Endoscopic features aiding the diagnosis of gastric mucosa-associated lymphoid tissue lymphoma / 영남의대학술지

The incidence of gastric mucosa-associated lymphoid tissue (MALT) lymphoma is increasing worldwide, but the diagnosis is difficult. Most patients are asymptomatic or complain of nonspecific gastrointestinal symptoms. As the endoscopic features of gastric MALT lymphoma are variable and nonspecific, the possibility of this condition may be overlooked during esophagogastroduodenoscopy, and it remain undiagnosed. Therefore, this condition needs to be considered when an abnormal mucosa is observed during this procedure. Biopsy performed during endoscopy is the primary diagnostic test, but false negative results are possible; large numbers of samples should be collected from both normal and abnormal mucosae. Endoscopic ultrasonography is useful to assess the depth of invasion and to predict the treatment response. After treatment, follow-up tests are required every 3 months until complete remission is achieved, and annually thereafter. Early diagnosis of gastric MALT lymphoma is difficult, and its diagnosis and follow-up require wide experience and competent endoscopic technique.

Bilateral Conjunctival Mucosa-Associated Lymphoid Tissue Type Lymphoma in a Kidney Transplant Recipient / 대한이식학회지

Lymphoproliferative disorder in a posttransplant setting has emerged as a difficult problem in kidney transplantation (KT). Lymphoma involving adnexa of the eye has rarely been reported due to scarcity of lymphoreticular tissue in the ocular area. This report presents a case of a 37-year-old KT recipient who was diagnosed with conjunctival mucosa-associated lymphoid tissue lymphoma with a chief complaint of seeing black spots. Unlike other post-transplant lymphoproliferative diseases associated with the Epstein-Barr virus (EBV) reactivation via immunosuppression, the lesion was not related to the virus. The patient received radiotherapy with concomitant conversion from the tacrolimus to the sirolimus. Overall, the results presented herein indicate lymphoma may be an important differential diagnosis when KT recipients complain of ocular discomfort.

Low-Dose Radiation Therapy for Primary Conjunctival Marginal Zone B-Cell Lymphoma / Journal of the Korean Cancer Association, 대한암학회지

PURPOSE: The purpose of this study was to evaluate the clinical features and the long-term outcomes of primary conjunctival marginal zone B-cell lymphoma (MZBCL) patients who were treated with radiation therapy (RT). MATERIALS AND METHODS: Retrospective data of 79 patients with 121 primary conjunctival MZBCL lesions were collected from January 1, 2001 till June 30, 2014. All lesions were treated by local RT (26 Gy) with patient-specific customized lens-shielding device. RESULTS: The current Korean patients' cohort showed younger median age at diagnosis (38 years), great female preponderance (78.5%) and more frequent bilateral involvement (53.2%) than the previous studies. Following 26 Gy's RT, excellent clinical outcomes were achieved: 5-year rates of overall survival, local relapse-free survival, and contralateral relapse-free survival were 100%, 98.1%, and 91.5%, respectively. Two patients (2.5%) developed local relapse and five (6.3%) developed relapse at initially uninvolved contralateral conjunctiva with median interval of 52.9 months, and late adverse events of grade 2 and 3 occurred in seven (8.8%) and two (2.5%) patients, respectively. CONCLUSION: 26 Gy's RT was highly effective and safe, with the use of lens-shielding device, in treating patients with primary conjunctival MZBCL.

Treatment outcome and risk analysis for cataract after radiotherapy of localized ocular adnexal mucosa-associated lymphoid tissue (MALT) lymphoma

PURPOSE: We retrospectively reviewed the results of radiotherapy for localized ocular adnexal MALT lymphoma (OAML) to investigate the risk factors of cataract. METHODS: Sixty-seven patients with stage IE OAML treated with radiotherapy at Seoul St. Mary's Hospital from 2001 to 2016 were included. Median treatment dose was 30 Gy. Lens protection was done in 52 (76%) patients. Radiation therapy (RT) extent was as follows: superficial (82.1%), tumor mass (4.5%), and entire orbital socket (13.4%). The risk factors for symptomatic cataract were analyzed using the Cox proportional hazard model. RESULTS: Median follow-up time was 50.9 months (range, 1.9 to 149.4 months). All patients were alive at the time of analysis. There were 7 recurrences and there was no local recurrence. Median time to recurrence was 40.4 months. There were 14 cases of symptomatic cataract. Dose >30 Gy had hazard ratio of 3.47 for cataract (p = 0.026). Omitting lens protection showed hazard ratio of 4.10 (p = 0.008). CONCLUSIONS: RT achieves excellent local control of ocular MALT lymphoma. Consideration of RT-related factors such as lens protection and radiation dose at the stage of RT planning may reduce the risk of RT-induced cataract after radiotherapy.

Treatment outcome and risk analysis for cataract after radiotherapy of localized ocular adnexal mucosa-associated lymphoid tissue (MALT) lymphoma

PURPOSE: We retrospectively reviewed the results of radiotherapy for localized ocular adnexal MALT lymphoma (OAML) to investigate the risk factors of cataract. METHODS: Sixty-seven patients with stage IE OAML treated with radiotherapy at Seoul St. Mary's Hospital from 2001 to 2016 were included. Median treatment dose was 30 Gy. Lens protection was done in 52 (76%) patients. Radiation therapy (RT) extent was as follows: superficial (82.1%), tumor mass (4.5%), and entire orbital socket (13.4%). The risk factors for symptomatic cataract were analyzed using the Cox proportional hazard model. RESULTS: Median follow-up time was 50.9 months (range, 1.9 to 149.4 months). All patients were alive at the time of analysis. There were 7 recurrences and there was no local recurrence. Median time to recurrence was 40.4 months. There were 14 cases of symptomatic cataract. Dose >30 Gy had hazard ratio of 3.47 for cataract (p = 0.026). Omitting lens protection showed hazard ratio of 4.10 (p = 0.008). CONCLUSIONS: RT achieves excellent local control of ocular MALT lymphoma. Consideration of RT-related factors such as lens protection and radiation dose at the stage of RT planning may reduce the risk of RT-induced cataract after radiotherapy.

Primary Mucosa-associated Lymphoid Tissue Lymphoma Metachronously Involving Esophagus and Stomach / 대한소화기학회지

Mucosa-associated lymphoid tissue (MALT) lymphoma is found in various organs as extranodal B cell lymphoma. The gastrointestinal tract is the most commonly involved extranodal site in MALT lymphoma. However, primary esophageal MALT lymphoma is very rare. In addition, few cases with metachronous gastric involvement have been reported. A 55-year-old man was diagnosed with MALT lymphoma by surveillance esophagogastroduodenoscopy. A 5 cm esophageal submucosal tumor-like lesion was incidentally revealed by screening esophagogastroduodenoscopy two years prior. Esophagogastroduodenoscopy showed a cylindrically elongated submucosal mass with normal overlying mucosa in the mid esophagus. He underwent surgery to confirm the diagnosis. The pathologic diagnosis was esophageal MALT lymphoma. He was treated with radiation, which achieved complete remission. Esophagogastroduodenoscopy and chest computed tomography were performed every three to six months, with no evidence of recurrence for 18 months. After 21 months, several elevated gastric erosions were found on the great curvature and posterior sides of the midbody and confirmed as MALT lymphoma pathologically. Here we report a case with MALT lymphoma metachronously involving the esophagus and stomach.

Management of Suspicious Mucosa-Associated Lymphoid Tissue Lymphoma in Gastric Biopsy Specimens Obtained during Screening Endoscopy

It is often difficult to differentiate gastric mucosa-associated lymphoid tissue (MALT) lymphoma from Helicobacter pylori-associated follicular gastritis, and thus, it becomes unclear how to manage these diseases. This study aimed to explore the management strategy for and the long-term outcomes of suspicious gastric MALT lymphoma detected by forceps biopsy during screening upper endoscopy. Between October 2003 and May 2013, consecutive subjects who were diagnosed with suspicious gastric MALT lymphomas by screening endoscopy in a health checkup program in Korea were retrospectively enrolled. Suspicious MALT lymphoma was defined as a Wotherspoon score of 3 or 4 upon pathological evaluation of the biopsy specimen. Of 105,164 subjects who underwent screening endoscopies, 49 patients with suspicious MALT lymphomas who underwent subsequent endoscopy were enrolled. Eight patients received a subsequent endoscopy without H. pylori eradication (subsequent endoscopy only group), and 41 patients received H. pylori eradication first followed by endoscopy (eradication first group). MALT lymphoma development was significantly lower in the eradication first group (2/41, 4.9%) than in the subsequent endoscopy only group (3/8, 37.5%, P = 0.026). Notably, among 35 patients with successful H. pylori eradication, there was only one MALT lymphoma patient (2.9%) in whom complete remission was achieved, and there was no recurrence during a median 45 months of endoscopic follow-up. H. pylori eradication with subsequent endoscopy would be a practical management option for suspicious MALT lymphoma detected in a forceps biopsy specimen obtained during screening upper endoscopy.

A Case of Coexistence of Bone Marrow-involved Refractory Marginal Zone B-Cell Lymphoma and Therapy-related Myelodysplastic Syndrome

Recent advances in chemotherapy have led to increased survival rates for patients with hematologic malignancies. However, standard chemotherapies, including alkylating agents for non-Hodgkin lymphoma, could induce therapy-related myeloid neoplasms (t-MNs), a group of disorders categorized by the World Health Organization in 2008. Here, we report a case of coexistence of bone marrow (BM)-involved refractory marginal zone B-cell lymphoma (MZL) and therapy-related myelodysplastic syndrome (t-MDS). Simultaneous presence of refractory lymphoma and t-MN in the BM is rare, and this is the first report in Korea. The patient received allogeneic hematopoietic stem cell transplantation (HSCT) to cure both the MZL and t-MDS. Since the HSCT, he has been stable for 21 months without any evidence of recurrence.

Extranodal Marginal Zone B-Cell Lymphoma of Mucosa-Associated Tissue Type Involving the Dura / Journal of the Korean Cancer Association, 대한암학회지

Primary central nervous system marginal zone B-cell lymphoma (MZBCL) is very rare, with only a few reported cases worldwide. It has an indolent disease course with high cure potential. We experienced a rare case of dural MZBCL of mucosa-associated lymphoid tissue (MALT) in a 69-year-old man who presented with headache. A magnetic resonance imaging scan of brain showed a 1.9×3.6-cm-sized extra-axial mass with a broad based dural attachment to the anterosuperior aspect of the falx cerebri, radiographically consistent with meningioma. Surgical resection yielded a MZBCL of the MALT type. Histopathology revealed a lymphoplasmacytic infiltration of the dura, and immunohistochemical study showed a B-cell phenotype with CD20, bcl-2, MUM-1, Ki-67 positive. He was treated with chemotherapy after complete surgical resection and remained free of disease at 30 months after chemotherapy. MALT lymphoma must be considered in the differential diagnosis in patients presenting radiographically with meningioma.

Extranodal Marginal Zone B-Cell Lymphoma of Mucosa-Associated Tissue Type Involving the Dura / Journal of the Korean Cancer Association, 대한암학회지

Primary central nervous system marginal zone B-cell lymphoma (MZBCL) is very rare, with only a few reported cases worldwide. It has an indolent disease course with high cure potential. We experienced a rare case of dural MZBCL of mucosa-associated lymphoid tissue (MALT) in a 69-year-old man who presented with headache. A magnetic resonance imaging scan of brain showed a 1.9×3.6-cm-sized extra-axial mass with a broad based dural attachment to the anterosuperior aspect of the falx cerebri, radiographically consistent with meningioma. Surgical resection yielded a MZBCL of the MALT type. Histopathology revealed a lymphoplasmacytic infiltration of the dura, and immunohistochemical study showed a B-cell phenotype with CD20, bcl-2, MUM-1, Ki-67 positive. He was treated with chemotherapy after complete surgical resection and remained free of disease at 30 months after chemotherapy. MALT lymphoma must be considered in the differential diagnosis in patients presenting radiographically with meningioma.

An Unusual Presentation of Pulmonary Mucosa-Associated Lymphoid Tissue Lymphoma as Diffuse Pulmonary Infiltrates with Spontaneous Regression / Journal of the Korean Cancer Association, 대한암학회지

A 57-year-old woman presented with cough and dyspnea for 2 months. Computed tomography of the chest showed diffuse ground-glass opacities in both lungs. Histologic examination via thoracoscopic lung biopsy revealed atypical lymphoproliferative lesion. Her symptoms and radiologic findings of the chest improved just after lung biopsy without any treatment. Therefore, she was discharged and monitored at an outpatient clinic. Two months later, pulmonary mucosa-associated lymphoid tissue (MALT) lymphoma was confirmed by the detection of API2-MALT1 translocation in fluorescent in situ hybridization analysis. Although the lung lesions resolved spontaneously, she received chemotherapy due to bone marrow involvement in her staging workup. Pulmonary MALT lymphoma is rare. Nodular or consolidative patterns are the most frequent radiologic findings. Although the disease has an indolent growth, it rarely resolves without treatment. We report an unusual case of pulmonary MALT lymphoma with diffuse interstitial abnormalities on image and spontaneous regression on clinical course.

Clinicopathological characteristics and treatment outcomes of 38 cases of primary thyroid lymphoma: a multicenter study

PURPOSE: Primary thyroid lymphoma (PTL) is a rare disease and it has been investigated in a limited number of studies. The present multicenter study evaluated the clinical features and treatment outcomes of PTL. METHODS: The medical records of patients diagnosed with PTL between 2000 and 2013 in three centers were retrospectively reviewed. RESULTS: The study included 11 men and 27 women with a median age of 63.3 years (range, 42-83 years). The median follow-up was 56.0 months (range, 3-156 months). Of the 38 patients included, 16 had mucosa-associated lymphoid tissue (MALT) lymphoma, six had mixed MALT and diffuse large B-cell lymphoma (DLBCL), and 16 had DLBCL. Thirty-five patients (92.1%) had early stage (stage I/II) disease. Of the 16 MALT lymphoma patients, 14 were treated by surgery, and radiotherapy (RT) or chemotherapy was combined in five patients. Two patients received RT or chemotherapy alone. Of the six mixed MALT and DLBCL patients, three underwent surgery with chemotherapy and three underwent chemotherapy alone, RT alone, or surgery with RT. All of the 16 DLBCL patients received chemotherapy, and surgery and RT was combined in 4 and 1 patients, respectively. The 5-year survival was 100% for MALT lymphoma (7 of 7) and mixed MALT and DLBCL patients (5 of 5) and 87.5% for DLBCL patients (7 of 8). CONCLUSION: Early stage PTL has an excellent prognosis when managed by single or combined treatment modalities. Clinicians should consider PTL in patients with underlying Hashimoto's thyroiditis presenting with an enlarging thyroid mass.

A Case of Primary Cutaneous Marginal Zone B-cell Lymphoma Showing Monoclonal Rearrangement in Immunoglobulin Light Chain Gene / 대한피부과학회지

No abstract available.

The Role of Radiotherapy in the Treatment of Gastric Mucosa-Associated Lymphoid Tissue Lymphoma / Journal of the Korean Cancer Association, 대한암학회지

PURPOSE: To assess radiotherapy for patients with early stage gastric mucosa-associated lymphoid tissue (MALT) lymphoma with respect to survival, treatment response, and complications. MATERIALS AND METHODS: Enrolled into this study were 48 patients diagnosed with gastric MALT lymphoma from January 2000 to September 2012. Forty-one patients had low grade and seven had mixed component with high grade. Helicobacter pylori eradication was performed in 33 patients. Thirty-four patients received radiotherapy alone. Ten patients received chemotherapy before radiotherapy, and three patients underwent surgery followed by chemotherapy and radiotherapy. One patient received surgery followed by radiotherapy. All patients received radiotherapy of median dose of 30.6 Gy. RESULTS: The duration of follow-up ranged from 6 to 158 months (median, 48 months). Five-year overall survival and cause-specific survival rates were 90.3% and 100%. All patients treated with radiotherapy alone achieved pathologic complete remission (pCR) in 31 of the low-grade and in three of the mixed-grade patients. All patients treated with chemotherapy and/or surgery prior to radiotherapy achieved pCR except one patient who received chemotherapy before radiotherapy. During the follow-up period, three patients developed diffuse large B-cell lymphoma in the stomach, and one developed gastric adenocarcinoma after radiotherapy. No grade 3 or higher acute or late complications developed. One patient, who initially exhibited gastroptosis, developed mild atrophy of left kidney. CONCLUSION: These findings indicate that a modest dose of radiotherapy alone can achieve a high cure rate for low-grade and even mixed-grade gastric MALT lymphoma without serious toxicity. Patients should be carefully observed after radiotherapy to screen for secondary malignancies.

Multiple Gastrointestinal Tract MALT Lymphomas with Bone Marrow Involvement: A Case Report and Literature Review / 대한내과학회지

The stomach is the most common site of gastrointestinal tract lymphoma, while synchronous mucosa-associated lymphoid tissue (MALT) lymphomas of the stomach and duodenum are very rare. A literature review found no reported case of synchronous gastroduodenal MALT lymphomas with involvement of the bone marrow and spleen. Here, we describe the case of a 62-year-old male who was diagnosed with synchronous MALT lymphomas of the stomach and duodenum based on upper gastrointestinal endoscopy and pathology. Other staging evaluations, including colonoscopy, abdominopelvic and chest computed tomography (CT), 18F fludeoxyglucose-positron emission tomography (FDG-PET), and a bone marrow examination, showed involvement of the bone marrow and spleen. We diagnosed stage EIV MALT lymphoma and began systemic chemotherapy. We report the first case of MALT lymphomas arising synchronously in both the stomach and duodenum with bone marrow involvement and review the literature.

A Case of Crescentic Glomerulonephritis and Marginal Zone B-cell Lymphoma / 대한내과학회지

Secondary rapidly progressive glomerulonephritis (RPGN) can be caused by many diseases and conditions, including vasculitis, systemic rheumatic diseases, infections, drugs and malignancies. Among the secondary RPGNs, malignancy-associated RPGN is extremely rare and causes renal function deterioration within several weeks to months. Thus, timely immunosuppressant therapy can improve renal outcome. Herein, we describe a case of RPGN detected simultaneously with marginal zone B-cell lymphoma. An 82-year-old male patient, who presented generalized edema and oliguria, was diagnosed with crescentic glomerulonephritis and marginal B-cell lymphoma. After the patient was given methylprednisolone pulse therapy, renal function was restored and hemodialysis was successfully discontinued without complications.

A Case of Crescentic Glomerulonephritis and Marginal Zone B-cell Lymphoma / 대한내과학회지

Secondary rapidly progressive glomerulonephritis (RPGN) can be caused by many diseases and conditions, including vasculitis, systemic rheumatic diseases, infections, drugs and malignancies. Among the secondary RPGNs, malignancy-associated RPGN is extremely rare and causes renal function deterioration within several weeks to months. Thus, timely immunosuppressant therapy can improve renal outcome. Herein, we describe a case of RPGN detected simultaneously with marginal zone B-cell lymphoma. An 82-year-old male patient, who presented generalized edema and oliguria, was diagnosed with crescentic glomerulonephritis and marginal B-cell lymphoma. After the patient was given methylprednisolone pulse therapy, renal function was restored and hemodialysis was successfully discontinued without complications.

Primary Mucosa-associated Lymphoid Tissue Lymphoma of the Esophagus, Manifesting as a Submucosal Tumor / 대한소화기학회지

We report a case of primary mucosa-associated lymphoid tissue (MALT) lymphoma in the esophagus that manifested as a large submucosal tumor (SMT). Primary esophageal lymphoma is very rare, occurring in less than 1% of all patients with gastrointestinal lymphoma. Only a few cases of MALT lymphoma in the esophagus have been reported in the English literature. A 53-year-old man was referred to Dongguk University Ilsan Hospital (Goyang, Korea) in July 2012 for further evaluation and treatment of an esophageal SMT. Endoscopy showed a cylindrically elongated submucosal mass with normal overlying mucosa in the mid esophagus, 25-30 cm from the incisor teeth. He underwent surgery to confirm the diagnosis. Pathologic findings showed diffuse small atypical lymphoid cells which were stained with Bcl-2, CD20, but not with CD3, CD5, CD23, Bcl-6, or cyclin D1. These cells showed a positive monoclonal band for immunoglobulin heavy chain gene rearrangement. Based on the pathological, immunohistochemical, and molecular biological features, the esophageal mass was diagnosed as extranodal marginal zone B-cell lymphoma of the MALT type.

Clinical Study of Ocular Adnexal Extranodal Marginal Zone B-cell Lymphoma: From the Perspective of Dermatology / 대한피부과학회지

BACKGROUND: The ocular adnexal extranodal marginal zone B-cell lymphoma has been known as the 2nd most common extranodal marginal zone B-cell lymphoma, which presents as the dermatologic symptom and sign, such as orbital mass and swelling. However, the ocular adnexal extranodal marginal zone B-cell lymphoma has been few reported in the dermatologic literature. OBJECTIVE: We described the clinical and histopathological features of ocular adnexal extranodal marginal zone B-cell lymphoma from the perspective of dermatology. METHODS: Ninety-nine cases of ocular adnexal extranodal marginal zone B-cell lymphoma patients were included in this study, among the 352 patients, who were diagnosed with extranodal marginal zone B-cell lymphoma between March 1998 and February 2011. Their medical records and histopathologic slides were retrospectively reviewed. RESULTS: Among the 352 patient of extranodal marginal zone B-cell lymphoma, ocular adnexal extranodal marginal zone B-cell lymphoma occurred in 99 patients (28.1%). The ratio between male and female was 1:2, and the mean age was 50 years (range; 20~54 years). Ocular adnexal extranodal marginal zone B-cell lymphoma presented as orbital mass (39.1%), conjunctival injection (25.6%), orbital swelling (19.5%), and etc. In most patients, lesions were located in conjunctiva (63.8%) or orbit (14.5%). 76.7% of patients presented with Ann Arbor stage I disease. Relapses occurred in 8.4% of patients during the follow-up period. CONCLUSION: When dermatologists meet patients presenting with orbital mass and swelling, dermatologists consider the possibility of ocular adnexal extranodal marginal zone B-cell lymphoma and consultation with the ophthalmologists.

Regression of Advanced Gastric MALT Lymphoma after the Eradication of Helicobacter pylori

A 66-year-old female presented with a 1-month history of dyspepsia. An initial upper gastrointestinal endoscopy with biopsy revealed a low-grade mucosa-associated lymphoid tissue (MALT) lymphoma. A rapid urease test was positive for Helicobacter pylori. Endoscopic ultrasound (EUS) and computed tomography (CT) revealed a 30x15-mm lymph node (LN) in the subcarinal area. Histopathologic and phenotypic analyses of the biopsy specimens obtained by EUS-guided fine-needle aspiration revealed a MALT lymphoma, and the patient was diagnosed with a stage 4E gastric MALT lymphoma. One year after H. pylori eradication, the lesion had disappeared, as demonstrated by endoscopy with biopsy, CT, fusion whole-body positron emission tomography, and EUS. Here, we describe a patient with gastric MALT lymphoma that metastasized to the mediastinal LN and regressed following H. pylori eradication.