
F.Intranuclear and intracytoplasmic inclusions may be found in plasma cells and lymphocytes.
1.Intranuclear inclusions (Dutcher bodies; see Fig. 14.43D) are PAS-positive collections of monoclonal macroglobulins.
Neoplasms and other tumors 583
Fig. 14.48 Multiple myeloma. A, Left exophthalmos present. B, Computed tomography scan shows mass (+) in orbital region. C, Immunoperoxidase-stained sections show many plasma cells with negative staining for λ light chains on left panel and positive staining for κ light chains on right. D, Electron microscopy shows abnormal plasma cells. (Case presented by Dr. MW Scroggs to the meeting of the Eastern Ophthalmic Pathology Society, 1989.)
Actually, the inclusions are cytoplasmic invaginations into the nucleus. In some cases, the inclusions show no PAS positivity.
2.Intracytoplasmic inclusions (Russell bodies; see Fig. 1.13, and p. 12 in Chapter 1) are PAS-positive collections of monoclonal or polyclonal macroglobulins.
Secondary Orbital Tumors
I.Direct extension†
A.Intraocular neoplasms, especially malignant melanoma and retinoblastoma
B.Eyelid neoplasms, especially basal cell carcinoma, squamous cell carcinoma, malignant melanoma, and sebaceous gland carcinoma
C.Conjunctival neoplasms, especially squamous cell carcinoma and malignant melanoma
D.Paranasal sinus cysts (mucoceles; Fig. 14.49) and neoplasms, especially squamous cell carcinoma, adenoid cystic carcinoma (malignant cylindroma), and mucoepidermoid carcinoma
E.Intracranial, especially meningioma II. Metastatic†
A.Neuroblastoma in children usually occurs as a late manifestation of the disease. Frequently, the orbital metastases are heralded by the onset of lower-lid ecchymosis (Fig. 14.50).
B.Lung carcinoma in adult men
C.Breast and lung carcinoma in adult women
In the past, breast carcinoma was much more common in women than lung carcinoma. With increased smoking by women, however, lung carcinoma has become much more common. Lung carcinoma tends to metastasize early, whereas breast metastases tend to be a late manifestation.
D.All other sites of primary neoplasms are rare.
584 Ch. 14: Orbit
A B
Fig. 14.49 Orbital mucocele. A, A 66-year-old man had left exophthalmos for 6 months and a 40-year history of sinusitis and a number of facial injuries. B, Histologic section of the surgically removed mucocele shows that the lumen is lined by respiratory-type, pseudostratified, ciliated columnar epithelium (shown with increased magnification in C) and contains scattered round inflammatory cells in its wall. (Courtesy of Dr. WR Green.)
C
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Fig. 14.50 Metastatic neuroblastoma. A, A 12-year-old child who had had abdominal neuroblastoma at age 4 years presented with sudden-onset ecchymosis of right lower lid. B, Computed tomography scan shows mass in right temporal fossa and erosion into sphenoid sinus and orbit. Histologic appearance similar to retinoblastoma in bone marrow (C) and orbit (D). (Case presented by Dr. E Torczynski at the meeting of the Eastern Opthalmic Society, 1994.)
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