Primary esophageal lymphoma is very rare, and most reported cases are histologically mucosa-associated lymphoid tissue lymphoma. Therefore, the principle treatment strategy for primary esophageal lymphoma focuses on local treatments, such as endoscopic mucosal resection or radiation therapy, but systemic chemotherapy plays the central role in the treatment of diffuse large B cell lymphoma (DLBCL). Generally, standard treatment for DLBCL is six or three cycles of R-CHOP chemotherapy followed by involved field radiation therapy according to stage. However, the optimal treatment strategy for primary esophageal DLBCL, and the role of additional radiation is not settled, due to a paucity of cases. Moreover, the clinical characteristics related to the etiology and natural course are also unknown. Here, we present two cases of primary esophageal DLBCL with a literature review.
Drug Therapy ; Esophagus ; Lymphoma ; Lymphoma, B-Cell* ; Lymphoma, B-Cell, Marginal Zone ; Lymphoma, Large B-Cell, Diffuse

Low grade MALT lymphoma of stomach is associated with H. pylori infection in more than 90% of cases, and eradication of H. pylori leads to regression of the low grade MALT lymphoma in 60~90% of cases. On the contrary, high grade MALT lymphoma is thought to be independent from H. pylori for growth and usually is thought to require antitumor chemotherapy. However, there have been recent reports of high grade MALT lymphoma regressing after H. pylori eradication. We experienced and are reporting a case of high grade MALT lymphoma arising in the background of low grade MALT lymphoma that showed complete regression after H. pylori eradication.
Drug Therapy ; Helicobacter pylori* ; Helicobacter* ; Lymphoma ; Lymphoma, B-Cell, Marginal Zone* ; Stomach

Gastric MALT lymphoma usually presents with various endoscopic morphologic characteristics. The majority of the endoscopic findings of low-grade gastric MALT lymphoma reveal multiple and superficial erosions or ulceration with mucosal nodularity, but high-grade gastric MALT lymphomas show ulceroinfiltrative and fungating lesion. However, low-grade gastric MALT lymphoma that presents as advanced gastric cancer is very rare. We experienced a case of low-grade gastric MALT lymphoma with perigastric lymph node infiltration; it presented as advanced gastric cancer, and it was treated with H. pylori eradication and CHOP chemotherapy. So we report on this case along with a review of related literatures.
Drug Therapy ; Lymph Nodes ; Lymphoma, B-Cell, Marginal Zone* ; Stomach Neoplasms* ; Ulcer

Approximately 90% of gastric mucosa-associated lymphoid tissue (MALT) lymphomas are associated with Helicobacter pylori infection and 10% of gastric MALT lymphomas occur independent of H. pylori infection. The gene translocation t(11;18)(q21;q21) is frequently observed in cases presenting with H. pylori-negative gastric MALT lymphomas. H. pylori-negative gastric MALT lymphomas do not differ from H. pylori-positive gastric MALT lymphomas in terms of clinical features and endoscopic findings and show similar prognosis. H. pylori eradication therapy could be considered as first-line treatment for gastric MALT lymphomas regardless of their H. pylori infection status. In cases with H. pylori-negative gastric MALT lymphomas, the rate of response to eradication therapy varies from 0 to 83%. If gastric MALT lymphoma persists for more than a year, secondary treatment with radiotherapy, chemotherapy, and/or surgery should be considered. Of note, the comparative outcome is excellent.
B-Lymphocytes ; Drug Therapy ; Helicobacter pylori ; Helicobacter* ; Lymphoid Tissue ; Lymphoma ; Lymphoma, B-Cell, Marginal Zone* ; Prognosis ; Radiotherapy

Primary nodal marginal zone B-cell lymphoma (MZBCL) is recognized as a rare and distinct entity. The rate of histologic transformation into diffuse large B-cell lymphoma (DLBCL) seems lower than the rate of transformation in follicular lymphoma. We herein report a rare case ofnodal MZBCL showing transformation into DLBCL. The patient was a 73-year-old female withcervical lymphadenopathy. On the initial biopsy, the lymph node architecture was diffuselyeffaced with an extensive interfollicular and parafollicular infiltrate of monocytoid B-cells. Therewere scattered large blastic B-cells without formations of compact sheets. The diagnosis ofnodal MZBCL was made. The patient did not receive chemotherapy and was treated with aconservatively supportive regimen. Forty two months later, the patient developed a new cervicallymphadenopathy and a biopsy was performed. Histologically, the lymph node revealeddiffuse sheets of transformed large B-cells showing prominent nucleoli. The diagnosis ofDLBCL transformed from nodal MZBCL was made. The patient was treated with 3 cycles ofcombined CHOP chemotherapy and she showed clinical improvement. These observationssuggest that an untreated primary nodal MZBCL may undergo high-grade transformation.
Aged ; B-Lymphocytes ; Biopsy ; Diagnosis ; Drug Therapy ; Female ; Humans ; Lymph Nodes ; Lymphatic Diseases ; Lymphoma ; Lymphoma, B-Cell ; Lymphoma, B-Cell, Marginal Zone* ; Lymphoma, Follicular

Marginal zone B-cell lymphoma of the gastrointestinal tract most commonly affects the stomach. Endoscopic findings of primary gastrointestinal lymphoma are various and heterogeneous. Lesions may appear as ulcers, erosions, or erythemas. Gastrointestinal lymphomas presenting as multiple polyps on endoscopy are rare. No case of marginal zone B-cell lymphoma manifesting as multiple polyps has been reported on the stomach, although a few cases have been reported in the colon. We present a rare case of a 77-year-old female patient diagnosed as primary gastric marginal zone B-cell lymphoma presenting as multiple polyps. She was fully treated by combination chemotherapy.
Aged ; Colon ; Drug Therapy, Combination ; Endoscopy ; Erythema ; Female ; Gastrointestinal Tract ; Humans ; Lymphoma ; Lymphoma, B-Cell, Marginal Zone ; Polyps ; Stomach ; Ulcer

Primary rectal lymphoma is a rare disease among the gastrointestinal (GI) lymphoma. In particular, diffuse large B-cell lymphoma (DLBCL) transformed from mucosa-associated lymphoid tissue (MALT) lymphoma is often the primary type of GI lymphoma, mostly in stomach or duodenum, but has never been reported in rectum. Here we report an unusual case in which a 75-year-old male patient diagnosed with DLBCL transformed from MALT lymphoma in the rectum. The patient was diagnosed as rectal DLBCL transformed from MALT lymphoma as Lugano stage II2 and was treated with chemotherapy (R-CHOP) with CD-20 monoclonal antibody (rituxaimb). Complete remission of multiple lymphadenopathy and mass forming ulcer of the rectum was achieved after 6 cycles of R-CHOP. He has been free from disease for 12 months.
Aged ; B-Lymphocytes* ; Drug Therapy ; Duodenum ; Humans ; Lymphatic Diseases ; Lymphoid Tissue ; Lymphoma ; Lymphoma, B-Cell* ; Lymphoma, B-Cell, Marginal Zone* ; Male ; Rare Diseases ; Rectum ; Stomach ; Ulcer

Marginal zone B-cell lymphoma (MZL) is the second most common subtype of non-Hodgkin's lymphoma in Korea (17.3%). Mucosa-associated lymphoid tissue (MALT) can develop in almost any organ as a result of exposure to a persistent stimulus, such as chronic infection or certain autoimmune processes. Under conditions of prolonged lymphoid proliferation, a malignant clone may emerge, which is followed by the development of a MALT lymphoma. Whereas MALT lymphoma of the stomach is the most common and the most extensively studied, we focus on non-gastric MZL studies conducted in Korea that highlight the most recent advances with respect to MZL definition, etiology, clinical characteristics, natural history, treatment approaches, outcomes, and prognostic factors. Moreover, we discuss current organ-specific considerations and controversies, and identify areas for future research.
Humans ; Korea ; Lymphoma, B-Cell, Marginal Zone/*diagnosis/*therapy ; Neoplasm Staging ; Organ Specificity ; Prognosis ; Splenic Neoplasms/diagnosis/therapy

PURPOSE: The aim of this study was to evaluate the clinical features, histologic classification and treatment outcomes of patients with malignant lymphoma of the eye. METHODS: The participants included 22 patients with malignant lymphoma of the orbit and ocular adnexa treated at our hospital between May 1994 and September 2001. The authors retrospectively analyzed the sex, age, location of mass, histopathologic type, stage and results of the treatment. RESULTS: Of the 22 patients, 14 cases were male and 8 were female. The age ranged from 26 to 68 years (mean age 43 years). The most common presenting complaint was slowly growing mass of the eye. Histopathologic studies revealed extranodal marginal zone B-cell lymphoma, MALT type in 20 cases (91%). The stages of lymphomas were included I AE in 14 cases (63%), II AE in 3 cases (14%), III AE in 3 cases (14%) and IV A in 2 cases (9%). Patients received radiotherapy and/or chemotherapy. Twenty out of 22 cases (91%) achieved a complete remission. During the mean follow-up of 26 months, two patients died of progression of the disease; one patient had extranodal marginal zone B-cell lymphoma, MALT type of stage III AE and the other patient had diffuse large cell lymphoma of stage II AE. CONCLUSIONS: Radiotherapy can produce durable remission in the majority of the patients with localized primary orbital lymphoma. Chemotherapy should be performed for the patients with highly malignant lymphomas or lymphomas with systemic manifestations.
Classification ; Drug Therapy ; Female ; Follow-Up Studies ; Humans ; Lymphoma* ; Lymphoma, B-Cell, Marginal Zone ; Lymphoma, Large B-Cell, Diffuse ; Male ; Orbit* ; Radiotherapy ; Retrospective Studies

Primary gastrointestinal lymphomas originating from the mucosa-associated lymphoid tissue (MALT) have been reported with increasing frequency. The stomach is the most frequent site of MALT lymphoma, and a relationship with Helicobacter pylori infection has been studied. However, primary MALT lymphoma arising from the ampulla of Vater is extremely rare, and its relationship with Helicobacter pylori infection is still obscure. We report here a case of a 71-year-old man with marginal zone B cell lymphoma of MALT with large B cell lymphoma of the ampulla of Vater that was not associated with Helicobacter pylori. A pancreaticoduodenectomy was performed, and the pathologic examination of the resected specimen confirmed the diagnosis. The patient tolerated just one course of systemic chemotherapy, but has been doing well for 18 months after surgery.
Aged ; Ampulla of Vater* ; Diagnosis ; Drug Therapy ; Helicobacter pylori ; Humans ; Lymphoid Tissue ; Lymphoma ; Lymphoma, B-Cell ; Lymphoma, B-Cell, Marginal Zone ; Pancreaticoduodenectomy ; Stomach