Lymphoma Of Mucosa-associated Lymphoid Tissue Type Research Articles

EXTRANODAL LYMPHOMA SIMILAR TO A BENIGN TUMOR IN THE ORAL CAVITY: A CASE REPORT

A 68-year-old woman arrived at our clinic with a slow-growing nodule in the buccal region, next to tooth 45. The patient had a 9-year history of a diagnosis of mucosa-associated lymphoid tissue-type lymphoma in the gastrointestinal region. She reported pain on palpation. In the intraoral examination, a submucosal nodule, sessile, of firm consistency and with the color of the mucosa was observed. The lesion did not appear on imaging exams. An excisional biopsy was performed under local anesthesia, because of the benign aspect, with a clinical diagnosis of pleomorphic adenoma. The histologic analysis showed atypical lymphocyte cells. Furthermore, the specimen showed positivity for CD20, Ki-67, and BCL-2. The diagnosis was of a lesion suggestive of MALT lymphoma. The patient was referred to and oncologist, where she underwent a computed tomography scan, but no other injured sites were found. The patient is under medical supervision. A 68-year-old woman arrived at our clinic with a slow-growing nodule in the buccal region, next to tooth 45. The patient had a 9-year history of a diagnosis of mucosa-associated lymphoid tissue-type lymphoma in the gastrointestinal region. She reported pain on palpation. In the intraoral examination, a submucosal nodule, sessile, of firm consistency and with the color of the mucosa was observed. The lesion did not appear on imaging exams. An excisional biopsy was performed under local anesthesia, because of the benign aspect, with a clinical diagnosis of pleomorphic adenoma. The histologic analysis showed atypical lymphocyte cells. Furthermore, the specimen showed positivity for CD20, Ki-67, and BCL-2. The diagnosis was of a lesion suggestive of MALT lymphoma. The patient was referred to and oncologist, where she underwent a computed tomography scan, but no other injured sites were found. The patient is under medical supervision.

Gastric marginal zone B-cell lymphoma of MALT type – An updated histo- and molecular pathological summary

Gastric marginal zone lymphoma (gMZBL) is the most frequent type of extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT). The histology of gMZBL shows typical small cell morphology, however, there also exists a large cell variant.

Gallbladder Burkitt’s lymphoma mimicking gallbladder cancer: A case report

BACKGROUNDMalignant lymphoma is a rare form of gallbladder malignancy. Most of these malignancies are diffuse large B-cell lymphomas or mucosa-associated lymphoid tissue-type lymphomas; however, Burkitt’s lymphoma of the gallbladder is extremely rare, and only two previous reports are available in the literature. Herein, we report a rare case of Burkitt’s lymphoma of the gallbladder mimicking gallbladder adenocarcinoma.CASE SUMMARYAn 83-year-old man with no abdominal complaints was found to have a gallbladder tumor and periportal lymph node enlargement on computed tomography (CT) performed for hypertension screening. His laboratory data revealed slightly elevated serum levels of carcinoembryonic antigen and soluble interleukin 2 receptor. Imaging examinations revealed two irregular and contrast-enhanced masses extending into the gallbladder lumen, but these did not infiltrate the serosa. Moreover, a periportal lymph node had enlarged to 30 mm. Based on these findings, we diagnosed the patient as having gallbladder adenocarcinoma with lymph node metastasis, which was treated using bile duct resection with gallbladder bed resection and periportal lymph node dissection. However, the patient was finally diagnosed as having Burkitt’s lymphoma. Although the surgical margin was pathologically negative, recurrence was noted at the hepatic radical margin and superior pancreaticoduodenal lymph nodes on positron emission tomography/CT soon after discharge. Thus, he was referred to a hematologist and started receiving treatment with reduced-dose cyclophosphamide, doxorubicin, vincristine, and prednisone.CONCLUSIONBurkitt’s lymphoma can occur in the gallbladder. Biopsy can be useful in cases with findings suggestive of gallbladder malignant lymphoma.

Safety of COVID-19 vaccination in patients with clonal mast cell disorders

Safety of COVID-19 vaccination in patients with clonal mast cell disorders

O29 Keeping a watchful eye: IgG4-positive lymphoma masquerading as IgG4-related ocular disease

Case report - IntroductionIgG4-related disease (IgG4-RD) is increasingly recognised in rheumatology practice. We describe a case of orbital swelling initially diagnosed as IgG4-RD. It was exquisitely steroid responsive and she was treated with 6 years of immunosuppression but her symptoms later deteriorated. She developed worsening left eye swelling that required debulking surgery; histopathology demonstrated IgG4-positive lymphoma. She had an excellent response to localised radiotherapy and remains in remission. Although her initial presentation was typical of IgG4-RD, her subsequent lack of response to steroids prompted reconsideration of the initial diagnosis and re-examination of her previous biopsy specimen, confirming IgG4-positive Iymphoma at both timepoints.Case report - Case descriptionA 25-year-old lady presented in 2008 with a swollen left eye. She had a history of childhood eczema, asthma and sinusitis. Diagnostic excision was undertaken; histology was considered to show chronic inflammation, scattered eosinophils, dense fibrosis and no granulomas. She was diagnosed with a ‘granulomatosis with polyangiitis-like’ orbital pseudotumour. It was exquisitely steroid responsive; mycophenolate mofetil was added from an early stage of treatment.The patient was referred to rheumatology 3 years after diagnosis as she was unable to reduce her prednisolone below 5mg daily. Her immunology screen was negative. Although her plasma IgG4 levels were normal at 0.14g/L, the original histology was reviewed and immunostained for IgG4. This demonstrated more than 80% of plasma cells to be IgG4 positive, with the previously noted dense fibrosis and chronic inflammatory infiltrate. Her diagnosis was revised to orbital IgG4-RD. She commenced azathioprine as she was planning a pregnancy.Following an uncomplicated pregnancy in 2014, her symptoms significantly deteriorated, with worsening pain, discharge and swelling in her left eye. A repeat MRI scan showed a new cuff of abnormal tissue within the lateral aspect of the left orbit. She was referred to ophthalmology and underwent left anterior orbitotomy with debulking. Histopathology at this time was consistent with extranodal marginal zone lymphoma (EMZL) of MALT (mucosa-associated lymphoid tissue) type, IgG4 positive. An extraordinary feature was an abundance of crystal-storing histiocytes, presumed to contain ingested IgG4.To investigate whether she had an IgG4-secreting lymphoma from the beginning or whether there had been malignant transformation of her orbital IgG4-RD, the histology from 2009 was reviewed. This was considered to indicate a MALT-type EMZL.The patient was referred to haematology and she was treated with localised radiotherapy with an excellent response. She remains in remission following this and she has subsequently been discharged from haematology follow-up.Case report - DiscussionIgG4-RD is a condition involving fibroinflammatory lesions that can affect any organ in the body. As a newly recognised condition, the role of IgG4 in IgG4-RD is still not fully understood. Questions that remain unanswered include whether or not IgG4 is directly pathogenic and whether there is an increased risk of malignancy in patients with IgG4-RD.Our patient’s case demonstrates how IgG4-positive lymphoma can mimic IgG4-RD. It is important to keep a broad differential diagnosis in complex cases, and to support the eventual diagnosis with blood results, imaging studies and histology. It is essential to involve colleagues in radiology and histopathology so that a multidisciplinary decision can be made with regards to management. IgG4-RD is a steroid-responsive condition. When there is poor response to conventional treatment, this should raise doubts regarding the diagnosis, and it would be prudent to consider a repeat biopsy as well as obtaining previous pathology specimens so that they can be re-reviewed.Ocular adnexal lymphomas form 1—2% of non-Hodgkin lymphomas and 8% of extranodal lymphomas. Over recent decades the incidence has increased, partly reflecting better diagnostic techniques. The most common subtype of ocular adnexal lymphomas are EMZL of MALT type, as in our patient.There are several case reports of lymphoma arising in patients with established IgG4-RD, raising the question of potential lymphomagenesis in IgG4-RD. The earliest reports come from Asia and most have been reported in patients with ocular IgG4-RD. However, the link between malignancy and IgG4-RD is still being investigated. It is important to consider a potential malignancy when managing patients with IgG4-RD, especially if there is a lack of response to steroids.Case report - Key learning pointsThere is no single diagnostic test for IgG4-RD. Diagnosis should be based on the overall clinical picture, supported by blood results, imaging and histopathology.IgG4-RD is a steroid-responsive disease. If there is a poor response to treatment, the diagnosis should be re-considered.Re-examination of previous biopsy specimens can be invaluable, especially in patients refractory to standard treatment.The link between IgG4-RD and malignancy has not been established and it is important to remain vigilant. As ocular adnexal lymphomas are not uncommon, it is important to keep this differential diagnosis in mind when reviewing patients with eye swelling thought to be secondary to IgG4-related ocular disease.

Is it Worthwhile to Undergo the Extended Stage Assessment in Orbital and Ocular Adnexal MALT Lymphoma with Symptoms Limited to the Ocular Adnexa?

Compared to ocular adnexal lymphoma of mucosa-associated lymphoid tissue-type (OAML) patients with symptoms related invasion beyond the ocular adnexa, the different approaches to staging assessment may be required in OAML patients with symptoms limited to the ocular adnexa. Seventy-six patients to be diagnosed with OAML and performed bone marrow biopsy and imaging study at Yeungnam University Hospital in 1995-2014 were enrolled. Patients with symptoms limited to the ocular adnexa were included. Computed tomography, magnetic resonance imaging, positron emission tomography (PET), and bone marrow biopsies were performed for diagnosis, NM staging and follow up evaluation. Most patients were treated with external beam irradiation (median dose, 30Gray (Gy)).The relapse-free survival (RFS) was analyzed according to the tumor laterality and TNM stage. The median follow-up period was 72months. The 5-year RFS and overall survival rates were 82.1% and 95.6%respectively.Of all76patients, lymph node and bone marrow involvement was identified in 1 patient, respectively. Among the 3 patients withT4 stage as tumor invasion beyond ocular adnexa, bone marrow involvement was confirmed in a patient with left cheek invasion. Only 11 of the 43 patients who underwent PET showed positive uptakes in orbital lesion. The patients with advanced stage were alive without recurrences. Bone marrow examination is useful in OAML patients with T4 for extended stage assessment. The AJCC TNM staging system was not significantly predictive factor for relapse, but may contribute to clarifying the patient group that needs bone marrow study.

S1798 Composite Lymphomas in the Colon: Two Separate Entities, Treated as One

INTRODUCTION: Composite lymphoma (CL) is defined as two histologically distinct variants of malignant lymphoma occurring in the same organ or mass. Our case describes an asymptomatic patient with two extranodal B-Cell lymphomas discovered after routine colonoscopy. Further, we discuss pathogenesis and treatment philosophy of follicular lymphoma and marginal cell lymphoma. CASE DESCRIPTION/METHODS: A 62 year old Japanese female with multiple medical morbidities including cirrhosis and hypothyroidism presented for surveillance colonoscopy. On presentation, patient denied any fever, chills, night sweats, unintentional weight loss, hematochezia, or melena. Colonoscopy revealed multiple sessile polyps, 2−5 mm in size. H&E and immunohistochemical staining of specimens demonstrated grade 3A follicular lymphoma with 100% follicular pattern and positron emission tomography/computed tomography (PET/CT) demonstrating a prominent, isolated pericaval lymph node. Per NCCN guidelines for follicular lymphoma grade 3A, stage II (non-contiguous), observation with follow up PET/CT and colonoscopy was recommended. Repeat colonoscopy after 6 months revealed multiple polyps with demonstrating extranodal marginal zone b-cell lymphoma of Mucosa-Associated Lymphoid Tissue (MALT). The diagnosis of MALT type lymphoma was rendered based on histologic patterns of infiltration, the cellularity of the infiltrate, their locations, presence of clear cells, and immunostaining. Of note, repeat PET/CT was negative for lymphadenopathy. 6 months later, another colonoscopy was performed, and 6 more polyps were biopsied. Histological examination was consistent with tubular adenomas and follicular lymphoid proliferation. Given that she was clinically stable with minimal disease, plan was to continue monitoring with colonoscopy and PET/CT annually. DISCUSSION: CLs are defined as 2 or more morphologically and/or immunophenotypically different components in the same anatomic site. They can arise synchronously or metachronously. CLs are rare, with a frequency ranging from 1 to 4.7% of lymphoma cases. More rarely, CL has seldom been witnessed in the gastrointestinal tract. To our knowledge, this is the first case reporting composite follicular lymphoma and extranodal marginal zone b-cell lymphoma of MALT in the colon. Current literature suggests the components of CLs progress as separate entities and treatment should be focused on the component with the least favorable prognosis.

Bilateral Conjunctival Mucosa-Associated Lymphoid Tissue Type Lymphoma in a Kidney Transplant Recipient

Bilateral Conjunctival Mucosa-Associated Lymphoid Tissue Type Lymphoma in a Kidney Transplant Recipient

P2.04-004 Thymectomy without Definitive Diagnosis Could Be Feasible in Patients with Suspicious of Thymic Epithelial Tumor: Topic: Thymic Malignancies Clinical and Translational

As for thymic epithelial tumors (TETs), National Comprehensive Cancer Network guideline has suggested that complete excision of tumor should be performed without preoperative biopsy when resectable. However, there have been very few evidences on this strategy of diagnosis and treatment. The purpose of this study is to evaluate the validity of radical resection of anterior mediastinal masses (AMMs) without pathological confirmation. Two hundred and fifty-eight patients with AMMs underwent surgical resection between 2004 and 2015 at the Nagoya University Hospital. Among them, 186 patients were suspected to have TETs by clinical features, serum tumor markers, and the findings of computed tomography (CT) and positron emission tomography (PET). We retrospectively reviewed cases of the patients with AMMs and evaluated the strategy of treatment for them. Of the186 patients with suspicious of TETs, 56 patients received preoperative biopsy and had the pathological diagnosis. The method included CT-guided needle biopsy in 49 patients (26%) and video-associated thoracic surgery biopsy in 4 (2%) to plan neoadjuvant therapy and/or to distinguish from malignant lymphomas or malignant germ cell tumors, and intraoperative pathologic examination using frozen section of the tumor in 3 (1.6%). The remaining 130 patients (70%) underwent thymectomy without pathological confirmation. Among them, the tumors in 115 patients (88%) were finally diagnosed as TETs including 100 thymomas, 11 thymic carcinomas and 4 thymic carcinoids. The patients except one received complete resection. The remaining 15 patients (12%) were diagnosed as 4 thymic cysts, 4 lymphomas of mucosa-associated lymphoid tissue type (MALT), 2 bronchogenic cysts, 2 mature teratomas and 3 other tumors. Thymic cysts with thick wall in part and small MALT lymphomas with intermediate accumulation of PET were sometimes difficult to distinguish from TETs preoperatively. Eighty-eight percent of the patients with suspicious of TETs who underwent thymectomy without biopsy were accurately diagnosed and properly treated with complete resection. Thymectomy without a definitive diagnosis could be feasible in patients with suspicious of TETs when they are considered resectable, although there are some tumors such as thymic cyst and MALT lymphoma hard to distinguish from TETs.

A retrospective analysis of clinical pathological characteristics and prognosis of 82 patients of primary intestinal lymphoma

Background: To evaluate the histological type, clinical presentation, treatment and prognosis of primary intestinal lymphoma (PIL). Methods: From 2003 to 2015, in a single institution in China, 82 patients with a diagnosis of PIL were enrolled in our study. According to World Health Organization (WHO) classification and Lugano staging system for gastrointestinal lymphoma, we divided patients into different subtypes and stages. Results: Male to female ratio was 1.8:1 and the median age was 59.5 years. Of the 82 patients, 68 patients (82.9%) suffered from intestinal B-cell lymphoma (IBCL) and 14 patients (17.1%) suffered from intestinal T-cell lymphoma (ITCL). Diffuse large B cell lymphoma (DLBCL) was the predominant histological subtype (59.8%) in all of the PIL cases. Mucosa-associated lymphoid tissue-type lymphoma (MALT) was the second most common subtype (15.9%). From the study we also concluded that PIL mostly occurred in the small intestine (45.1%), followed by colon and rectum (26.8%). The univariate prognosis analysis revealed that Lugano stage I/II, B-cell phenotype and treatment based on surgery plus chemotherapy were independent prognostic factors (P=0.003, 0.000 and 0.000, respectively). According to multivariate analysis, age of onset, Lugano stage, immunophenotype and the method of treatment were significant predictive factors for better survival (P=0.005, 0.005, 0.000 and 0.000, respectively). Conclusions: The patients with younger age of onset, B cell lymphoma, earlier stages, and treatment based on surgery plus chemotherapy have a better prognosis. To ensure the diagnosis of PIL to be made as soon as possible, we should make efforts to select the proper laboratory parameters and imaging findings of PIL.

Chemoimmunotherapy for Mucosa-Associated Lymphoid Tissue-Type Lymphoma: A Review of the Literature.

Biological treatments, chemoimmunotherapy, and radiotherapy are associated with excellent disease control in both gastric and extragastric mucosa-associated lymphoid tissue (MALT) lymphomas. Systemic treatment approaches with both oral and i.v. agents are being increasingly studied, not only for patients with disseminated MALT lymphoma, but also for those with localized disease. To date, however, recommendations for the use of available systemic modalities have not been clearly defined. The present report reviews the current data on systemic treatment options for patients with MALT lymphoma and provides recommendations for their use in everyday practice. Different chemotherapeutic agents, including anthracyclines, alkylators, and purine analogs, have been successfully tested in patients with MALT lymphoma. Reducing side effects while maintaining efficacy should be the main goal in treating these indolent lymphomas. From the data from the largest trial performed to date, the combination of chlorambucil plus rituximab (R) appears to be active as first-line treatment. Similarly, R-bendamustine also seems to be highly effective, but a longer follow-up period is needed. R-monotherapy results in lower remission rates, but seems a suitable option for less fit patients. New immunotherapeutic agents such as lenalidomide (with or without rituximab) or clarithromycin show solid activity but have not yet been validated in larger collectives. Patients with MALT lymphoma should be treated within prospective trials to further define optimal therapeutic strategies. Systemic treatment is a reasonable option with potentially curative intent in everyday practice. Based on the efficacy and safety data from available studies, the present review provides recommendations for the use of systemic strategies.

AUY922 effectively targets against activated B cell subtype of diffuse large B-cell lymphoma and low-grade lymphoma cells harboring genetic alteration-associated nuclear factor-κB activation

Recurrent genetic alterations that are frequently observed in some low-grade lymphomas, such as activated B cell subtype of diffuse large B-cell lymphoma (ABC-DLBCL) and mucosa-associated lymphoid tissue type lymphoma (MALT lymphoma) are usually associated with nuclear factor-κB (NF-κB) activation and confer resistance to therapy. In this study, we investigated the therapeutic efficacy and molecular mechanisms of AUY922, a novel Hsp90 inhibitor, in representative cell lines OCI-Ly3 (ABC-DLBCL) and MA-1 (a low-grade lymphoma cell line with t(14;18)/IgH-MALT1translocation) to explore its potential use in the treatment of refractory B-cell lymphoma. Our results showed that AUY922 effectively induced growth inhibition and apoptosis of OCI-Ly3 and MA-1 cells, which were accompanied by down-regulation of the expression levels of NF-κB and Bcl-2 family proteins, as well as molecules of multiple signaling pathways involving cell proliferation, growth and survival. The growth inhibitory effect of AUY922 was further confirmed in a mouse xenograft model. These findings indicate the potential use of AUY922 in B cell lymphomas.

Endoscopic ultrasound-guided deep and large biopsy for diagnosis of gastric infiltrating tumors with negative malignant endoscopy biopsies.

To assess the diagnostic yield and safety of a deep and large biopsy technique under the guidance of endoscopic ultrasound (EUS) for diagnosis of gastric infiltrating tumors with negative malignant endoscopy biopsies. From January 2009 to March 2014, 36 patients in whom gastric infiltrating tumors had been diagnosed by EUS received negative results for malignancy after endoscopic biopsies. The deep and large biopsy technique combined bite-on-bite technique with or without endoscopic mucosal resection (EMR) to obtain submucosal tissue from lesions. EUS was used to select the appropriate biopsy sites. If the lesion protruded into the cavity, EMR was performed for removal of the overlying mucosa and then bite-on-bite technique was conducted in the resected area to obtain submucosal tissue. If the lesion appeared to be flat or was difficult to lift by injection, the bite-on-bite technique was directly used. Twenty-eight of the 36 patients were treated by EMR followed by bite-on-bite technique, while 8 patients only underwent bite-on-bite technique. Histological results showed 23 of the 36 lesions were poorly differentiated adenocarcinomas, 2 diffuse large B cell lymphomas, 4 mucosa-associated lymphoid tissue-type lymphomas, and 7 undiagnosed. The deep and large biopsy technique provided a definitive and conclusive diagnosis in 29 (80.6%) of the 36 patients. The 12 gastric linitis plastica and 6 lymphoma patients received chemotherapy and avoided surgery. Minor oozing of blood in 2 mucosal resection wounds was managed by argon plasma coagulation and in 5 cases after deep biopsies by epinephrine (0.001%). Neither severe hemorrhage nor perforation occurred in any patient. The deep and large biopsy technique is superior to ordinary endoscopic biopsy for achieving an accurate diagnosis of gastric infiltrating tumors. This procedure guided by EUS is an effective and safe diagnostic method for gastric infiltrating tumors in which endoscopic biopsy results were negative for malignancy.

11. A case of extranodal marginal zone lyphoma of mucosa-associated lymphoid tissue (MALT) type and hepatocellular carcinoma

Primary hepatic mucosa-associated lymphoid tissue (MALT) lymphoma is a rare disease. MALT lymphoma is a type of low grade extranodal B-cell lymphoma which is commonly diagnosed in the stomach where there is a well-documented association with Helicobacter pylori infection. In reported cases of primary liver MALT, it is often an incidental finding during imaging or surgery for other disease and patients usually do not have symptoms specifically related to the lymphoma. The information available from reported cases of primary liver MALT lymphoma have included associated inflammatory diseases of the liver including hepatitis B or hepatitis C (either with or without cirrhosis) or primary biliary cirrhosis. This supports the role of chronic inflammation in the pathogenesis of the condition regardless of primary site. As primary hepatic MALT lymphoma is usually asymptomatic it is likely to be an under-reported diagnosis. Only one case of concurrent hepatocellular carcinoma with hepatic MALT lymphoma on a background of hepatitis B infection is reported in the literature. We present a case of a 62 year old patient who presented with non-cirrhotic hepatocellular carcinoma on a background of cleared chronic hepatitis B infection. The patient has positive hepatitis B core antibody with negative hepatitis B surface antigen. The right hepatectomy specimen showed moderately differentiated hepatocellular carcinoma as well as an incidental diagnosis of extranodal marginal zone lymphoma of MALT type and the gallbladder specimen also showed involvement. We present this additional case of concurrent hepatocellular carcinoma and hepatic MALT lymphoma to bring to attention this extremely rare entity.

Detection rate of fluorine-18-fluorodeoxyglucose positron emission tomography in patients with marginal zone lymphoma of MALT type: a meta-analysis.

The aim of this article is to meta-analyse published data about the detection rate (DR) of fluorine-18-fluorodeoxyglucose ((18) F-FDG) positron emission tomography (PET) and PET/computed tomography (CT) in the evaluation of patients with marginal zone lymphoma of the mucosa-associated lymphoid tissue (MALT). A comprehensive literature search of studies published through February 2014 was performed. Pooled DR of (18) F-FDG PET or PET/CT including 95% confidence intervals (95% CI) was calculated on a per-patient-based analysis. Twenty studies including 376 patients with MALT lymphoma were selected. The pooled DR of (18) F-FDG PET or PET/CT was 71% (95% CI: 61-80%). A significant difference between the DR of PET/CT (69%; 95% CI: 61-80%) and that of PET alone (73%; 95% CI: 60-84%) was not demonstrated. A better DR of (18) F-FDG PET or PET/CT in bronchial (94%; 95% CI: 85-99%) and head-and-neck (90%; 95% CI: 78-98%) MALT lymphomas compared with gastric (62%; 95% CI: 46-77%) and ocular (49%; 95% CI: 36-63%) MALT lymphomas was found. This meta-analysis demonstrates that MALT lymphoma is an (18) F-FDG-avid tumour in most of the cases, suggesting a potential clinical role of (18) F-FDG PET or PET/CT in the initial evaluation of these patients. In particular, the DR of (18) F-FDG PET or PET/CT is related to the primary site of the MALT lymphoma.

Cutaneous Lymphomas

: Primary cutaneous B-cell lymphomas (PCBCL) are the second most common form of primary cutaneous lymphomas and account for approximately 25%-30% of all primary cutaneous lymphomas. Both forms of low-grade malignant PCBCL, primary cutaneous follicle center lymphoma (PCFCL) and primary cutaneous marginal zone lymphoma of mucosa-associated lymphoid tissue-type (MALT lymphoma) (PCMZL) represent the vast majority of PCBCL and show an indolent slowly progressive course and an excellent prognosis despite a high recurrence rate. Genetic analysis indicates that PCMZL differ from other forms of extranodal MALT lymphomas. The more common class-switched and the non-class-switched form of PCMZL can be distinguished as two distinctive subsets that differ in the cellular composition, IgM expression, and biological behavior with extracutaneous involvement found in the non-class-switched form. Recently, unusual clinical and histological forms of PCMZL and PCFCL manifesting with miliary or agminated lesions have been described that are diagnostically challenging. In contrast to PCMZL and PCFCL, primary cutaneous diffuse large B-cell lymphoma, leg type, and other rare forms of large B-cell lymphomas such as intravascular large B-cell lymphoma have an unfavorable prognosis. There is an emerging group of Epstein-Barr virus (EBV)-driven B-cell lymphoproliferations including posttransplant lymphoproliferative disorders and mucocutaneous ulcer occurring in immunocompromised patients and EBV-associated diffuse large B-cell lymphoma of the elderly arising in the setting of senescence-linked immunodeficiency. This review reports on recent findings expanding the spectrum of clinicopathological features, differential diagnostic aspects, and the pathogenesis of PCBCL and discusses the group of EBV-associated B-cell lymphoproliferations involving the skin.

Primary Parotid Gland Lymphomas

Objectives: 1) Describe our experience in the diagnosis and management of the parotid gland lymphomas. 2) Present a rare case of synchronous MALT lymphoma of the parotid and high- grade non-Hodgkin lymphoma of the breast (DLBCL). Primary lymphomas of the salivary glands are rare, consisting less than 5% of all cases of extranodal lymphomas and about 2%-5% of salivary gland neoplasms. The most common subtype in the parotid is marginal zone B-cell mucosa-associated lymphoid tissue type lymphoma (MALT). Methods: We retrospectively reviewed the histopathologic reports of 251 parotidectomies that were performed in our department during the last decade. All patients presented with parotid gland swelling and had no previous diagnosis or symptoms of lymphoma. Management included computed tomography and/or magnetic resonance imaging scan and fine-needle aspiration (FNA). Results: Of the 251 patients, 10 were diagnosed with non- Hodgkin lymphoma (3.98%). Among them, 3 were identified as MALT subtype (30% of parotid lymphomas, 1.2% of all parotid neoplasms). FNA accuracy was 100% for the7 non-Hodgkin lymphomas, but only 33.3% (1 in 3 cases) regarding the MALT subtype. Interestingly, in these two patients, Warthin’s tumor was diagnosed by FNA. In one of the 3 cases of MALT lymphoma we came across a synchronous MALT lymphoma of the left parotid and high-grade non-Hodgkin lymphoma of the right breast (DLBCL). Conclusion: Our data suggest that MALT lymphoma of the parotid gland is rare and could be difficult to diagnose with FNA, probably due to similar underlying cytologic features to benign tumors.

Malt Lymphoma of the Colon: a Rare Occurrence

Non-Hodgkin's lymphoma (NHL) is one of the leading causes of cancer in the US. NHL can be seen in the gastrointestinal tract in extralymphatic presentation. Primary GI lymphomas are rare. When present, they are more common in the stomach and small bowel. The most common histologic subtype affecting the gastrointestinal tract and colon is diffuse large B cell lymphoma. These are generally aggressive and are composed of rapidly proliferating cells of B cell origin. The second most common colorectal lymphoma is mucosa-associated lymphoid tissue (MALT)-associated low-grade B cell lymphoma. MALT-associated lymphomas are low-grade tumors arising from B cells associated with mucosal immunity. MALT is predominantly found in the gastrointestinal tract and plays a vital role in immune surveillance, mucosal regeneration, and in carcinogenesis. In the colon, lymphoid tissue consists of isolated lymphoid follicles which are composed mainly of B lymphocytes. These cells occasionally undergo abnormal proliferation and give rise to lymphoma of the MALT type. Colonic MALT lymphoma is a rare entity of increasing recognition, for which no definitive treatment has been established. There are very few case reports of colonic MALT type lymphoma given its uncommon occurrence, thereby lacking sufficient data required in evaluation, management, and treatment of these patients. We describe a rare case of colon marginal-zone B cell lymphoma of MALT (MALT lymphoma) found on routine colonoscopy. Case Presentation

A Case of Synchronous Lung Adenocarcinoma and Extranodal Marginal Zone B-Cell Lymphoma of Mucosa-Associated Lymphoid Tissue Type

Abstract SESSION TYPE: Cancer Global Case Report PostersPRESENTED ON: Tuesday, October 23, 2012 at 01:30 PM - 02:30 PMINTRODUCTION: Extranodal marginal zone B-cell lymphoma (extranodal MZL) of mucosa-associated lymphoid tissue (MALT) type, also called MALT lymphoma, is a distinct subgroup of non-Hodgkin's lymphoma (NHL). More than two thirds of primary pulmonary NHLs are extranodal MZLs but they account for less than 0.5% of all primary lung neoplasms. Only a few cases of simultaneous lung cancer and pulmonary extranodal MZL have been reported. Also, it is extremely rare that primary extranodal MZL occurs in the large airways. To our knowledge, only one case of lung cancer coexisting with extranodal MZL of the large airway has been reported. We report a case of synchronous lung adenocarcinoma and primary extranodal MZL of the left main bronchus.CASE PRESENTATION: A 60-year old woman was referred to our hospital with a pulmonary nodule in the right upper lung seen on chest radiograph during a routine health checkup. She had never smoked. A chest radiograph showed a nodule in the right upper lung. A chest computed tomographic (CT) scan showed a peripherally located 2.4 × 1.8 cm sized solid nodule in the anterior segment of the right upper lobe. Fluorine 18-labeled fluorodeoxyglucose (FDG)-positron emission tomography (PET)-CT scan revealed abnormal FDG uptake in the right upper lobe lung nodule and in the right hilar lymph node. She underwent CT image-guided percutaneous core needle biopsy of the nodule in the right upper lobe. Additionally, a flexible bronchoscopic examination was performed to identify the endobronchial lesion. At bronchoscopy, a few small variable-sized protrusions were observed in the proximal left main bronchus and a biopsy was performed . The needle biopsy specimens of the right lung nodule showed non-mucinous adenocarcinoma. Immunohistochemical stains were positive for CK7 and TTF-1, and negative for CK20. Histopathology of the bronchoscopic biopsy specimens of the left main bronchus lesions showed diffuse proliferation of atypical lymphoid cells beneath the mucosal epithelium. Immunohistochemical stains showed diffuse strong positive for CD20, and were negative for CKpan and CD10. The bronchial lesion was diagnosed as extranodal marginal zone B-cell lymphoma of MALT type. She underwent right upper lobectomy and lymph node dissection for the lung adenocarcinoma. However, she did not receive specialized therapy for extranodal MZL of the left main bronchus. Since then she has been observed regularly.DISCUSSION: Synchronous primary malignancy and extranodal MZL in the same anatomical sites, such as stomach and lung is very unusual. To our knowledge, there is only one other report showing the coexistence of lung cancer and pulmonary extranodal MZL of the large airway. However, in that case, the histology of lung cancer was squamous cell carcinoma. The present case is the first report of the simultaneous occurrence of lung adenocarcinoma and pulmonary extranodal MZL of the large airway. The cause of the synchronous lesions in our case is uncertain. We postulate that a pulmonary extranodal MZL may coincide with a lung adenocarcinoma by accident. Little is known about the mechanism of the simultaneous occurrence of these different tumors. Moreover, the two different malignancies were located in different lungs in this case. However, other possible causes may include common etiologic factors and genetic or epigenetic alterations participating in the development of both the lung adenocarcinoma and extranodal MZL. Further studies will be required to clarify the relationship between lung adenocarcinoma and pulmonary extranodal MZL. It is necessary to accumulate more cases with both malignancies.CONCLUSIONS: In conclusion, we report a unique case of pulmonary extranodal MZL of main bronchus coexisting with lung adenocarcinoma. The patient was asymptomatic and was diagnosed with pulmonary extranodal MZL by chance during investigation of an endobronchial lesion.1) Jaffe ES, Harris NL, Stein H, Vardiman JW. World Health Organization Classification of Tumors. Pathology and Genetics of Tumors of Haematopoietic and Lymphoid tissue. Lyon: International Agency for Research on Cancer; 2001.DISCLOSURE: The following authors have nothing to disclose: Chi Young Jung, Young June JeonNo Product/Research Disclosure InformationKeimyung University Dongsan Hospital, Daegu, Republic of Korea.

Ophthalmologic outcomes after chemotherapy and/or radiotherapy in non-conjunctival ocular adnexal MALT lymphoma

In the present study, we evaluated the ophthalmologic outcomes of 24 patients who received chemotherapy and/or radiotherapy for the treatment of non-conjunctival ocular adnexal mucosa-associated lymphoid tissue-type (MALT) lymphoma. Ophthalmologic outcomes were assessed in patients who received chemotherapy and/or radiotherapy from March 2004 until May 2010. Outcomes were determined according to common symptoms following chemotherapy and/or radiotherapy, which consisted of decreased visual acuity, dry eye symptoms, retinopathy, optic neuropathy, increased intraocular pressure, and blepharitis. Nine patients received chemotherapy alone, eight patients received radiotherapy alone, and seven patients received chemotherapy with additional radiotherapy (chemoradiation therapy). Patients treated by chemotherapy alone showed better ophthalmologic outcome scores (mean score, 1.56) than those treated by radiation alone or chemoradiation therapy (mean score, 4.01). In conclusion, the treatment of ocular adnexal lymphoma including radiotherapy showed poor ophthalmologic outcomes due to radiation-induced complications. Recently, many new treatment options have emerged, such as immunotherapy or radioimmunotherapy. In the future study, to select a better treatment modality with fewer complications, well-designed prospective trials with ophthalmologic outcomes are needed.