Tuesday, October 30, 2012

Marginal zone lymphoma of the thoracic dura causing spinal cord compression.

Marginal zone lymphoma of the thoracic dura causing spinal cord compression.

Sept 2012


Section of Neurosurgery, Department of Surgery, University of Chicago Medical Center, Chicago, IL 60637, USA.


Primary extra-nodal marginal zone B cell lymphoma (Ex-MZBCL) or mucosa-associated lymphoid tissue (MALTlymphoma of the cranial dura is a rare but well-known entity. We describe a 58-year-old woman with primary MALT lymphoma of the spinal dura causing extreme thickening of the dura and spinal cord compression who initially presented with acute spinal cord compression from a chronic epidural lesion. She was treated with surgery and radiotherapy and diagnosed with a mature B-cell lymphoma based on gene rearrangement studies. Two years following the completion of radiotherapy, she presented with an increase in the size of the residual mass that was suggestive of an epidural lesion. On re-exploration, no epidural lesion was found; however, the dura was extremely thickened causing spinal cord compression. Clinical course, histological evaluation, immunostaining and gene rearrangement studies resulted in a final diagnosis of primary Ex-MZBCL of the spinal dura. To our knowledge, this is the first report of Ex-MZBCL in the spinal dura. This diagnosis should be considered when evaluating spinal cord lesions in patients with primary central nervous system (CNS) lymphoma, especially recurrent lesions, since this group of tumors carries a favorable outcome compared to other primary CNS lymphomas.

Simultaneous primary gastric and duodenal MALT lymphoma presenting with gastrointestinal bleeding.

Simultaneous primary gastric and duodenal MALT lymphoma presenting with gastrointestinal bleeding.

Jul 2012


Units of Gastroenterology and Digestive Endoscopy, Pathologic Anatomy, Sandro Pertini Hospital; Unit of Gastroenterology, Sapienza University, Rome, Italy.


The gastrointestinal tract, particularly the stomach, is the most common site of mucosa-associated lymphoid tissue lymphoma (MALToma). Many studies describe primary MALT lymphoma arising from the gastric mucosa, especially in association with Helicobacter pylori infection. On the contrary, primary MALT duodenal lymphoma is a very rare neoplasm. We report a case of a patient with gastrointestinal bleeding in whom primary gastric and duodenal MALT lymphoma were occurred simultaneously.

Complete Text La Clinica Terapeutica

Friday, October 26, 2012

Colonic mucosa-associated lymphoid tissue lymphoma.

Colonic mucosa-associated lymphoid tissue lymphoma.

May 2012


Division of Gastroenterology and Hepatology, Department of Internal Medicine, Iwate Medical University, Morioka, Japan.


Colonic mucosa-associated lymphoid tissue (MALT) lymphomas are rare and a definitive treatment has not been established. Solitary or multiple, elevated or polypoid lesions are the usual appearances of MALT lymphoma in the colon and sometimes the surface may reveal abnormal vascularity. In this paper we report our experience with four cases of colonic MALT lymphoma and review the relevant literature. The first patient had a smooth elevated lesion in the rectum and histopathologic examination of the biopsy from the lesion showed centrocyte-like cells infiltrating the lamina propria. Endoscopic ultrasonography (EUS) revealed thickening of the submucosa and muscularis propria. The patient underwent radiation therapy, and 9 months later a repeat colonoscopy showed complete resolution of the lesion. In case 2, colonoscopy showed a polyp in the cecum; the biopsy was diagnostic of MALT lymphoma. EUS detected a hypoechoic lesion confined to the mucosal layer of the colonic wall. The patient underwent endoscopic mucosal resection of the lesion and after 6 years of follow-up there was no evidence of recurrence. The third patient had a sessile elevated lesion in the sigmoid colon for which she underwent sigmoidectomy. Pathological examination of the surgical specimen was suggestive of MALT lymphoma. The last patient had a smooth elevated lesion in the rectum and magnification endoscopy showed irregular vascular pattern. The patient underwent endoscopic submucosal dissection, and biopsy examination showed the tumor to be MALT lymphoma. Although rare, awareness of MALT lymphoma of the colon is important to evaluate the patient appropriately and to plan further management.

Rituximab, used alone or in combination, is superior to other treatment modalities in splenic marginal zone lymphoma.

Rituximab, used alone or in combination, is superior to other treatment modalities in splenic marginal zone lymphoma.

Nov 2012


Royal Marsden Hospital and the Institute of Cancer Research, Sutton, UK.


Splenic marginal zone lymphoma (SMZL) is a rare B-cell malignancy, with no standard treatment other than splenectomy. Rituximab has shown encouraging results. We therefore retrospectively assessed 43 patients from two centres, who received rituximab, either alone or with chemotherapy. All patients responded, 34/43 (79%) achieving a complete response (CR), compared with 3/10 (30%) after chemotherapy without rituximab (P = 0·005). Of these 10 patients, 9 (90%) subsequently achieved a CR after rituximab (P = 0·02). Rituximab monotherapy appeared equally as effective as rituximab combination therapy (90% vs. 79% CR, P = 0·7) with significantly less toxicity (12·5% vs. 83%, P = 0·002). Splenectomized patients were more likely to obtain a CR with rituximab (16/16, 100%) than unsplenectomized patients (18/27, 67%, P = 0·008). Disease-free survival (DFS) at 3 years was better after rituximab than after splenectomy alone [79% (95% confidence interval 60-89) vs. 29% (8-54), Hazard ratio (HR) 0·28 (0·12-0·68), P = 0·003] and better than after chemotherapy without rituximab [25% (4-55), HR 0·21 (0·08-0·51), P = 0·0004]. Survival at 3 years after rituximab was 98%. In summary, the CR and DFS rates after rituximab, given alone or with chemotherapy, were significantly better than after chemotherapy without rituximab in the same patients, with manageable toxicity. Rituximab, with or without splenectomy, should be considered for the treatment of SMZL

Saturday, October 20, 2012

Marginal Zone Lymphoma

Marginal Zone Lymphoma


Lymphoma is the most common blood cancer. The two main forms of lymphoma are Hodgkin lymphoma (HL) and non-Hodgkin lymphoma (NHL). Lymphoma occurs when lymphocytes, a type of white blood cell, grow abnormally. The body has two main types of lymphocytes that can develop into lymphomas: B-lymphocytes (B-cells) and T-lymphocytes (T-cells). Cancerous lymphocytes can travel to many parts of the body, including the lymph nodes, spleen, bone marrow, blood or other organs, and can accumulate to form tumors.

Marginal zone lymphoma is a group of indolent (slow-growing) B-cell lymphomas, which account for approximately 12 percent of all B-cell lymphomas. The median age for diagnosis is 65.


There are three types of marginal zone lymphoma:

Extranodal marginal zone lymphoma of mucosa-associated lymphatic tissue (MALT) is the most common form of marginal zone lymphoma. It occurs outside the lymph nodes, such as the stomach, small intestine, salivary gland, thyroid, eyes and lungs. MALT is divided into two categories: gastric MALT, which develops in the stomach, and non-gastric MALT, which develops outside of the stomach. This form of lymphoma makes up approximately 9 percent of all B-cell lymphomas.

In many cases of MALT lymphoma, there is a previous medical history of inflammation or autoimmune disorders. For example, Helicobacter pylori (H. pylori), a microbial pathogen linked to chronic gastritis, has been associated with a significant portion of gastric MALT patients.

Nodal marginal zone lymphoma (sometimes called monocytoid B-cell lymphoma) occurs within the lymph nodes and makes up approximately 2 percent of all B-cell lymphomas.

Splenic marginal zone lymphoma occurs mostly in the spleen and blood. It has been associated with Hepatitis C. This form of lymphoma makes up approximately 1 percent of all B-cell lymphomas. 

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Friday, October 19, 2012

Anti-Helicobacter pylori therapy in primary MALT lymphoma of rectum.

Anti-Helicobacter pylori therapy in primary MALT lymphoma of rectum.

Jul 2012


Primary rectal MALT lymphoma is a very rare entity among extranodal MALT lymphomas and its therapeutic management has not been standardized. Different approaches including surgery, chemotherapy and radiotherapy have been proposed in the last decades. There have been reports on complete responses after anti-Helicobacter pylori therapy, also in patients without serological or histological evidence of Helicobacter pylori infection. In our patient we obtained a complete response with anti-Helicobacter pylori therapy and a disease-free survival of 34 months. Endoscopic ultrasound was useful for diagnosis and follow-up. Although the mechanisms that determined this response remain a matter of debate, anti-Helicobacter pylori therapy can be considered as first-line therapy in stage IE, nonbulky primary rectal MALT lymphoma. Endoscopic ultrasound could play a relevant role in the management of this rare condition

Absence of TCL1A expression is a useful diagnostic feature in splenic marginal zone lymphoma.

Absence of TCL1A expression is a useful diagnostic feature in splenic marginal zone lymphoma.

Oct 2012


Department of Pathology and Diagnostics, Section of Anatomic Pathology, University of Verona, P.le Scuro 10, 37134, Verona, Italy.


Splenic marginal zone lymphoma (SMZL) is a low-grade lymphoma showing a rather nonspecific immunophenotype. Gene expression profiling studies suggested that TCL1A could be a marker of SMZL, but reported data are conflicting. We evaluated TCL1A expression in a series of spleen and bone marrow samples involved by SMZL and correlated the findings with other immunophenotypical, morphological, and clinical data. In addition, we evaluated the expression of TCL1A in a series of spleens and lymph nodes involved by lymphomas that might mimic SMZL (13 nodal marginal zone lymphomas (NMZL), 39 follicular lymphomas (FL), 30 B-cell chronic lymphocytic leukemias (B-CLL), 31 mantle cell lymphomas (MCL), 1 lymphoplasmacytic lymphoma) and 15 bone marrow specimens involving hairy cell leukemia (HCL). TCL1A staining was negative in 24/31 cases of SMZL (77 %); 27/31 MCL and all B-CLL were positive for TCL1A; 32/34 cases of nodal FL (96 %) and all five splenic FL were positive for TCL1A, although at a lower intensity. Eight of 13 NMZL were positive for TCL1A, often showing a heterogeneous staining pattern. All HCL samples were strongly positive for TCL1A. No correlation was found between the pattern of splenic infiltration, TCL1A expression, and the clinical course. TCL1A-positive SMZL showed a higher rate of DBA44 staining compared to the negative ones, and this difference was statistically significant (Fisher test, single-tailed, p = 0.0397). Our data support the use of TCL1A in the panel of diagnostic markers used in the differential diagnosis of splenic low-grade B-cell lymphoma; a possible prognostic value, however, needs a larger series to be established.

Eradication Therapy Is Effective for Helicobacter pylori-Negative Gastric Mucosa-Associated Lymphoid Tissue Lymphoma.

Eradication Therapy Is Effective for Helicobacter pylori-Negative Gastric Mucosa-Associated Lymphoid Tissue Lymphoma.



Division of Gastroenterology, Tohoku University Graduate School of Medicine.


Mucosa-associated lymphoid tissue (MALT) lymphomas are extra-nodal B-cell lymphomas arising from MALT, and the most commonly affected organ is the stomach. Helicobacter pylori (H. pylori) eradication therapy with proton-pump inhibitors and antibiotics is the first-line therapy for H. pylori-positive gastric MALT lymphomas, but the effectiveness of the therapy for H. pylori-negative gastric MALT lymphomas remains controversial. Hence, we aimed to evaluate the effectiveness of this eradication therapy for H. pylori-negative MALT lymphomas. The H. pylori infection status of 158 gastric MALT lymphomapatients followed in our unit was judged by urea breath test, rapid urease test, histology of the biopsy specimen taken from the stomach during endocopy, and serum antibody against H. pylori. Seventeen patients that were negative for all four tests and did not have gastric mucosal atrophy were treated with antibiotic eradication therapy. The average age at diagnosis was 56.8 years old (range: 36-73 years), and the median follow-up period after H. pylori eradication in all 17 patients was 5.3 years (range: 0.3-12.7 years). Five patients (29.4%) achieved complete remission (CR) by eradication therapy alone. Comparison between the responding and non-responding patients revealed that the patients endoscopically diagnosed to have a single lesion of gastric MALT lymphoma were seen only in the responding group, whereas all non-responding patients had multiple lesions (P < 0.05). In conclusion, H. pylori eradication therapy achieved a favorable CR rate in H. pylori-negative gastric MALT lymphoma patients and could be considered as a first-line therapy, especially for patients with a single lesion.