J Korean Ophthalmol Soc.  2016 Jul;57(7):1144-1149. 10.3341/jkos.2016.57.7.1144.

A Case of Primary Orbital Peripheral T-cell Lymphoma with Panniculitis-like Features

• Kang MK
• Sung YM
• Paik JS
• Yang SW

• KMID: 2317577
• DOI: http://doi.org/10.3341/jkos.2016.57.7.1144

Abstract

PURPOSE
To report a case of complete remission of primary orbital peripheral T-cell lymphoma with panniculitis-like features after chemotherapy.
CASE SUMMARY
A 57-year-old healthy female presented with periorbital swelling and symptoms of diplopia. The patient was first treated with high-dose systemic corticosteroids, however, symptoms persisted. Therefore, anterior orbitotomy with excisional biopsy was performed for diagnostic purposes. On microscopic examination, the excised mass showed localized dense lymphocyte infiltrates, and cytologic atypia was observed under a high-power field. On immunehistochemical examination, tumor cells were positive for CD3 and CD8 but negative for CD4, CD20 and CD56. Based on histopathological results, primary orbital peripheral T-cell lymphoma with panniculitis-like features was diagnosed. Additionally, molecular pathological testing was positive for Epstein-Barr virus. Subsequently, the patients underwent chemotherapy and complete remission was obtained.
CONCLUSIONS
Peripheral T-cell lymphoma often manifests as systemic symptoms, including lymph node enlargement and B symptom. The primary form of the disease in an orbit is very rare, and has a poor prognosis with a high mortality rate because the disease quickly progresses. Herein, the authors report a rare case of a healthy patient without any past medical history who achieved complete remission of a fast-growing primary orbital T-cell lymphoma with no preceding systemic symptoms.

Keyword

Orbital tumor; Panniculitis-like features; Peripheral T-cell lymphoma

MeSH Terms

Adrenal Cortex Hormones
Biopsy
Diplopia
Drug Therapy
Female
Herpesvirus 4, Human
Humans
Lymph Nodes
Lymphocytes
Lymphoma, T-Cell
Lymphoma, T-Cell, Peripheral*
Middle Aged
Mortality
Orbit*
Prognosis
Adrenal Cortex Hormones

Figure

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  Figure 1. Clinical photographs. (A) The right eye, erythematous, eyelid swelling and (B) extraocular movement limitation at initial presentation. (C) After chemotherapy, eyelid swelling was improved.

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  Figure 2. Serial orbital computed tomography. Axial (A) and coronal (B, C) computed tomography images show a large infiltrative mass with homogenous and high-density lesion in the right preseptal and retrobulbar region.

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  Figure 3. Serial orbital magnetic resonance imaging. T1-weighted (fat suppressed) axial (A) pre-contrast, (B) post-contrast and (C, D) coronal magnetic resonance imaging showed a contrast-enhancing lesion occupying the right conal and ex-traconal space, with mild expansion.

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  Figure 4. Microscopic examination. (A) dense lobular panniculitis composed of small to medium-sized lymphoid cells with some atypical nuclei (H&E, ×100),(B) atypical lymphoid cells rim-ming individual lipocytes (H&E, ×400). On immunohistochemical staining, (C) atypical lymphocytes show diffuse strong immunoreactivity for CD3 along the cytoplasmic membrane (×200), negative for both (D) CD56 and (E) CD20 (×200), which is consistent with peripheral T-cell lymphoma and panniculitis-like features.

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  Figure 5. Chemotherapy results after 4 months. Axial (A) and coronal (B) contrast-enhanced magnetic resonance images show decreased size and a mildly enhanced lesion in the right conal and ex-traconal space as well as regressed thickening and enhancement of the right eyelids.

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