Peripheral T-cell lymphoma of the eyelid.
ABSTRACT To report a case of a 25-year-old woman with previously treated peripheral T-cell lymphoma (PTCL) presenting with a recurrent lower eyelid lesion.
A 25-year-old young woman with previously treated PTCL noted an induration in the skin of her left lower eyelid. Upon diagnosis of a chalazion, antibiotic eye drops and ointments as well as steroid eye drops were administered. However, the condition worsened rapidly and swelling of the lower eyelid became remarkable. An excisional biopsy revealed that the palpebral lesion was diagnosed as recurrence of PTCL. Electron irradiation was applied as a radical treatment, and the prognosis was satisfactory without recurrence at 10 months after the last irradiation.
Although lymphoma isolated to the ocular adnexa is rare, it should always be included in the differential diagnosis of any patient presenting with progressive swelling of the eyelid or ocular region.
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ABSTRACT: Lymphoma is the most common malignancy arising in the ocular adnexa, which includes conjunctiva, lachrymal gland, lachrymal sac, eyelids, orbit soft tissue, and extraocular muscles. Ocular adnexal lymphoma (OAL) accounts for 1%-2% of non-Hodgkin lymphoma and 5%-15% of extranodal lymphoma. Histology, stage, and primary localizations are the most important variables influencing the natural history and therapeutic outcome of these malignancies. Among the various lymphoma variants that could arise in the ocular adnexa, marginal zone B-cell lymphoma (OA-MZL) is the most common one. Other types of lymphoma arise much more rarely in these anatomical sites; follicular lymphoma is the second most frequent histology, followed by diffuse large B-cell lymphoma and mantle cell lymphoma. Additional lymphoma entities, like T-cell/natural killer cell lymphomas and Burkitt lymphoma, only occasionally involve orbital structures. Because they are so rare, related literature mostly consists of anecdotal cases included within series focused on OA-MZL and sporadic case reports. This bias hampers a global approach to clinical and molecular properties of these types of lymphoma, with a low level of evidence supporting therapeutic options. This review covers the prevalence, clinical presentation, behavior, and histological and molecular features of uncommon forms of primary OAL and provides practical recommendations for therapeutic management.The Oncologist 06/2013; · 4.54 Impact Factor
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Clinical Ophthalmology 2009:3 527–529
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Peripheral T-cell lymphoma of the eyelid
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C A s e r e P O rT
Department of Ophthalmology,
Akita University Faculty of Medicine,
Correspondence: Makoto Ishikawa
Department of Ophthalmology, Akita
University Faculty of Medicine,
1-1-1 Hondo, Akita 010-8543, Japan
Tel +81 18 834 1111
Fax +81 18 836 2621
Purpose: To report a case of a 25-year-old woman with previously treated peripheral T-cell
lymphoma (PTCL) presenting with a recurrent lower eyelid lesion.
Patients and method: Case report.
Results: A 25-year-old young woman with previously treated PTCL noted an induration in the
skin of her left lower eyelid. Upon diagnosis of a chalazion, antibiotic eye drops and ointments
as well as steroid eye drops were administered. However, the condition worsened rapidly
and swelling of the lower eyelid became remarkable. An excisional biopsy revealed that the
palpebral lesion was diagnosed as recurrence of PTCL. Electron irradiation was applied as a
radical treatment, and the prognosis was satisfactory without recurrence at 10 months after the
Conclusions: Although lymphoma isolated to the ocular adnexa is rare, it should always be
included in the differential diagnosis of any patient presenting with progressive swelling of the
eyelid or ocular region.
Keywords: peripheral T-cell lymphoma, chalazion, eyelid
Peripheral T-cell lymphoma (PTCL) represents a broad spectrum of disease with
several different manifestations. PTCL often occurs in the extremities, but rarely in
the eyelid. Periocular involvement by PTCL usually occurs in the setting of the Sézary
syndrome or mycosis fungoides, which is a variant of PTCL.1–4 We report a case of
a 25-year-old young woman with previously treated PTCL presenting with a recurrent
lower eyelid lesion.
The patient’s history dates back to 2006 when she had multiple skin lesions that were
diagnosed as PTCL and subsequently excised. Submandibular and parotid lymph nodes
were palpable, but there was no swelling of the liver or spleen. Blood tests were negative
for adult T-cell leukemia antibody and human immunodeficiency virus (HIV) antibody
and soluble interleukin-2 receptor was within normal range. No other lesion was
detected by gallium scintigraphy or systemic computed tomography. A bone marrow
trephine biopsy did not show the involvement of lymphoma. Immunostaining revealed
the cells to be positive for CD3 and CD5, but negative for CD30, CD10, and CD75.
Based on the findings, the patient was classified as PTCL Stage IVB according to the
new World Health Organization classification.5 As the patient was chemorefractory
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against initial treatment with combined cyclophosphamide,
deoxorubicin, vincristine, and prednisone, she underwent
allogenic stem cell transplantation from a matched non-
sibling donor in March 2007 after salvage treatment with
cyclophosphamide, cytosine arabinoside, etoposide, and
dexamethasone. Transplant-conditioning therapy was cyclo-
phosphamide, 2900 mg/day for three days, plus total body
irradiation for three days (total 12 Gy). Graft-versus-host
disease prophylaxis was provided with cyclosporine A and
prednisolone. Cyclosporin levels were maintained between
300 and 400 µg/L until tapering was initiated. Initial dose
of prednisolone was 17.5 mg per oral.
In January 2008, the patient presented to our department
to check for any ocular manifestations of the graft-versus-host
disease. At that time, significant superficial punctate
keratopathy was noted in both eyes. Systemic skin and eyelids
were macroscopically normal. Systemic lymph nodes were
not palpable, and there was no swelling of the liver or spleen.
The superficial punctate keratopathy significantly improved
with eyedrops containing hyaluronan.
On July 11, 2008, the patient noted an induration in the
skin of her left lower eyelid (Figures 1A, B). Conjunctival
congestion and pain were present. Upon diagnosis of an acute
chalazion, conservative management was used. However, the
condition worsened rapidly, and swelling of the lower eyelid
became remarkable (Figures 1C, D).
On August 25, 2009, the patient underwent an
excisional biopsy of the palpebral lesion after informed
consent. A retention cyst in chalazion was not detected during
the intraoperative microscopic examination. Pathological
examination demonstrated that medium-sized lymphoma
cells with round nuclei (Figures 2A, B) had infiltrated
subcutaneously. Immunostaining revealed the cells to be
positive for CD3 (Figures 2C). Considering these results, the
palpebral lesion was diagnosed as recurrence of PTCL.
As the lesion was limited to the left lower eyelid, 25.2 Gy
electron irradiation was applied as a radical treatment. Globe
shielding was used to help prevent radiation retinopathy. The
eyelid tumor was dramatically improved after irradiation
(Figures 2D, 2E). The prognosis was satisfactory without
recurrence at 10 months after the last irradiation.
Ocular manifestations of PTCL can include both intraocular
and extraocular conditions.1–4 Cook and colleagues6 reported
that eyelid ectropion was the most common findings in their
patients with PTCL, though other ocular manifestations,
including eyelid thickening or edema, placoid tumor, tight
skin, blepharitis, and corneal abnormalities, were also found.
Stenson and Ramsay7 found eyelid tumors in eight of 30
consecutive patients with mycosis fungoides diagnosed by
biopsy. Isolated PTCL of the eyelid is rare.8,9 Gilbertson
and colleagues1 reported a case of a 38-year-old acquired
immunodeficiency syndrome (AIDS) patient with isolated
eyelid PTCL, and found only eight previous reports of
isolated PTCL, most diagnosed as mycosis fungoides. In this
type of lymphoma, relapses are common and can produce
an indolent course. Kirsch and colleagues reported a patient
with multiple recurrences of systemic T-cell lymphoma after
initial presentation with only an eyelid mass that was treated
with systemic chemotherapy and local radiation.4
The present case was a single episode of lymphoma of
the eyelid with clinical symptoms resembling a chalazion.
However, an excisional biopsy of the current lesion con-
firmed the recurrence of the actual tumor and so ruled out
a chalazion. The current case still requires close follow-up
observation, particularly of the skin around the lesion. When
radiation therapy is used for eyelid lesions, great care must
be taken with ocular side effects, including xerophthalmia
and radiation retinopathy.6 Although lymphoma isolated to
the ocular adnexa is rare, it should always be included in the
differential diagnosis of any patient presenting with progres-
sive swelling of the eyelid or ocular region, particularly with
a history of lymphoma.
Figure 1 A) Patient with chalazion-like induration of the left lower eyelid on July 11,
2008. B) High magnification of the lower eyelid with chalazion-like presentation.
C) After one month, induration of the lower eyelid became rapidly enlarged. Note a
diffuse, erythematous, thickened lower eyelid. D) High magnification of the thickened
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Figure 2 A) Light micrograph of hematoxylin and eosin stained eyelid biopsy specimen. Lymphoma cells were infiltrated into the subcutaneous tissue. P, palpebral skin;
Bar = 200 µm. B) High magnification of the lymphoma cells infiltrated in the orbicular muscles (arrows). Bar = 100 µm. C) Light micrograph of eyelid biopsy specimen immu-
nostained with CD3 antibody. CD3 positive lymphoma cells were distributed in the subcutaneous tissue. Bar = 100 µm. Photographs of eyelids before D) and after irradiation
E) The eyelid tumor was dramatically improved after irradiation.