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

Primary splenic lymphoma (PSL) is often defined as generalized lymphoma with splenic involvement as the dominant feature. It is a rare disease that comprises approximately 1% of all malignant lymphomas. We investigated three cases of non-Hodgkin's splenic lymphoma that had different clinical features on presentation. The patients' survival times from diagnosis ranged from 59 to 143 months, without evidence of relapse after splenectomy and chemotherapy, with or without radiotherapy. This data suggest that PSL is potentially curable. Further studies are needed to evaluate the impact that different treatment modalities without splenectomy have on patient survival.
Splenic Neoplasms/*diagnosis/pathology/therapy ; Splenectomy ; Middle Aged ; Male ; Lymphoma, Large-Cell, Diffuse/*diagnosis/pathology/therapy ; Lymphoma, B-Cell/*diagnosis/pathology/therapy ; Humans ; Female

OBJECTIVETo investigate the best diagnostic and therapeutic method for primary lymphoma of the spleen.

METHODSClinicopathologic features of 23 patients treated from January 1956 to August 1999 were analyzed retrospectively.

RESULTSAll patients but one for exploration only (96%) underwent resection of the tumor. They accepted chemotherapy after operation. 23 patients were confirmed pathologically. B-cell type non-Hodgkin's lymphoma was noted in 21 patients and T-cell letion in 2. According to Ahman's staging, 9 patients belonged to stage I, 8 stage II, and 6 stage III. The 5-year survival rates were 50%, 40% and 16% respectively.

CONCLUSIONSThe diagnosis of splenic lymphoma is dependent mainly on B-ultrasound examination and CT scanning. Splenectomy combined with chemotherapy may provide optimum therapy for patients with splenic lymphoma.

Adult ; Aged ; Combined Modality Therapy ; Drug Therapy ; Female ; Humans ; Lymphoma ; diagnosis ; drug therapy ; surgery ; Male ; Middle Aged ; Retrospective Studies ; Splenectomy ; Splenic Neoplasms ; diagnosis ; drug therapy ; surgery

Portal annular pancreas is one of the pancreatic fusion anomalies in which the uncinate process of the pancreas extends to fuse with the dorsal pancreas by encircling the portal vein or superior mesenteric vein. We report two consecutive patients with portal annular pancreas. The first case is a 71-year-old male patient who underwent a pancreaticoduodenectomy for intraductal papillary mucinous neoplasm in the head of pancreas. His preoperative computed tomography scan showed the suprasplenic type portal annular pancreas. The second case is a 74-year-old female patient who underwent a laparoscopic anterior radical antegrade modular pancreatosplenectomy (RAMPS) for pancreatic body cancer. In operative finding, portal confluence (superior mesenteric vein-splenic vein-portal vein) was encased with the uncinate process of pancreas in both cases. Therefore, they required pancreatic division at the pancreatic neck portion twice. During the postoperative period, grade B and A, respectively, postoperative pancreatic fistulas occurred and were controlled by conservative management. Surgeons need to know about this rare pancreatic condition prior to surgical intervention to avoid complications, and to provide patients with well-designed, case-specific pancreatic surgery.
Adenocarcinoma, Mucinous/diagnosis/surgery ; Aged ; Female ; Humans ; Male ; Mesenteric Veins/radiography ; Pancreas/abnormalities ; Pancreatic Diseases/*diagnosis/therapy ; Pancreatic Fistula/etiology ; Pancreatic Neoplasms/diagnosis/surgery ; Pancreaticoduodenectomy/adverse effects ; Portal Vein/radiography ; Splenic Vein/radiography ; Tomography, X-Ray Computed

Antineoplastic Combined Chemotherapy Protocols ; therapeutic use ; Breast ; pathology ; Breast Neoplasms ; drug therapy ; pathology ; Cyclophosphamide ; therapeutic use ; Diagnosis, Differential ; Doxorubicin ; therapeutic use ; Female ; Follow-Up Studies ; Humans ; Lymphoma, B-Cell ; drug therapy ; pathology ; Lymphoma, Large B-Cell, Diffuse ; drug therapy ; pathology ; Male ; Middle Aged ; Prednisone ; therapeutic use ; Sarcoma ; pathology ; Spleen ; pathology ; Splenic Neoplasms ; drug therapy ; pathology ; Vincristine ; therapeutic use

OBJECTIVETo explore the clinicopathologic features of lymphoplasmacytic lymphomas (LPL).

METHODSRoutine histological examination was performed on hematoxylin-eosin stained sections of 24 bone marrow biopsies and available 6 concurrent lymph node specimens. Immunohistochemistry study was performed using EliVision methods.

RESULTSAmong 24 cases, the male-to-female ratio was 2.4:1 and the median age was 59.5 years (42 - 75). The most common symptom was weakness (83.3%, 20/24). Hyperviscosity and "B" symptoms occurred in 20.8% (5/24) and 8.3% (2/24) respectively. 41.7% (10/24) patients presented with lymphadenopathy. Anemia, leukocytosis and thrombocytopenia were seen in 79.2% (19/24), 8.3% (2/24) and 37.5% (9/24) respectively. Monoclonal Ig light chain expression was detected by serum immunofixation electrophoresis in 23 cases (95.8%), including IgM (20 cases), IgG (2 cases) and IgA (1 case). Basing on the histology and immunohistochemistry findings, the diagnosis was made in 22 bone marrow and 2 lymph node biopsies, respectively. Histologically, the bone marrow and lymph node specimens composed of small lymphocytes, plasmacytoid lymphocytes and plasma cells. The most frequent pattern of bone marrow involvement was diffuse in appearance (63.6%, 14/22), while nodular and interstitial patterns were less common (22.7%, 5/22 and 13.6%, 3/22, respectively). Lymph node involvement was also to be diffuse in pattern. The proliferative cells expressed Pax5, CD20, CD38 and CD138, but were negative for CD5, CD10, CD23, CyclinD1, CD3, CD7 and MPO.

CONCLUSIONSLPL has distinct clinicopathological features. Histological and immunohistochemistry findings are important for its differential diagnosis with chronic lymphocytic leukemia/small lymphocytic lymphoma, splenic marginal zone lymphoma and follicular lymphoma. Waldenström macroglobulinemia is LPL.

ADP-ribosyl Cyclase 1 ; metabolism ; Adult ; Aged ; Antigens, CD20 ; metabolism ; Antineoplastic Combined Chemotherapy Protocols ; therapeutic use ; Bone Marrow ; pathology ; Cyclophosphamide ; therapeutic use ; Diagnosis, Differential ; Doxorubicin ; therapeutic use ; Female ; Follow-Up Studies ; Humans ; Immunoglobulin Light Chains ; metabolism ; Immunophenotyping ; Leukemia, Lymphocytic, Chronic, B-Cell ; metabolism ; pathology ; Lymph Nodes ; pathology ; Lymphoma, B-Cell, Marginal Zone ; metabolism ; pathology ; Lymphoma, Follicular ; metabolism ; pathology ; Male ; Middle Aged ; Neoplasm Invasiveness ; PAX5 Transcription Factor ; metabolism ; Prednisone ; therapeutic use ; Splenic Neoplasms ; metabolism ; pathology ; Survival Rate ; Syndecan-1 ; metabolism ; Vincristine ; therapeutic use ; Waldenstrom Macroglobulinemia ; drug therapy ; metabolism ; pathology