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.
Antigens, CD20/metabolism ; Bone Marrow/pathology ; Esophageal Neoplasms/*diagnosis/pathology/surgery ; Gastroscopy ; Humans ; Immunohistochemistry ; Lymphoma, B-Cell, Marginal Zone/*diagnosis/pathology/surgery ; Male ; Middle Aged ; Mucous Membrane/pathology ; Proto-Oncogene Proteins c-bcl-2/metabolism ; Tomography, X-Ray Computed

<b>OBJECTIVEb>To study the clinicopathologic features, immunohistochemical findings, differential diagnosis and prognosis of type II enteropathy-associated T-cell lymphoma (EATL).

<b>METHODSb>Fourteen cases of type II EATL encountered in Department of Pathology, Nanjing General Hospital were retrospectively reviewed. The clinical data, histologic features, immunohistochemical findings and follow-up information were analyzed, with literature review.

<b>RESULTSb>There were altogether 12 males and 2 females. The median age of patient was 49 years. The sites of involvement included jejunum (10 cases) and ileum/colon (4 cases). The patients often presented with an abdominal mass, abdominal pain, diarrhea and constitutional symptoms such as fever, night sweating and cachexia. There was no clinical evidence of gluten-sensitive enteropathy. Histologically, the lymphoma cells showed full-thickness infiltration of the intestinal wall. They contained round hyperchromatic nuclei and pale cytoplasm. The stroma was minimally inflamed, with or without associated coagulative necrosis. A remarkable finding was the presence of villous atrophy, cryptal hyperplasia and intraepithelial lymphocytosis. Immunohistochemical study showed that the tumor cells expressed CD3, CD43 and CD8 (14/14). Some of them were also positive for CD56 (11/14) and CD30 (2/14). The staining for CD4, CD20, CD79a and myeloperoxidase was negative. A high proliferation index was demonstrated by Ki-67 immunostain. In-situ hybridization for EBER was negative. Follow-up data were available in 9 cases. The duration of follow-up ranged from 6 months to 36 months. Seven patients died within 14 months.

<b>CONCLUSIONSb>EATL is a rare type of lymphoma with intestinal involvement. Associated enteropathy is not demonstrated, in contrast to cases encountered in Nordic countries. A correct diagnosis requires evaluation of clinical manifestations, pathologic features and ancillary study results.

Adolescent ; Adult ; Aged ; CD3 Complex ; metabolism ; CD8 Antigens ; metabolism ; Diagnosis, Differential ; Enteropathy-Associated T-Cell Lymphoma ; genetics ; immunology ; pathology ; surgery ; Female ; Follow-Up Studies ; Gene Rearrangement, T-Lymphocyte ; Humans ; Ileal Neoplasms ; genetics ; immunology ; pathology ; surgery ; Jejunal Neoplasms ; genetics ; immunology ; pathology ; surgery ; Leukosialin ; metabolism ; Lymphoma, B-Cell, Marginal Zone ; metabolism ; pathology ; Lymphoma, Extranodal NK-T-Cell ; metabolism ; pathology ; Lymphoma, Large B-Cell, Diffuse ; metabolism ; pathology ; Male ; Middle Aged ; Retrospective Studies ; Young Adult

Mucosa-associated lymphoid tissue (MALT) lymphoma is a typical primary gastrointestinal lymphoma, particularly in the stomach. Although primary rectal lymphoma is rare, it may present as a subepithelial tumor. Several techniques have been proposed for a tissue diagnosis in subepithelial tumor, including endoscopic ultrasonography (EUS)-guided fine needle aspiration (EUS-FNA), EUS-guided trucut biopsy (EUS-TCB), and tacked biopsy. However the diagnostic efficacy of these techniques appears to be limited. The unroofing technique involves removal of the overlying mucosa, thereby exposing the subepithelial lesion. It was originally reported as a method for endoscopic treatment of colorectal lymphangioma. In this case, a subepithelial tumor of the rectum was diagnosed using the endoscopic unroofing technique. This is a useful modality for the diagnosis of subepithelial tumor, because it provides histologic results in a safe and rapid manner.
Adult ; Antigens, CD20/metabolism ; Colonoscopy ; Humans ; Immunohistochemistry ; Intestinal Mucosa/surgery ; Lymphoma, B-Cell, Marginal Zone/*diagnosis/pathology/ultrasonography ; Male ; Rectal Neoplasms/*diagnosis/pathology/ultrasonography ; Tomography Scanners, X-Ray Computed

<b>OBJECTIVEb>To study the clinical manifestations and radiological characteristics, diagnostic methods and outcomes of pulmonary mucosa-associated lymphoid tissue-derived(MALT) lymphoma.

<b>METHODSb>A retrospective review of clinical, radiological and follow-up data of 29 pulmonary MALT lymphoma cases at Shanghai Pulmonary Hospital affiliated to Tong Ji University from January 2002 to June 2010 was performed.

<b>RESULTSb>Among these patients, there were 19(65.5%) males and 10 (34.5%) females aged from 27 to 73 (median 53) years old. Common clinical manifestations were cough (51.7%), fever (20.7%), apnea (17.2%), chest pain (17.2%), fatigue (13.8%) and weight loss (13.8%), while 9(31.0%) cases had no symptoms at diagnosis. The characteristics of the chest CT showed that 22 (75.9%) of the cases had patch infiltration or consolidation of the lung, 7(24.1%) of the cases had mass, and 15 (51.7%) unilateral and 14(48.3%) bilateral lesions. Their diagnosis duration varied between 0.5 and 96 months. 18(62.1%) cases were confirmed by surgery (15 open lung and 7 video-assisted thoracic surgery, VAST), 4 (13.8%) by percutaneous lung biopsy, 5 (17.2%) by bronchoscopic biopsy, and 2 (6.9%) by peripheral lymph node biopsy. The treatment methods included surgery, combined chemotherapy, radiotherapy and Chinese herbal medicine. The 1- and 3-year-survival rates were 92.3% and 87.4%, respectively.

<b>CONCLUSIONSb>Pulmonary MALT lymphoma is atypical in clinical manifestations and radiological characteristics, and easy to be misdiagnosed. Local diseases are mainly treated by operation while extensive diseases receive combined chemotherapy. A proper diagnosis is mainly based on pathological biopsy. Patients with MALT lymphoma have a favorable outcome. Poor prognosis may be connected with poor performance status and long diagnosis duration.

Adult ; Aged ; Antigens, CD20 ; metabolism ; Antineoplastic Combined Chemotherapy Protocols ; therapeutic use ; Combined Modality Therapy ; Cyclophosphamide ; therapeutic use ; Doxorubicin ; therapeutic use ; Drugs, Chinese Herbal ; therapeutic use ; Female ; Follow-Up Studies ; Humans ; Lung ; pathology ; Lung Neoplasms ; diagnosis ; diagnostic imaging ; pathology ; therapy ; Lymphoma, B-Cell, Marginal Zone ; diagnosis ; diagnostic imaging ; pathology ; therapy ; Male ; Middle Aged ; Neoplasm Staging ; Pneumonectomy ; methods ; Prednisone ; therapeutic use ; Proto-Oncogene Proteins c-bcl-2 ; metabolism ; Retrospective Studies ; Survival Rate ; Thoracic Surgery, Video-Assisted ; Tomography, X-Ray Computed ; Vincristine ; therapeutic use

<b>OBJECTIVEb>To study the clinicopathologic features of primary thymic extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT).

<b>METHODSb>The clinical and pathologic findings were evaluated in 3 cases of biopsy confirmed thymic MALT lymphoma. The clincopathologic features, treatment and prognosis were discussed and literatures reviewed.

<b>RESULTSb>One male and two female patients presented with asymptomatic mediastinal masses with a history of Sjögren syndrome. They were aged 36, 35 and 41 years respectively, and only one patient had B symptoms. Grossly, all three tumors were encapsulated and had multiple variable-sized cysts on cut-surface. Histopathologically, the normal thymic lobular architecture was effaced by abnormal dense lymphoid infiltration. Prominent lymphoepithelial lesions were formed by centrocyte-like cells infiltrating and expanding Hassall's corpuscles and epithelial cyst lining. All cases showed apparent plasmacytic differentiation. Immunohistochemically, the tumor cells were positive for CD20, CD79a, bcl-2 and negative for CD3, CD5, cyclin D1, CD43, CD10, bcl-6, and CD23. The plasma cells showed kappa light chain restriction. Immunoglobulin heavy chain rearrangement in three cases was confirmed by PCR. All patients were at early stage and received routine chemotherapy with or without radiotherapy after surgical removal. All patients achieved complete remission with 24, 18 and 3 months follow-up, respectively.

<b>CONCLUSIONSb>Primary thymic MALT lymphoma may be a rare distinctive lymphoma. It can be diagnosed by HE and immunohistochemical study and should be differentiated from reactive lymphoid proliferation, other types of lymphoma and mediastinal thymoma.

Adult ; Antibodies, Monoclonal, Murine-Derived ; therapeutic use ; Antigens, CD20 ; metabolism ; Antineoplastic Combined Chemotherapy Protocols ; therapeutic use ; Cyclophosphamide ; therapeutic use ; Diagnosis, Differential ; Doxorubicin ; therapeutic use ; Female ; Follow-Up Studies ; Gene Rearrangement, B-Lymphocyte, Heavy Chain ; Humans ; Immunoglobulin Heavy Chains ; genetics ; Keratin-19 ; metabolism ; Lymphoma, B-Cell, Marginal Zone ; drug therapy ; genetics ; metabolism ; pathology ; surgery ; Male ; Prednisone ; therapeutic use ; Pseudolymphoma ; pathology ; Thymus Hyperplasia ; pathology ; Thymus Neoplasms ; drug therapy ; genetics ; metabolism ; pathology ; surgery ; Vincristine ; therapeutic use

No abstract available.
Antigens, CD20/metabolism ; Colonic Neoplasms/*diagnosis/pathology/surgery ; Colonoscopy ; Humans ; Lymphoma, B-Cell, Marginal Zone/*diagnosis/pathology/surgery ; Male ; Middle Aged ; Proto-Oncogene Proteins c-bcl-2/metabolism

<b>BACKGROUND AND OBJECTIVEb>Amyloid deposition is rare. If there was a great amount of amyloid depositions in the skin tissue, it would be considered to be amyloid deposition disease at first, and then primary cutaneous marginal zone B-cell lymphoma (PCMZL). This study was to analyze the diagnosis and differential diagnosis of two cases of PCMZL with amyloid deposition.

<b>METHODSb>Clinicopathologic characteristics and follow-up of two cases of PCMZL were analyzed. Immunohistochemical staining was performed by EnVision method using antibodies LCA, CD19, CD20, CD79a, CD3, CD7, MUM1, kappa, lambda, Ki-67. IgH and TCRgamma gene rearrangement was detected by polymerase chain reactive (PCR).

<b>RESULTSb>Case 1, a 71-year-old Chinese male, had a subcutaneous mass on the right elbow that was initially diagnosed with "amyloidosis" in 2004. Three years after the initial diagnosis, he developed recurrences on the right para-auxillary that was still diagnosed with "probably amyloidosis". Four years after the first diagnosis, the patient presented a lesion on the right para-auxillary with a diameter of 2 cm and a lesion on the temporal-parietal dural with a size of 6.0 cmx3.0 cmx3.0 cm. Case 2, a 68-year-old Chinese male, had a subcutaneous mass next to back of the left ear with a size of 9.0 cmx5.0 cm, and he underwent a operation one year previously because of subcutaneous mass in the same site. Microscopically, the tumors of both cases were located in dermis and subcutaneous, tumor cells were medium size with a nodular or diffuse distribution, and some of tumor cells were plasmacytoid/plasma cells. Morphologically, the temporal-parietal dural lesion was similar to subcutaneous lesion and infiltrated into cranial (case 1). Juxtaposed the tumor cells of two cases, there were the large amyloid deposits of amorphous hyaline material and concentrically laminated hyaline spherules in case 1, while cord-like amyloid deposits in case 2. Reactive lymphoid follicles with germinal centers and foreign body giant cells in the stroma were found surrounding the amyloid deposits. Congo red staining showed positive of amyloid deposition in tumor tissues of both cases. Immunohistochemical staining revealed that LCA, CD19, CD20, CD79a and MUM1 expressions were positive in tumor cells, and Ki-67 expression was about 8%-10%. IgL restricted expression as kappa positive while lambda negative was found in both cases. PCR results showed monoclone gene rearrangement of IgH gene in both cases.

<b>CONCLUSIONSb>Our findings suggest that amyloid deposition rarely present in both primary and metastatic tumors in PCMZL, and its diagnosis should be considered to avoid misdiagnosis. The patients with PCMZL should undergo regular examinations and chemotherapy as well as a long-term follow-up since it is apt to recur or relapse.

Aged ; Amyloidosis ; complications ; drug therapy ; metabolism ; pathology ; surgery ; Antigens, CD ; metabolism ; Antineoplastic Combined Chemotherapy Protocols ; therapeutic use ; Cyclophosphamide ; therapeutic use ; Diagnosis, Differential ; Doxorubicin ; therapeutic use ; Elbow ; Follow-Up Studies ; Head and Neck Neoplasms ; complications ; drug therapy ; metabolism ; pathology ; surgery ; Humans ; Interferon Regulatory Factors ; metabolism ; Leukocyte Common Antigens ; metabolism ; Lymphoma, B-Cell, Marginal Zone ; complications ; drug therapy ; metabolism ; pathology ; surgery ; Magnetic Resonance Imaging ; Male ; Neoplasm Recurrence, Local ; Prednisone ; therapeutic use ; Skin Neoplasms ; complications ; drug therapy ; metabolism ; pathology ; surgery ; Vincristine ; therapeutic use

Adult ; Antigens, CD20 ; metabolism ; CD3 Complex ; metabolism ; Diagnosis, Differential ; Facial Dermatoses ; metabolism ; pathology ; surgery ; Follow-Up Studies ; Humans ; Lymphoma, B-Cell, Marginal Zone ; metabolism ; pathology ; Lymphoma, Large-Cell, Anaplastic ; metabolism ; pathology ; Male ; Pseudolymphoma ; metabolism ; pathology ; surgery ; Skin Neoplasms ; metabolism ; pathology

Gastric mucosa-associated lymphoid tissue (MALT) lymphoma is the most common form of primary extranodal lymphomas. In most cases, it is developed as multifocal and mucosal lesions, and its initial diagnosis is made by biopsy of suspicious lesions on endoscopy. However, when gastric MALT lymphoma afflict submucosal site without typical mucosal lesion, further procedures are necessary for diagnosis, such as endoscopic mucosal resection and endoscopic ultrasonography. We recently experienced two cases of submucosal tumor-like gastric MALT lymphoma. Both cases were without any mucosal lesion. One case was confirmed by endoscopic mucosal resection, and the latter was by wedge resection. Treatment modalities included endoscopic mucosal resection, surgery, H. pylori eradication, and/or chemotherapy. Both cases achieved complete remission until our 18 months' and 16 months' follow up.
Anti-Bacterial Agents/therapeutic use ; Endosonography ; Female ; Gastric Mucosa/*pathology ; Gastroscopy ; Helicobacter Infections/drug therapy ; Helicobacter pylori ; Humans ; Lymphoma, B-Cell, Marginal Zone/*diagnosis/pathology/surgery ; Male ; Middle Aged ; Stomach Neoplasms/diagnosis

<b>OBJECTIVEb>To investigate the clinicopathologic features, diagnosis, differential diagnosis and the prognosis of hairy cell leukemia (HCL).

<b>METHODSb>Fifteen splenectomy specimens of HCL patients were investigated retrospectively using HE and immunohistochemistry in correlation with the follow-up information.

<b>RESULTSb>(1) The male to female ratio was 2.75:1, age ranged from 36 to 68 years with a median of 47 years. The most consistent clinical feature at presentation was marked splenomegaly (100%). Other symptoms included anemia (80.0%), thrombocytopenia (60.0%), leucocytosis (53.3%), pancytopenia (20.0%) and the absence of B-symptom. (2) The proportion of hairy cells was (14.6 +/- 7.2)% in periphery blood and (47.3 +/- 23.8)% in bone marrow. The positive rate of TRAP assay was 62.5% in bone marrow; 85.7% for TPA test and the detection rate for RLC was 25% by transmission electric microscopy. The frequency of bone marrow involvement was 100%. (3) The average weight of 15 spleens was (3012 +/- 1974) g. The size of 6 spleens ranged from 16 cm x 10 cm x 5 cm to 32 cm x 20 cm x 14 cm. The white pulp of spleen showed a characteristic atrophy feature or even absent due to leukemic infiltration, predominantly involving the red pulp with some sinusoidal pattern. "Blood pool" change was an infrequent feature (3/15 cases). The nuclei of leukemic cells were round (13 cases) or bean-shaped (2 cases), nucleoli inconspicuous or disappeared. The abundant cytoplasm and prominent cell border resulted in a "fried egg" appearance. By immunohistochemistry, leukemic cells were positive for CD45RA, CD20, PAX-5, CD25, CD11c, Annexin A1 and cyclinD1, but negative for CD3 and CD43. (4) 13 cases (86.7%) have been followed-up and all are alive. Among them, 9 cases are living well more than 5 years and 7 more than 10 years.

<b>CONCLUSIONSb>Splenomegaly is frequently the first manifestation of patients with HCL and occurred predominantly in the middle to elderly adults. Definite diagnosis of HCL requires a combined histological and immunohistochemical assessment of the splenectomy specimen, bone marrow biopsy and aspirate.

Adult ; Aged ; Annexin A1 ; metabolism ; Antigens, CD20 ; metabolism ; CD11c Antigen ; metabolism ; CD79 Antigens ; metabolism ; Diagnosis, Differential ; Female ; Follow-Up Studies ; Humans ; Ki-67 Antigen ; metabolism ; Leukemia, Hairy Cell ; metabolism ; pathology ; surgery ; Leukemia, Lymphocytic, Chronic, B-Cell ; metabolism ; pathology ; Leukemia, Prolymphocytic ; metabolism ; pathology ; Leukocyte Common Antigens ; metabolism ; Lymphoma, B-Cell, Marginal Zone ; metabolism ; pathology ; Lymphoma, Follicular ; metabolism ; pathology ; Lymphoma, Mantle-Cell ; metabolism ; pathology ; Male ; Middle Aged ; Retrospective Studies ; Spleen ; pathology ; Splenectomy ; Survival Rate