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Figure 1.
Clinical photographs, computed tomographic scan, and magnetic resonance image. A, The right eye, showing obvious protrusion of the eyelid and proptosis. B, Lateral view of the right eye. C, Contrast-enhanced computed tomography of the brain revealed a huge mass occupying the right orbit with no bony destruction. D, Magnetic resonance imaging of the brain showed a heterogeneously enhancing soft-tissue mass extending from the eyeball to the optic canal.

Clinical photographs, computed tomographic scan, and magnetic resonance image. A, The right eye, showing obvious protrusion of the eyelid and proptosis. B, Lateral view of the right eye. C, Contrast-enhanced computed tomography of the brain revealed a huge mass occupying the right orbit with no bony destruction. D, Magnetic resonance imaging of the brain showed a heterogeneously enhancing soft-tissue mass extending from the eyeball to the optic canal.

Figure 2.
Histopathological analysis of the tumor. A, Dense infiltration of atypical lymphocytes associated with vascular proliferation (hematoxylin-eosin, original magnification ×400). B, A higher-magnification view reveals small to medium-sized atypical lymphocytic infiltration (hematoxylin-eosin, original magnification ×1000). C, Most tumor cells around the small vessel are immunohistochemically positive for CD2 (original magnification ×1000). D, The tumor cells show positive immunohistochemical reactivity for CD3 (original magnification ×1000).

Histopathological analysis of the tumor. A, Dense infiltration of atypical lymphocytes associated with vascular proliferation (hematoxylin-eosin, original magnification ×400). B, A higher-magnification view reveals small to medium-sized atypical lymphocytic infiltration (hematoxylin-eosin, original magnification ×1000). C, Most tumor cells around the small vessel are immunohistochemically positive for CD2 (original magnification ×1000). D, The tumor cells show positive immunohistochemical reactivity for CD3 (original magnification ×1000).

Figure 3.
Negative immunohistochemical staining results for CD56 (A), CD15 (B), CD30 (C), and latent membrane protein 1 (D) (original magnification ×1000).

Negative immunohistochemical staining results for CD56 (A), CD15 (B), CD30 (C), and latent membrane protein 1 (D) (original magnification ×1000).

Figure 4.
Polymerase chain reaction–based analysis and positron emission tomographic scans. A, Polymerase chain reaction–based analysis of T-cell receptor gene rearrangement indicating the presence of a monoclonal cell population in the expected range of the T-cell receptor γ chain gene (arrow) (ranged from 160-200 base pairs). Lane P represents the patient sample. B and C, Fluorine 18 (18F)–labeled fluorodeoxyglucose positron emission tomographic scan. B, Intense 18F-fluorodeoxyglucose uptake in the right orbital region. C, A coronal maximum-intensity projection positron emission tomographic scan shows no significant 18F-fluorodeoxyglucose uptake.

Polymerase chain reaction–based analysis and positron emission tomographic scans. A, Polymerase chain reaction–based analysis of T-cell receptor gene rearrangement indicating the presence of a monoclonal cell population in the expected range of the T-cell receptor γ chain gene (arrow) (ranged from 160-200 base pairs). Lane P represents the patient sample. B and C, Fluorine 18 (18F)–labeled fluorodeoxyglucose positron emission tomographic scan. B, Intense 18F-fluorodeoxyglucose uptake in the right orbital region. C, A coronal maximum-intensity projection positron emission tomographic scan shows no significant 18F-fluorodeoxyglucose uptake.

1.
Janatpour  KAChoo  PHLloyd  WC  III Primary orbital peripheral T-cell lymphoma: histologic, immunophenotypic, and genotypic features. Arch Ophthalmol 2007;125 (9) 1289- 1292
PubMedArticle
2.
Woog  JJKim  Y-DYeatts  RP  et al.  Natural killer/T-cell lymphoma with ocular and adnexal involvement. Ophthalmology 2006;113 (1) 140- 147
PubMedArticle
3.
Coupland  SEFoss  HDAssaf  C  et al.  T-cell and T/natural killer-cell lymphomas involving ocular and ocular adnexal tissues: a clinicopathologic, immunohistochemical, and molecular study of seven cases. Ophthalmology 1999;106 (11) 2109- 2120
PubMedArticle
Research Letters
August 2009

Primary Peripheral T-Cell Lymphoma of the Orbit

Arch Ophthalmol. 2009;127(8):1070-1072. doi:10.1001/archophthalmol.2009.168

A 34-year-old woman had a progressively protruding and painful mass of the right orbit for 1 month. On examination, the soft-tissue mass was about 5 cm in diameter and firmly fixed to the right orbit with resultant protrusion of the lower eyelid and proptosis (Figure 1A and B). Visual acuity was no light perception, coupled with limited eyeball motility. There was neither hepatosplenomegaly nor lymphadenopathy. Her family history was noncontributory. Computed tomography of the orbits revealed a large mass measuring 7.1 × 4.9 × 4.8 cm and completely occupying the expanded right orbit without bony destruction (Figure 1C). Magnetic resonance imaging of the orbits showed a large, heterogeneously enhancing soft-tissue mass compressing and pushing the eyeball anteriorly and extending posteriorly to the optic canal (Figure 1D). A small incision was made through the superotemporal conjunctiva and Tenon capsule, where tumor cell invasion with a cicatrizing nature was identified and incised. Histopathological analysis of orbital biopsy specimens demonstrated atypical lymphoid cell infiltration with positive immunoreactivities for CD3 and CD2 (Figure 2). The neoplastic cells were negative for CD56, CD15, CD30, and latent membrane protein 1 (Figure 3). Evaluation of T-cell receptor gene rearrangement using polymerase chain reaction showed monoclonality over the T-Cell receptor γ chain gene, consistent with peripheral T-cell lymphoma (Figure 4A). Staging fluorine 18 (18F)-labeled fluorodeoxyglucose positron emission tomography showed lesions with striking 18F-fluorodeoxyglucose uptake over the right ortibal region without evidence of hepatic, splenic, or lymph node involvement, supporting the diagnosis of primary peripheral T-cell lymphoma of the orbit (Figure 4B and C). At the 3-month follow-up, the patient refused to receive chemotherapy but completed radiotherapy. Proptosis and the protruding mass in the right eye failed to achieve satisfactory resolution.

Lymphoid neoplasms have been described as the most common malignant orbital tumor, accounting for 6% to 8% of all orbital tumors and up to 15% of all ocular adnexal tumors.1,2 A rapidly progressive protruding mass in the orbit is an unusual clinical feature that is typical of peripheral T-cell lymphoma. The study by Coupland et al3 shows the heterogeneous nature of T-cell lymphoma involving the ocular and ocular adnexal tissues and a varied associated prognosis. Our case highlights that physicians must maintain a heightened awareness of the distinct manifestation of peripheral T-cell lymphoma in developing a differential diagnosis for rapid-onset proptosis.

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Article Information

Correspondence: Dr Tai, Department of Ophthalmology, Tri-Service General Hospital, National Defense Medical Center, No. 325, Section 2, Cheng-Kung Road, Neihu 114, Taipei, Taiwan (mingchengt@yahoo.com).

Financial Disclosure: None reported.

References
1.
Janatpour  KAChoo  PHLloyd  WC  III Primary orbital peripheral T-cell lymphoma: histologic, immunophenotypic, and genotypic features. Arch Ophthalmol 2007;125 (9) 1289- 1292
PubMedArticle
2.
Woog  JJKim  Y-DYeatts  RP  et al.  Natural killer/T-cell lymphoma with ocular and adnexal involvement. Ophthalmology 2006;113 (1) 140- 147
PubMedArticle
3.
Coupland  SEFoss  HDAssaf  C  et al.  T-cell and T/natural killer-cell lymphomas involving ocular and ocular adnexal tissues: a clinicopathologic, immunohistochemical, and molecular study of seven cases. Ophthalmology 1999;106 (11) 2109- 2120
PubMedArticle
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