Sunday, March 31, 2013

Cutaneous MALT-lymphoma: from cutaneous immunocytoma and pseudolymphoma to the current (and future) conceptions.

Cutaneous MALT-lymphoma: from cutaneous immunocytoma and pseudolymphoma to the current (and future) conceptions.



Department of Anatomic Pathology, Hospital El Bierzo, Ponferrada, Spain;


The current report examines the evolution of the concepts of immunocytoma and pseudolymphoma in a historical perspective, paying special attention to their evolvement into the groups of marginal-zone lymphoma and cutaneous MALT-lymphoma. It also examines the current conception of the existence of at least two types of cutaneous MALT-lymphomas and their relation to the duality immunocytoma/pseudolymphoma from the old literature.

Full Length Article - Romanian Journal of 
Morphology & Embryology


Epidermotropic marginal zone lymphoma simulating mycosis fungoides.

Epidermotropic marginal zone lymphoma simulating mycosis fungoides.

Feb 2013


Department of Medicine, Division of Dermatology, Montefiore Medical Center, Albert Einstein College of Medicine, Bronx, NY, USA; Dermpath Diagnostics, Port Chester, NY, USA.


Cutaneous lymphomas can usually be distinguished by architectural features, where most atypical lichenoid infiltrates implicate cutaneous T-cell lymphoma (CTCL). We report a case of an 80-year-old man who presented with asymptomatic golden brown patches and diffuse pink papules on his trunk, buttocks and hips. Biopsies revealed a lichenoid infiltrate and areas of epidermotropism. Although the overall architectural pattern was compatible with mycosis fungoides, the lymphocytes had a more monocytoid and plasmacytoid appearance, and there were interspersed mature plasma cells. Immunohistological studies revealed that the pleomorphic lymphocytes were predominantly B cells (CD20\+ and CD79a\+) with a subpopulation of smaller bland T cells (CD3\+). Moreover, the B-cell immunophenotype was compatible with marginal zone differentiation (bcl-2+, bcl-6-, CD10- and CD5-) and showed a lambda light chain restriction, confirming monoclonality. These findings were diagnostic for cutaneous marginal zone B-cell lymphoma (MZL) with epidermotropism an entity which has only been reported twice in the literature, once in the setting of primary cutaneous disease, and once as cutaneous involvement of systemic disease. This case illustrates a rare pattern of cutaneous MZL, and underscores the importance of immunophenotypic characterization of cutaneous lymphomas in order to prevent the misdiagnosis of a CTCL.


  • cutaneous marginal zone B-cell lymphoma;
  • epidermotropism;
  • mycosis fungoides

Tuesday, February 26, 2013

Secondary mucosa-associated lymphoid tissue (MALT) lymphoma of the colon.

Secondary mucosa-associated lymphoid tissue (MALTlymphoma of the colon.



Department of Internal Medicine, Harvard Medical School, Massachusetts General Hospital, Harvard University, 50 Fruit Street, Boston, MA, USA.


Mucosa-associated lymphoid tissue (MALT)-type lymphomas most commonly occur in the stomach and have been associated with Helicobacter pylori infection. However, MALT-type lymphoma of the colon is a rare entity. It commonly manifests with symptoms of weight loss, low-grade fever, constipation, melena, and hematochezia. Unlike gastric lymphoma, it is difficult to detect MALT-type lymphoma of the colon by imaging. Colonoscopy may reveal lesions whose biopsy most commonly shows abundant B lymphocytes. There is no universal immunohistochemistry profile for MALT-type lymphoma but CD 20 staining is commonly seen. Trisomies and translocations have been described and their presence has been correlated with treatment response. Due to the rarity of colonic MALT-type lymphoma, no standard guidelines are available for its management. It often occurs individually and rarely occurs simultaneously with concurrent colon adenocarcinoma. This case report describes the presentation and clinical course of a secondary MALT-type lymphoma in a patient who underwent colectomy for a prior colon adenocarcinoma.

Friday, February 15, 2013

Mucosa-associated lymphoid tissue lymphoma of the thyroid with abundant IgG4-positive plasma cells.

Mucosa-associated lymphoid tissue lymphoma of the thyroid with abundant IgG4-positive plasma cells.

Feb 2013


Department of Otolaryngology, Head and Neck Surgery, Okayama Saiseikai General Hospital, Okayama, Japan.


A case of thyroidal mucosa-associated lymphoid tissue (MALTlymphoma mimicking IgG4-related disease is described. A 54-year-old male presented with acute swelling of the anterior neck. Anaplastic thyroid carcinoma (ATC), malignantlymphoma (ML), or acute deterioration of Hashimoto's thyroiditis were initially suspected, and an emergent tracheostomy was required for progressive airway obstruction; a simultaneous biopsy from the thyroid tissue was performed. Histopathologically, the lesion consisted of sclerotic fibrosis and diffuse and dense infiltration by small lymphoid cells without atypia and plasma cells, many of which were IgG4-positive. Blood examination also revealed high serum IgG4 levels. Riedel's thyroiditis was suspected. However, despite medical treatments, a firm swelling of the thyroid still remained. In an in situ hybridization study, IgG4-negative plasma cells showed immunoglobulin light-chain restriction (κ-monotype), and immunoglobulin heavy (IgH) chain gene monoclonal re-arrangement was detected by polymerase chain reaction. The lesion was finally diagnosed asMALT lymphoma. When IgG4-related disease is suspected, it is important to thoroughly exclude other possibilities

Thursday, February 7, 2013

MALT-type parotid lymphoma - a case report and the review of the literature.

MALT-type parotid lymphoma - a case report and the review of the literature.

Jan  2013


Klinika Otolaryngologii i Onkologii Laryngologicznej, Uniwersytet Medyczny im. K. Marcinkowskiego w Poznaniu, Kierownik: prof. dr hab. med. W. Szyfter, Poland.


Primary lymphomas of the salivary glands are rare. It is estimated that they constitute no more than 5% of all lymphomas in different locations. The most common subtype developing in parotid glands is marginal zone B-cell mucosa associated lymphoid tissue type lymphoma (MALT) that belongs to a group of low-grade tumours. There are many factors associated with the incidence of that proliferative process: environmental and infectious agents as well as immune deficiency states. We describe a case of primary non-Hodgkin's lymphoma of the parotid gland arising in the background of previously undiagnosed and untreated Sjögren's syndrome in a 52-year-old woman. The article concerns a short review of the literature regarding etiology, symptoms, treatment and survival prognosis in that rare disease as well. MALT lymphomas should always be considered in the differential diagnosis of the tumors and swelling of the parotid gland area. A special, regular monitoring should include all patients with Sjögren's syndrome as those with the proven greater risk of developing that proliferative disease. The role of the laryngologist in the case of MALT-type lymphoma of the parotid gland should focus on a diagnosis and possible tumor cytoreduction with maximal saving of the facial nerve. The essential treatment of this pathology is one of the oncologists and haematologists.

Mucosa-associated Lymphoid Tissue (MALT) Lymphoma of the Lung Treated by Surgery and Rituximab;Report of a Case

Mucosa-associated Lymphoid Tissue (MALTLymphoma of the Lung Treated by Surgery and Rituximab;Report of a Case

Feb 2013

[Article in Japanese]


Department of General Thoracic, Breast and Endocrinological Surgery, Faculty of Medicine, Kagawa University, Kagawa, Japan.


We report a case of mucosa-associated lymphoid tissue (MALTlymphoma of the lung treated by surgery and rituximab. A 47-year-old man was referred to our hospital because of the lesion in the right middle lobe, which had enlarged gradually. Chest computed tomography(CT) scanning showed an infiltrative shadow of the right middle lobe. He underwent right middle lobectomy for the MALT lymphoma whose diagnosis and treatment. The tumor was pathologically diagnosed as CD20 immunostaining was positive and the adjuvant treatment by rituximab was performed.

Wednesday, January 30, 2013

Thymic mucosa-associated lymphoid tissue lymphoma involving lymph nodes.

Thymic mucosa-associated lymphoid tissue lymphoma involving lymph nodes.

Dec 2012


Department of Thoracic Surgery, Akita Red Cross Hospital, Akita 010-1495, Japan. Electronic address:



Thymic mucosa-associated lymphoid tissue (MALTlymphoma involving lymph nodes is quite rare with only 13 previous cases reported in the literature.


The 33-years-old female was referred to our department for the investigation of abnormalities on computed tomographic (CT) scans. CT scans showed a 9-cm×3-cm mass composed of a mixture of soft tissue and fat at the anterior mediastinum with lymphadenopathy in the neck, axillary and mediastinal regions. She was underwent complete surgical resection of the mass with regional lymph node dissection through a median sternotomy. Histological examination of the surgical specimens confirmed the diagnosis of MALT lymphoma arising in the thymus with nodal metastasis. She achieved complete remission after postoperative rituximab combined chemotherapy.


Thymic MALT lymphoma occurs most frequently in Asian female aged 40-60 years and commonly appears anterior mediastinal masses on CT scans. The excised tissue is necessary to confirm the accurate histological diagnosis. The disease usually remains localized for a long time, making local surgical resection highly effective. However, when the lymph nodes are involved, effective treatment approaches of the disease is still undefined.


We report a case of thymic MALT lymphoma involving lymph nodes, in which the patient was successfully treated with primary site resection with regional lymph node dissection followed by rituximab combined chemotherapy. Surgery provided not only a useful approach for collecting tissue for an accurate histological diagnosis, but also an effective local treatment, even in the case of advanced-stage thymic MALT lymphoma.

Monday, January 28, 2013

Comparative outcomes of oncologic therapy in gastric extranodal marginal zone (MALT) lymphoma: analysis of the SEER-Medicare database.

Comparative outcomes of oncologic therapy in gastric extranodal marginal zone (MALTlymphoma: analysis of the SEER-Medicare database.

Jan 2013


The Cancer Center at Memorial Hospital of Rhode Island, Pawtucket.



Therapy for gastric marginal zone (MALTlymphoma is largely based on single-arm trials. This observational study compared survival with radiotherapy, rituximab and combination chemoimmunotherapy in this disease.

Patients and methods
Gastric MALT lymphoma cases diagnosed between 1997 and 2007 were selected from the Surveillance, Epidemiology and End Results-Medicare database. Propensity score analysis and competing risk models were used to compare survival in patients with stage IE treated with radiation or chemotherapy, and in patients of all stages treated with rituximab alone or with chemoimmunotherapy.

Among 1134 patients, 21% underwent radiation and 24% chemotherapy as initial treatment. In the balanced cohort of 347 patients with stage IE, radiotherapy alone was associated with a better cause-specific survival [hazard ratio (HR) 0.27, P < 0.001]. Patients receiving systemic therapy had better survival if it incorporated rituximab (HR 0.53, P = 0.017). After adjustment for confounding, the outcomes of those who received rituximab alone or combination chemoimmunotherapy were not statistically different (P = 0.14).

elderly patients with stage IE gastric MALT lymphoma, radiotherapy was associated with lower risk of lymphoma-related death than chemotherapy. In those requiring systemic treatment, addition of cytotoxic chemotherapy to rituximab in the first-line regimen was not associated with improved survival.

Saturday, January 19, 2013

Thymic Extranodal Marginal Zone Lymphoma of Mucosa-associated Lymphoid Tissue: A Gene Methylation Study.

Thymic Extranodal Marginal Zone Lymphoma of Mucosa-associated Lymphoid Tissue: A Gene Methylation Study.

Jan 2013


Although rare, thymic mucosa-associated lymphoid tissue (MALTlymphoma is considered to be a distinct clinicopathological entity. Using a methylation-specific polymerase chain reaction, we analyzed thymic MALT lymphomas (n=18) for their methylation of the following seven tumor suppressor genes: DAPK1, p16(INK4A), p14(ARF), CDH1, RARB, TIMP3, and MGMT. Reactive lymph nodes (n=16) were used as a control. Of the seven genes examined, thymic MALTlymphomas had an increased number of genes that were methylated (2.9 genes) as compared with reactive lymph nodes (0.63, p=0.0003). In particular, thymic MALT lymphomas showed a frequent methylation of DAPK1, CDH1, TIMP3, and p14(ARF). In addition, gene methylation of the p14(ARF) was associated with a larger tumor size while that of the other three genes was not associated with any clinicopathological features examined. This study suggests that methylation of tumor suppressor genes may play an important role in thymic MALT lymphoma.

Friday, January 11, 2013

Addition of Rituximab to Chlorambucil Produces Superior Event-Free Survival in the Treatment of Patients With Extranodal Marginal-Zone B-Cell Lymphoma: 5-Year Analysis of the IELSG-19 Randomized Study.

Addition of Rituximab to Chlorambucil Produces Superior Event-Free Survival in the Treatment of Patients With Extranodal Marginal-Zone B-Cell Lymphoma: 5-Year Analysis of the IELSG-19 Randomized Study.

Jan 2013


Emanuele Zucca and Franco Cavalli, Oncology Institute of Southern Switzerland, Bellinzona, Switzerland; Annarita Conconi, Amedeo Avogadro University of Eastern Piedmont, Novara; Daniele Laszlo and Giovanni Martinelli, European Institute of Oncology; Irene Floriani, Pharmacological Research Institute, Milan; Umberto Vitolo, S. Giovanni Battista Hospital, Torino; Stefano A. Pileri, University of Bologna, Bologna; Maurizio Martelli, University "La Sapienza," Rome, Italy; Armando López-Guillermo and Elias Campo, Hospital Clinic, Barcelona, Spain; Reda Bouabdallah, Institut Paoli-Calmettes, Marseille; Bertrand Coiffier, Centre Hospitalier Lyon-Sud, Pierre-Benite; Catherine Sebban, Centre Léon Bérard, Lyon; Fabrice Jardin, Centre Henri Becquerel, Rouen; Franck Morschhauser, Hôpital Claude Huriez, Lille; Christiane Copie-Bergman, Assistance Publique-Hopitaux de Paris Groupe Henri Mondor-Albert Chenevier, L'Institut National de la Santé et de la Recherche Médicale (INSERM) U955, Créteil; Catherine Thieblemont INSERM Institut Universitaire d'Hématologie U728, Hopital Saint Louis, Paris, France; Andrew Jack, St. James's University Hospital, Leeds; and Peter Johnson, Southampton General Hospital, Southampton, United Kingdom.


Apart from localized gastric disease, there is no consensus on standard initial treatment of mucosa-associated lymphoid tissue lymphoma. The IELSG-19 study (Randomized Trial of Chlorambucil Versus Chlorambucil Plus Rituximab Versus Rituximab in MALT Lymphoma) was launched to compare chlorambucil alone versus chlorambucil plus rituximab in patients not previously given systemic anticancer therapy. 

Patients not responding to or not suitable for local therapy were eligible. In arm A, chlorambucil was given daily 6 mg/m(2) orally (PO) for 6 weeks. Responding patients and those with stable disease continued to be given daily chlorambucil 6 mg/m(2) PO for 14 consecutive days every 28 days for four cycles. In arm B, intravenous rituximab 375 mg/m(2) per day was added on days 1, 8, 15, 22, 56, 84, 112, and 140. After completion of the planned accrual, the protocol was amended to introduce a third arm with rituximab alone. We report the planned final analysis of the first two arms (113 patients in arm A and 114 in arm B).

At a median follow-up of 62 months, the 5-year event-free survival (EFS) was significantly better for the patients treated in arm B (68% v 50%; P = .002) who, despite similar overall response rates (90% v 87%), achieved a higher complete remission rate (78% v 65%; P = .025). Progression-free survival was also improved but it did not reach statistical significance (P = .057). Five-year overall survival (OS) was 89% in both arms. Both treatments were well tolerated without unexpected toxicities. 

Both treatments were active; the better response rate and EFS obtained with the addition of rituximab did not translate into improved OS.

Full text:

Friday, January 4, 2013

Study of regulatory T-cells in patients with gastric malt lymphoma: influence on treatment response and outcome.

Study of regulatory T-cells in patients with gastric malt lymphoma: influence on treatment response and outcome.



Departments of Pathology, Hospital del Mar, Barcelona, Spain ; IMIM (Hospital del Mar Medical Research Institute), Barcelona, Spain.



FOXP3+ regulatory T cells (Treg) play an essential role in modulating host responses to tumors and infections. The role of these cells in the pathogenesis of MALT lymphomas remains unknown. The aims of the study were to quantify the number of infiltrating FOXP3+ and CD3+ cells in patients with gastric MALT lymphoma at diagnosis and to study kinetics of these cells and CD20+ tumor cells after treatment and during long-term follow-up.


FOXP3+, CD3+ and CD20+ cells were analyzed by immunohistochemistry and the number of cells was quantified using a micrometric ocular. Samples of 35 patients with gastric MALT lymphoma at diagnosis and after treatment were included. Diagnostic samples were compared to 19 cases of chronic gastritis and diffuse large B-cell lymphoma(DLBCL) of the stomach.


The median number of FOXP3+ infiltrating cells was higher (27 cells/cm(2)) in gastric MALT patients than in DLBCL (10 cells; p = 0.162) but similar to chronic gastritis (20 cells; p = 0.605). No characteristic or specific distribution pattern of infiltrating FOXP3+ cells was found. Gastric MALT lymphoma patients responding to bacterial eradication therapy had higher number of FOXP3+ cells at study entry. Kinetics of both infiltrating FOXP3+ cells and tumor CD20+ cells were strongly dependent on the treatment administered.


Gastric MALT lymphomas have a number of Treg cells more similar to chronic gastritis than to DLBCL. Patients with higher number of tumor infiltrating FOXP3+ cells at study entry seem to have better response to antibiotics. Kinetics of Treg and tumor cells are influenced by type of treatment.