• Journal of Yeungnam Medical Science
• /
• 제39권1호
• /
• pp.3-11
• /
• 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
• /
• 제14권11호
• /
• pp.6439-6443
• /
• 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
• /
• 제23권10호
• /
• pp.976-985
• /
• 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
• /
• 제20권2호
• /
• pp.139-146
• /
• 2002

목적 : 원발성 안와 림프종로 진단 받고 방사선치료를 받은 환자들을 대상으로 치료 반응, 재발 양상, 생존률 및 치료 부작용에 대해 알아보고자 하였다. 대상 및 방법 : 1991년 2월부터 2001년 4월까지 서울중앙병원에서 원발성 안와 림프종으로 진단 받고 방사선치료를 받은 31명의 환자를 대상으로 후향적 분석을 시행하였다. 성별분포는 남자가 18명, 여자가 13명이었다. 연령은 3세에서 73세까지 분포하였으며 중앙값은 44세였다. 원발 부위는 결막이 9명, 안검이 12명, 안와가 10명이었다. 병리학적 소견은 MALT (low-grade B-cell lymphoma of mucosa-associated lymphoid tissue type) 림프종이 28명, diffuse large B-cell 림프종이 1명, anaplastic large cell 림프종이 1명이었으며 lymphoblastic 림프종이 1명이었다. Ann Arbor staging에 따른 병기 분포를 살펴보면 1기가 31명으로 전부를 차지하였으며 각각 비장, 신장, 골수와 맥락얼기(choroid plexus)에 병변이 있었던 환자 4명은 연구에서 제외하였다. 전체 환자 중, 양측 안와 침범이 있는 환자는 6명이었다. 방사선치료는 결막과 안검 부위는 $6\~16\;MeV$의 전자선을 이용하여 전방 1문 치료로 30 Gy/10 fractions을 조사하였고, 안와 부위는 4 MV, 6 MV의 광자선을 이용하여 쐐기를 이용한 전사방 2문 치료로 $20\~28$회에 걸쳐 총 $40\~50.4\;Gy$를 조사하였다. 특히 결막과 안검 부위는 수정체를 보호하기 위한 납차폐물이 사용되었다. 항암화학요법은 12명의 환자에서 시행되었다. 중앙 추적 관찰기간은 53개월이었다. 결과 : 전체 환자의 5년 생존율은 $90.7\%$이었으며, 2명이 질병과 무관하게 폐렴과 비소세포성 폐암으로 사망하여 질병관련(cause-specific) 5년 생존율은 $96.0\%$이었으며 5년 무병 생존율은 $80.0\%$, 5년 국소제어율은 $90.5\%$였다. 방사선치료를 받은 31명 모두가 치료 후 6개월 이내에 완전관해를 보여 완전관해율은 $100\%$였다. 이 중 국소 재발은 방사선치료 후 16개월과 18개월에 2명의 환자에서 보였고 구제 방사선치료 후 모두 완전관해를 보였다. 2명이 조사 영역 밖에서 재발하였고 lymphoblastic 림프종 환자가 방사선치료 후 18개월에 골수에서 재발하여 사망하였고 MALT 림프종 환자가 방사선치료 후 41개월에 폐에 재발하여 구제 방사선치료 후 완전관해를 보였다. 12명의 환자가 방사선치료 전에 항암화학요법을 받았으며 부분관해가 11명에서 나타났고 1명은 반응을 보이지 않았다. 방사선치료 당시 시력이 있었던 29명 중 5명에서 방사선유발 백내장이 발생하였으며 2명에서 안구건조증이 발생하였다. 결론 : 원발성 안와 림프종의 대부분이 MALT 림프종이었다. 방사선치료는 매우 좋은 치료 반응과 국소 제어율을 보였으며 높은 생존율을 보였다. 국소 재발한 경우에도 방사선치료가 구제치료의 역할을 할 수 있음을 알 수 있었으며 대부분의 환자에서 심각한 후유증을 보이지 않는 안전한 치료로 생각된다.

• 한국방사선학회논문지
• /
• 제18권3호
• /
• pp.283-292
• /
• 2024

안와림프종의 전자선 치료 시 안구 부속기관의 방사선 부작용 및 합병증으로 인해 후낭하백내장 발생률이 높아질 수 있다. 이에 본 연구는 몬테카를로 전산모사를 활용하여 의료용 선형가속기와 안구의 수학적 모델을 모사하고, 안구 부속기관에 대한 선량평가와 수정체 차폐체 두께에 따른 안구 부속기관별 차폐율을 비교 분석하였다. 안구 부속기관의 선량평가 결과, 차폐체 미사용 시 선량을 기준으로 수정체의 민감 영역에서 가장 높은 흡수선량 분포를 보였으며, 그 외 수정체의 비민감 영역, 전방, 유리체, 각막, 눈꺼풀 순으로 점차 낮아지는 경향을 나타내었다. 차폐체 사용 시 선량 분석 결과, 2 mm 두께 사용 시 수정체의 민감 영역에서 90% 이상의 선량감소효과를 나타냈으며, 비민감 영역과 전방은 83% 이상, 유리체, 각막, 눈꺼풀에서는 30 ~ 62%의 선량감소효과를 보였다. 안와림프종의 전자선 치료 시 수정체 민감 영역의 선량 저감을 위해서는 최소 2 mm 이상의 차폐체 사용이 필요할 것으로 판단되며, 수정체 이외 안구 부속기관에 대한 차폐체 두께 및 면적을 고려한 차폐 방안이 필요할 것으로 사료된다.

• Journal of the Korean Association of Oral and Maxillofacial Surgeons
• /
• 제35권3호
• /
• pp.187-192
• /
• 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.

• 대한방사선치료학회지
• /
• 제26권2호
• /
• pp.281-287
• /
• 2014

목 적 : 안와 림프종(orbital lymphoma) 방사선치료를 IMRT(intensity-modulated radiotherapy)와 VMAT (volumetric modulated arc therapy)을 이용한 RapidArc로 비교 분석해 보고자 한다. 대상 및 방법 : 본원에서 치료받은 안와 림프종 환자 4명을 대상으로 IMRT, VMAT을 이용한 partial-single arc(SA)와 partial-double arc(DA)로 전산화치료계획을 수립하였다. 각 치료계획의 평가는 PTV의 CI(conform -ity Index)값과 PCI(Paddick's Conformity Index)값, HI(Homogeneity Index)값을 구하여 비교 평가하였으며 주변 정상조직의 선량평가를 시행하였다. 또한 각 치료계획의 MU(Monitor Unit)값을 비교하고 치료시간과 수정체의 선량을 측정해보았다. 결 과 : PTV의 CI값을 분석한 결과 각 0.88, 0.86, 0.92로 DA의 값이 가장 컸으며 PCI값은 1.41, 1.44, 1.33으로 DA에서 값이 가장 작게 나타났다. 또한 HI값은 1.18, 1.20, 1.15로 DA에서 가장 낮은 것을 확인할 수 있었다. 안와 림프종 주변 정상조직의 mean dose와 $V_30$, $V_20$, $V_10$, $V_5$의 값을 분석하였다. 그 결과 반대측 수정체, 눈물샘, 시신경, 안와의 선량이 SA에서 가장 높았고 IMRT에서 가장 낮게 나타났다. 평균 MU값과 치료시간은 IMRT가 SA에 비해 약 3배 정도 높게 나타났다. 결 론 : 안와 림프종 환자 IMRT 치료는 장애를 최소화하고 종양 내 선량 균등성을 높이기 위한 일반적인 치료법이다. 하지만 최근 시행하고 있는 RapidArc 치료계획에서 종양 내 선량 균등성이 IMRT 치료계획보다 우수한 결과 값을 보였고 정상조직의 선량 비교에서 큰 차이를 보이지 않았을 뿐만 아니라 MU와 치료시간 또한 약 1/3로 줄일 수 있었다. 이러한 이점은 안와 림프종 환자의 RapidArc 치료계획에 대한 지속적인 연구를 통해 치료의 효율성을 높일 수 있을 것으로 사료된다.

• 대한두경부종양학회지
• /
• 제37권2호
• /
• pp.105-109
• /
• 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
• /
• 제35권3호
• /
• pp.321-324
• /
• 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
• /
• 제53권4호
• /
• pp.307-313
• /
• 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.