FNA Cytology of Ophthalmic Tumors

C. V. Biscotti, A. D. Singh, C.V., Biscotti, A.D., Singh, Philippe Vielh, Philippe, Vielh

• Publisher: S. Karger
• Year: 2011
• Print ISBN:
  9783805598705
• eBook ISBN:
  9783805598712
• Topic: Medicine
• Subtopic: Medical Theory, Practice & Reference

This comprehensive and well-illustrated volume in the outstanding Monographs in Clinical Cytology series examines the spectrum of ophthalmic cytology including uveal, retinal, and orbital tumors. It includes historical perspectives, current indications, surgical techniques, and practical aspects of the cytological preparation of ophthalmic fine needle aspiration samples. The importance of a close collaboration between cytologists and ophthalmologists in the field of ophthalmic tumors is emphasized. The monograph presents high-quality illustrations accompanied by very informative legends that reveal the details of the clinical-cytological correlation evident in many of the cases managed by the authors. It presents a complete spectrum of ophthalmic tumors covering uveal melanoma, uveal metastasis, intraocular lymphoma, and orbital tumors. Recent innovations in the instrumentation and use of digital cytology in fine needle aspiration biopsy are also reviewed. This publication is an excellent reference text on rare tumors. It is highly recommended reading for ophthalmic pathologists, cytologists, and oncologists.

Chapter 4

Biscotti CV, Singh AD (eds): FNA Cytology of Ophthalmic Tumors.
Monogr Clin Cytol. Basel, Karger 2012, vol 21, pp 31-43

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Uveal Lymphoma

Deborah J. Chute^a · Charles V. Biscotti^a · Arun D. Singh^b

^aDepartments of Anatomic Pathology and ^bOphthalmology, Cleveland Clinic Foundation, Cleveland, Ohio, USA

Primary uveal lymphoma is rare and, unlike the vitreoretinal lymphomas, is typically indolent in nature [1-3]. In the past these tumors were termed ‘reactive lymphoid hyperplasia’ or ‘uveal pseudotumor’ because of their low-grade appearance [4-6]. However, convincing evidence with modern techniques has demonstrated that the majority of these lesions are low-grade B-cell lymphomas, most commonly extranodal marginal zone B-cell lymphomas [1, 5, 7] according to the current WHO classification (table 1) [8]. Primary high-grade lymphomas involving the uvea are extremely rare [26]. Uveal lymphoma may be considered primary if the uvea is the only or the initial site and secondary if there is secondary involvement of the uvea by systemic non-Hodgkin lymphoma [13, 27-34]. Hence, a careful clinical evaluation for involvement of other sites is required at the time of initial diagnosis and subsequent follow-up.

Etiology/Pathogenesis

The uvea does not contain normal lymphoid tissue, although this is not required for the development of an extranodal marginal zone B-cell lymphoma. The etiology of primary uveal lymphomas remains unknown.

Clinical Features

Choroidal lymphoma is typically unilateral, occurs more frequently in men than in women, and occurs more often in the 50-60s [1-3]. The most common presentation is recurrent, painless blurred vision and metamorphopsia due to retinal detachment. Other signs can include increased intraocular pressure, which can become painful. Proptosis can occur when extraocular involvement occurs, and if extending into the subconjunctival or episcleral regions, ‘salmon patches’ may be present [1, 2, 35-38].

On funduscopic examination, multifocal, yellow-pink choroidal infiltration occurs early (fig. 1a), followed by diffuse thickening of the uveal tract in late disease (fig. 1b). The vitreous fluid typically remains clear. Ancillary studies such as fluorescein angiography, indocyanine angiography (fig. 1c), optical coherence tomography (fig. 1d) and ultra-sonography (fig. 1e) are essential in detecting subclinical involvement.

The differential diagnosis of primary uveal lymphoma includes both benign and malignant conditions such as choroidal hemangioma, posterior scleritis, uveal effusion syndrome, sarcoidosis and infectious agents, while metastasis and amelanotic melanoma must also be excluded [2, 3, 35, 37, 39-42].

Diagnostic Evaluation

Technique

Obtaining adequate, viable tissue for the diagnosis of primary uveal lymphoma can be difficult. The task becomes easy if concomitant adnexal involvement is detected (fig. 1e). Many different techniques are available, including transvitreal fine needle aspiration (FNA) and transscleral FNA. Chorioretinal biopsy is another option for obtaining tissue for analysis [Chapter 1, this vol., pp. 1-9] [43-45].

Table 1. The current WHO classification of lymphomas [8]

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Hodgkin lymphoma

Nodular lymphocyte-predominant Hodgkin lymphoma

Classical Hodgkin lymphoma

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Non-Hodgkin lymphomas

B-cell lymphomas

Small cell lymphomas

Chronic lymphocytic leukemia/small lymphocytic

lymphoma¹ [9, 10]

Extranodal marginal zone lymphoma² [1]

Follicular lymphoma¹ [11, 12]

Hairy cell leukemia

Lymphoplasmacytic lymphoma

Mantle cell lymphoma¹ [13, 14]

Nodal marginal zone lymphoma

Splenic marginal zone lymphoma

Large cell lymphomas

Burkitt lymphoma¹ [15-17]

Diffuse large B-cell lymphoma² [3, 18]

Intravascular large B-cell lymphoma¹ [19]

Lymphomatoid granulomatosis

Mediastinal (thymic) large B-cell lymphoma

Primary effusion lymphoma

Plasma cell neoplasms¹ [20, 21]

T-cell lymphomas

Adult T-cell leukemia/lymphoma¹ [22, 23]

Aggressive NK cell leukemia

Anaplastic large cell lymphoma¹ [24]

Angioimmunoblastic T-cell lymphoma

Enteropathy-type T-cell lymphoma

Extranodal NK/T-cell lymphoma¹ [24]

Hepatosplenic T-cell lymphoma

Mycosis fungoides¹ [25]

Peripheral T-cell lymphoma, unspecified¹, ² [24]

Subcutaneous panniculitis-like T-cell lymphoma

Primary cutaneous CD30-positive T-cell lymphoproliferative

disorders (e.g. lymphomatoid papulosis)

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NK=Natural killer.

¹ Reported to secondarily involve the orbit or eye.

² Can occur as a primary intraocular or uveal lymphoma.

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Sample Handling

Regardless of the biopsy method, it is critically important to work closely with the cytopathologist to ensure that tissue is collected and triaged appropriately. The cytopathologist must be made aware that lymphoma is in the differential diagnosis so that a portion of the sample can be set aside for special studies. In addition, the biopsy should be transported rapidly to the laboratory for evaluation. Lymphoma cells are fragile and can quickly degrade, resulting in a non-diagnostic specimen [2].

Cytological material can be kept undiluted or rinsed into a tissue medium solution such as Roswell Park Memorial Institute 1640 (Invitrogen, Carlsbad, Calif., USA), which maintains cell viability. The specimen should be refrigerated while awaiting transportation to maintain cell viability. However, if there is a prolonged delay in transportation, then an appropriate preservative should be used (e.g. Cytolyt, Cytyc Corp., Boxborough, Mass., USA). This will maintain cell morphology for cytological evaluation and allow immunohistochemical stains for further analysis, but precludes flow-cytometric analysis.

Sample Processing

Once the specimen has arrived in the cytology laboratory, a portion will be processed for cytological evaluation. The cells can be centrifuged into a pellet and the supernatant removed for interleukin studies if clinically requested (see below). If the processing laboratory does not perform interleukin testing, the supernatant can be frozen and held for processing at another laboratory. By centrifuging the sample, the ...

Table of contents

• Cover Page
• Front Matter
• History, Indications, Techniques and Limitations
• Cytological Preparation
• Uveal Metastases
• Uveal Lymphoma
• Uveal Melanoma: Diagnostic Features
• Uveal Melanoma: Prognostication
• Vitreous Biopsy: Specimen Preparation and Interpretation
• Fine Needle Aspiration Biopsy of Retinal Tumors
• Fine Needle Aspiration Cytology in Orbital Tumors
• Future of Ophthalmic Fine Needle Aspiration Biopsy
• Author Index
• Subject Index