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OJHAS Vol. 7, Issue 2: (2008
Apr-Jun) |
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Radiation Therapy in a Case of
Orbital Lymphoma |
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Molinaro MA,
U.O.Radiation Oncology and
Radiobiology A.O., PUGLIESE-CIACCIO Catanzaro |
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Address For Correspondence |
Dr. Mariangela Molinaro, Via G Verdi 202,
87036 Rende Cosenza, ITALY E-mail:
mmariangel@libero.it |
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Molinaro MA. Radiation Therapy in a Case of
Orbital Lymphoma. Online J Health Allied Scs.
2008;7(2):8 |
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Submitted: April 24, 2007; Accepted:
June 28, 2008; Published: July 21, 2008 |
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Abstract: |
Introduction:
Orbital lymphoma is a rare presentation of extranodal non-Hodgkin's
lymphoma, accounting for less than 1% of the total. The role of radiotherapy
in its management is well-established. This is a report of a case
of orbital lymphoma. Materials and Methods: Clinical records and pathologic material of a patient woman
of 62 age treated with local radiotherapy for localized orbital lymphoma.
Treatment consisted of 36 Gy in 1.8-Gy fractions of irradiation using
6-MV photons with complex treatment planning for retrobulbar lesions.
Median follow-up was 18 months. Results: Local
control was 100%. Cataracts was not observed in 8 of the treated eyes.Conclusion: In this case,
localized orbital MALT lymphoma was well controlled with radiotherapy.
Key Words:
Eye, Non-Hodgkin's
lymphoma, Orbit, Radiotherapy |
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An extranodal marginal zone
B-cell lymphoma of mucosa associated lymphoid tissue (MALT lymphoma),
first described in 1983 by Isaacson and Wright, was recognized in 1994
as a distinct entity of low-grade B-cell lymphoma in the revised European-American
lymphoma (REAL) classification among marginal zone B-cell lymphomas,
as well as in the most recent classification of the World Health Organization
(WHO).[1-3] For localized MALT lymphoma, radiotherapy is the most
frequently applied management, and most patients show good response
to radiotherapy, although several recent reports suggest that radiotherapy
alone may not provide for a superior outcome.[4-8] For the management
of lymphomas in the ocular adnexa, especially for localized disease,
radiotherapy is a safe and effective form of local treatment. Histology
according to the REAL or WHO classification can be used to accurately
predict the prognosis of lymphomas in the ocular adnexa, and the MALT
type has a more favorable prognosis than do malignant lymphomas of differing
histology Although there have been few analyses of large numbers of
MALT lymphomas in the ocular adnexa, its prognosis is thought to be
better.
A 62-year-old woman consulted
a nearby hospital because of left exophthalmus in May 2004, and was
treated with eye drops (betamethasone sodium phosphate) for the diagnosis
of pseudolymphoma. Two years later, he developed exophthalmus and orbital
pain, and consulted the department of ophthalmology of our hospital.
Computed tomography (CT) showed a left intra-orbital extraconal tumor
(Fig. 1).
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Fig 1: Non-contrast (right)
and post-contrast computed tomography (CT) scans show
the homogenous soft tissue mass adherent to the ocular wall
extending from the anterior to posterior of the globe. |
An
excision biopsy was performed in the spring of 2006, and the findings of immunohistopathologic examination revealed a MALT lymphoma.
Full body CT and bone marrow biopsy revealed stage I AE, and the IPI
(international prognostic index) indicated a low-risk group. She was
referred to our department of radiology to receive radiation therapy.
She was irradiated with a dose of 3600 cGy in 20 fractions using a 6
MV X-ray. Left anterior oblique and a right anterior oblique fields
with a 60° wedge were delivered to the left orbita. The tumor decreased markedly in size
(Fig. 2) on CT after the completion of radiation therapy. In December
2006. Although his visual acuity decreased from 0.1 (0.4) to 0.1 (0.1),
no relapse was noted during the two-year follow-up.
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Fig 2: The orbital mass was decreased
in size after the completion of radiation
therapy. |
The cause of orbital MALT lymphoma
is not known. Fifty-five percent of malignant tumors arising in the orbita
are malignant lymphomas.6 MALT lymphoma is the most common,
accounting for 38%, followed by follicular
center lymphoma at 29%, diffuse large B cell lymphoma
at 19%, and mantle cell lymphoma at 7%. Its prognosis
is considered to be somewhat better than that of
MALT lymphoma of other sites,[8] and radiation therapy
is an excellent modality for treatment. Radiotherapy is a well-established
treatment modality for orbital lymphoma.. Primary
chemotherapy has minimal efficacy in localised
low-grade orbital lymphoma and thus is not advocated as
a first-line treatment.8 Different radiation techniques
can be used depending on the extent of
involvement. For tumours limited to the eyelids, superficial
radiation with electrons or orthovoltage is adequate.
However, the majority of orbital lymphomas have involvement
of the retrobulbar structures. These
lesions require the entire orbit to be included in the
radiation portals. This can be accomplished by using a wedge
pair arrangement or a direct field. There have been numerous series
advocating low dose radiation for treatment of
orbital lymphomas.[9-10] In general, 30 Gy is recommended for low
grade lymphomas and 40 Gy for intermediate
grade lymphomas. In our serie, the prescribed dose
was 36 Gy. Cataracts are a complication
of radiation to the orbit. When doses to the lens exceed
15 Gy, there is a 50% chance of cataract formation
and hence, visual impairment. The time to cataract formation
is 3 to 8 years after radiation. Esik et al[11] reported
that unshielded >20 Gy will result in cataract
formation if follow-up time is adequate, but they did not
specify the time interval required. Various techniques
of lens shielding have been devised. However, care must
be taken that the tumour is not inadvertently shielded
as well. Other complications of radiation to the eye for
orbital lymphoma include: dry eye syndrome, and rarely
glaucoma. Long-term local control of
orbital lymphoma can be achieved with radiation therapy.
- Isaacson PG, Wright
D. Malignant lymphoma of mucosa-associated lymphoid tissue: a distinctive type
of B-cell lymphoma. Cancer 1983;52:1410-1416
- Harris NL, Jaffe
ES, Stein H et al. A revised European-American classification of lymphoid neoplasm: a proposal
from the International Lymphoma Study Group. Blood 1994;84:1361-1392.
- Isaacson PG, Muller-Hermelink
HK, Pris MA et al. Extranodal marginal zone B-cell lymphoma of mucosa-associated
lymphoid tissue (MALT lymphoma). In: Jaffe ES, Harris NI, Stein H, et al.
(eds): World Health Organization Classification of Tumors. Pathology and Genetics of Haematopoietic
and Lymphoid Tissue. Lyon: IARC Press 2001; 157-160.
- Raderer M, Streubel
B, Woehrer S et al. High relapse rate in patients with MALT lymphoma warrants lifelong
follow-up. Clin Cancer Res 2005;11:3349-3352.
- Aviles A, Nambo
MJ, Neri N et al. Mucosa-associated lymphoid tissue (MALT) lymphoma of the stomach: results
of a controlled clinical trial. Med Oncol 2005;22:57-62.
- Wenzel C, Fiebiger
W, Dieckmann K et al. Extranodal marginal zone B-cell lymphoma of mucosa-associated
lymphoid tissue of the head and neck area. Cancer 2003;97:2236-2241.
- Tsang RW, Gospodarowicz
MK, Pintilie M et al. Localized mucosa-associated lymphoid tissue lymphoma treated
with radiation therapy has excellent clinical outcome. J Clin Oncol 2003;21:4157-4164.
- Tsang RW, Gospodarowicz
MK, Pintilie M et al. Stage I and II MALT lymphoma: results of treatment with radiotherapy.
Int J Radiat Oncol Biol Phys 2001;50:1258-1264
- Minehan K J, Martenson
J A, Garrity J A, Kurtin P J, Banks P M, Chen M G, et al. Local control and complications
after radiation therapy for primary orbital lymphoma: A case for
low dose treatment. Int J Radiat Oncol Biol Phys 1991;20:791-6.
- Chao C K, Lin H
S, Devineni V R, Smith M. Radiation therapy for primary orbital lymphoma. Int J Radiat
Oncol Biol Phys 1995;31:929-34.
- Esik O, Ikeda H,
Mukai K, Kaneko A. A retrospective analysis of different modalities for treatment of
primary orbital non-Hodgkin's lymphomas. Radiother Oncol 1996;38:13-8.
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