• Journal of Yeungnam Medical Science
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• v.39 no.1
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• pp.3-11
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• 2022

Lymphoma is the most common primary tumor of the orbit, accounting for 55% of all orbital malignancies. When divided into histopathological subtypes, extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma) comprises the largest proportion. Clinical manifestations are unspecific, but in patients with slow-growing painless orbital mass, or red conjunctival lesion suggestive of 'salmon patch', ocular adnexa lymphoma (OAL) should be suspected. Although the pathogenetic mechanism of ocular adnexal MALT lymphoma (OAML) is not yet fully understood, the relationship between OAML and Chlamydia psittaci has been hypothesized recently, similar to that between gastric MALT lymphoma and Helicobacter pylori. This suggests a new treatment option for OAML; bacterial eradication therapy with systemic antibiotics. Several other treatment methods for OAML have been introduced, but no treatment guidelines have been established yet. In this article, we summarize the current knowledge on the clinical features, pathogenesis, diagnostic methods, therapeutic strategies, and prognosis of OAML.

• Asian Pacific Journal of Cancer Prevention
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• v.14 no.11
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• pp.6439-6443
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• 2013

Objective: To investigate whether the expression level of valosin-containing protein (VCP) is correlated with the prognosis of primary orbital mucosa-associated lymphoid tissue (MALT) lymphoma. Methods: VCP expression in 58 samples from primary orbital MALT lymphoma patients was determined by immunohistochemisty using monoclonal antibodies. Correlations between VCP expression level and prognosis were clarified by statistical analysis. Results: It was found that the percentage of VCP positive cells in samples of primary orbital MALT lymphoma ranged from 32% to 95%. The samples were divided into two groups (level 1 and level 2) according to the median value (45%) of the percentage of VCP positive cells. It was found that the expression level of VCP was significantly correlated with recurrence (P=0.003) and tumor size (P=0.008). At the same time, the 5-year disease-free and overall survival rate of patients of level 1 was significantly better than that of level 2 (P=0.001; P=0.032). There was no observed correlation between the expression level of VCP and other clinical features. Conclusion: VCP could be a useful marker for predicting the prognosis of primary orbital MALT lymphoma.

• Korean Journal of Radiology
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• v.23 no.10
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• pp.976-985
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• 2022

Objective: To compare the clinical and radiological features of various etiologies of chronic diffuse lacrimal gland enlargement. Materials and Methods: We retrospectively reviewed 91 consecutive patients who underwent surgical biopsy for chronic diffuse lacrimal gland enlargement and were diagnosed with non-specific dacryoadenitis (DA) (n = 42), immunoglobulin G4-related dacryoadenitis (IgG4-RD) (n = 33), and lymphoma (n = 16). Data on patient demographics, clinical presentation, and CT imaging findings (n = 73) and MRI (n = 43) were collected. The following radiologic features of lacrimal gland enlargement were evaluated: size, unilaterality, wedge sign, angle with the orbital wall, heterogeneity, signal intensity, degree of enhancement, patterns of dynamic contrast-enhanced, and apparent diffusion coefficient value. Radiological features outside the lacrimal glands, such as extra-lacrimal orbital involvement and extra-orbital head and neck involvement, were also evaluated. The clinical and radiological findings were compared among the three diseases. Results: Compared to the DA and IgG4-RD groups, the lymphoma group was significantly older (mean 59.9 vs. 46.0 and 49.4 years, respectively; p = 0.001) and had a higher frequency of unilateral involvement (62.5% vs. 31.0% and 15.2%, respectively; p = 0.004). Compared to the IgG4-RD and lymphoma groups, the DA group had significantly smaller lacrimal glands (2.3 vs. 2.8 and 3.3 cm, respectively; p < 0.001) and a lower proportion of cases with a wedge sign (54.8% vs. 84.8% and 87.5%, respectively; p = 0.005). The IgG4-RD group showed more frequent involvement of the extra-orbital head and neck structures, including the infraorbital nerve (36.4%), paranasal sinus (72.7%), and salivary gland (58.6%) compared to the DA and lymphoma groups (4.8%-28.6%) (all p < 0.005). Conclusion: Patient age, unilaterality, lacrimal gland size, wedge sign, and extra-orbital head and neck involvement differed significantly different between lymphoma, DA, and IgG4-RD. Our results will be useful for the differential diagnosis and proper management of chronic lacrimal gland enlargement.

• Radiation Oncology Journal
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• v.20 no.2
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• pp.139-146
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• 2002

Purpose : This study evaluated the treatment outcomes, patterns of failure, and treatment related complications of primary lymphoma patients who received definitive radiation therapy. Materials and Methods : A retrospective analysis was undertaken for 31 patients with primary orbital lymphoma at the Asan Medical Center between February 1991 and April 2001. There were 18 males and 13 females with ages ranging from 3 to 73 years (median, 44 years). The involved sites were 9 conjunctivae, 12 eyelids and 10 other orbits. The histological types were 28 MALT lymphomas (low-grade B-cell lymphoma of mucosa-associated lymphoid tissue type), 1 diffuse large B-cell lymphoma, 1 anaplastic large cell lymphoma and 1 lymphoblastic lymphoma. The Ann Arbor stages were all IE $(100\%)$. Ann Arbor stage III or IV patients were excluded from this study, Bilateral orbital involvement occurred in 6 cases. Radiation therapy was given with one anterior port of high energy electrons $(6\~16\;MeV)$ for the lesions located at the anterior structures like the conjunctivae or eyelids. Lesions with a posterior extension or other orbital lesions were treated with 4 or 6 MeV photons with appropriately arranged portals. In particular, lens blocks composed of lead alloy were used in conjunctival or eyelid lesions. Twelve patients received chemotherapy. The median follow-up period was 53 months. Results : The 5-year overall, cause-specific, and disease-free survival was $91\%,\;96\%,\;and\;80\%$, respectively. The complete response rate 6 months after radiation therapy was $100\%$. Local recurrences were observed in 2 patients at 16 and 18 months after completion of radiation treatment. They were salvaged with additional radiation therapy. Two patients developed distant metastases. A MALT lymphoma patient with a lung relapse was successfully salvaged with radiotherapy, but the other lymphoblastic lymphoma patient with bone marrow relapse expired. There were no severe complications but 5 patients developed radiation-induced cataracts and 2 patients developed dry eye. Conclusion : Most primary orbital lymphomas consisted of MALT lymphomas. Radiation therapy was a successful treatment modality for orbital lymphoma without any severe complications. In cases of local relapses, radiation therapy is also a very successful salvage treatment modality.

• Journal of the Korean Society of Radiology
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• v.18 no.3
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• pp.283-292
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• 2024

Radiation side effects and complications on the ocular adnexa during electron beam therapy for orbital lymphoma can increase the incidence of posterior subcapsular cataracts. This study simulated a medical linear accelerator and a mathematical model of the eye using monte carlo simulations to evaluate the dose to the ocular adnexa and compare the shielding effectiveness on different parts of the ocular adnexa based on lens shield thickness. The dose assessment results of the ocular adnexa showed that the lens's sensitive area had the highest absorbed dose distribution when no shield was used, followed by the lens's non-sensitive area, the anterior chamber, vitreous humor, cornea, and eyelid in descending order. With the use of a shield, a 2 mm thick shield demonstrated a dose reduction effect of over 90% in the lens's sensitive area, over 83% in the non-sensitive area and anterior chamber, and a dose reduction effect of 30 to 62% in the vitreous body, cornea, and eyelid. For dose reduction in the lens's sensitive area during electron beam therapy for orbital lymphoma, it is necessary to use a shield of at least 2 mm thickness. Additionally, shielding strategies considering the thickness and area of the shield for other ocular adnexa besides the lens are required.

• Journal of the Korean Association of Oral and Maxillofacial Surgeons
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• v.35 no.3
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• pp.187-192
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• 2009

Out of all oral malignant tumor, malignant lymphoma occurs in only 3.5%. Especially, most of the primary malignant lymphomas, which occur in the head & neck region are high-grade diffuse large B-cell lymphoma and mucosa-associated lymphoid tissue (MALT) marginal zone B-cell lymphoma is very rare. In the head & neck region, malignant lymphoma is reported to occur in the thyroid, salivary gland, trachea, larynx, orbital lobe and the Waldeyer's ring. Among the Waldeyer's ring, palatal tonsil is reported to be the most common region, but, only 1 case report was published in Korea. Until now, there were no case reports of MALT lymphoma that occurred in the tongue. The purpose of this case report is to report and discuss on a case of MALT lymphoma of the tongue.

• The Journal of Korean Society for Radiation Therapy
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• v.26 no.2
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• pp.281-287
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• 2014

Purpose : The purpose of this study is to compare the plan quality of volumetric-modulated arc therapy (VMAT) and intensity-modulated radiation therapy (IMRT) for the treatment of orbital lymphoma. IMRT, partial single arc(SA) and partial-double arc(DA) VMAT plans for four patients with orbital lymphoma treated at our institution were used for this study. Conformity Index(CI), Paddick's Conformity Index(PCI) and Homogeneity Index(HI) of planning target volume(PTV) were used to evaluate dosimetric quality of each plan. The Monitor Unit (MU), treatment time and dose of ipsilateral lens from each type of plan were measured for comparison. Materials and Methods : The CI of PTV for IMRT, SA and DA were measured as 0.88, 0.86, 0.92. The PCI of DA was the lowest as 1.33. Also HI of DA was the lowest in measured plans as 1.15. Mean dose of lens, lacrimal gland, optic chiasm, the opposite optic nerve and both orbit was analyzed with V30, V20, V10, V5. The result showed that the lowest dose in IMRT highest in SA in opposite lens, lacrimal gland, optic nerve, orbit. Results : Treatment time and average MU of IMRT was about three times higher than SA. Conclusion : Considering the superior plan quality as well as the delivery efficiency of VMAT compared with that of IMRT, VMAT may be the preferred modality for treating orbital lymphoma.

• Korean Journal of Head & Neck Oncology
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• v.37 no.2
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• pp.105-109
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• 2021

Follicular thyroid carcinoma (FTC) is the second most common thyroid cancer, following papillary carcinoma. Metastasis to the orbital rim from FTC is very rare. We recently experienced a case of FTC with metastasis to the orbital rim in a 74-year-old woman, who initially presented with a huge thyroid mass and an asymptomatic solitary orbital rim lesion. The solitary orbital rim lesion was suspected to be a separate disease entity such as lymphoma from the preoperative imaging, but bone metastasis from FTC was finally confirmed after orbital rim resection and total thyroidectomy. During follow-up, the patient presented multiple bone metastasis, so the solitary orbital rim lesion was considered a clinical sign of systemic metastasis from FTC. Therefore, we present this unique case with a review of the literature.

• Archives of Plastic Surgery
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• v.35 no.3
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• pp.321-324
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• 2008

Purpose: Lymphoma originated from mucosa associated lymphoid tissue(MALT) is most common in gastrointestinal system, and rarely found in salivary gland, thyroid, bronchus or orbit. We experienced a case of MALT lymphoma which was originated from conjunctiva and involving lower eyelid without metastasis. Methods: A 40-year-old man suffered palpable mass on right lower eyelid without pain. Orbital computed tomographic and ultrasonographic findings showed a conical mass($1.9{\times}1.2{\times}0.9cm$ size) inside lower eyelid. The mass was completely excised under local anesthesia and histopathological examination was followed. Results: Microscopic finding showed a multiple follicular colonization. In the follicle, small lymphocytes and plasma cells differentiated to centrocyte-like cell, monocyte B cell, plasma cell were diffusely infiltrated. Immunophenotyping was preformed on fixed section. The majority of the small cells were immunoreactive for the B cell marker CD20. Based on the typical histological findings supported by immunostaining, the mass was defined as MALT lymphoma. After excision, SPECT, abdominal CT was carried out and there were no evidence of extraorbital disease. Conclusion: Biopsy and pathological examination should be performed in patients who complain palpable mass on lower eyelid because of possibility of MALT lymphoma. Although MALT lymphoma is rarely metastasized, it is necessary to evaluate the extraorbital involvement using SPECT or other radiologic exams. For detecting extraorbital involvement, periodic follow-up examination is need.

• BLOOD RESEARCH
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• v.53 no.4
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• pp.307-313
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• 2018

Background Primary ocular adnexal extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (POML) is the most common subtype of lymphoma involving the eyes in Thailand. We sought to assess the characteristics and treatment outcomes of patients with POML in Thailand. Methods We retrospectively reviewed patient data and included patients diagnosed with POML between January 2004 and December 2016 at Chiang Mai University Hospital and King Chulalongkorn Memorial Hospital, Thailand. We collected and analyzed patients' clinical characteristics and treatment outcomes. Results Among 146 patients with lymphoma involving the eyes, 121 (82%) were diagnosed with POML. Sixty-four (52.9%) were women with median age 58 (range, 22-86) years. The most common presenting symptom was orbital mass (71.1%). Common sites of origin were the orbit (46.3%) and lacrimal gland (34.7%). At presentation, 22.3% of patients had bilateral eye involvement. About half of patients had stage I disease (N=59, 56.2%) and 20% had stage IV. Most patients (73.3%) had a low-risk International Prognostic Index. Radiotherapy was the main treatment for patients with limited-stage disease (66.7% in stage I and 56.5% in stage II). The overall response rate was 100% with complete response rates 80%, 77.3%, and 64.7% for stages I, II, and IV, respectively. Five-year progression-free survival (PFS) and overall survival were 66.1% and 94.0%, respectively. For patients with limited-stage disease, radiotherapy significantly improved PFS compared with treatment not involving radiotherapy (5-year PFS 89.9% vs. 37.3%, P=0.01). Conclusion We revealed that POML has good response to treatment, especially radiotherapy, with excellent long-term outcome.