The Dilemma for Early-Stage Conjunctival Mucosa-Associated Lymphoid Tissue Lymphoma: To Treat or Not to Treat?

BACKGROUND: Primary ocular adnexal mucosa-associated lymphoid tissue lymphoma (MALToma) is typically treated with radiotherapy. Some studies suggested a "wait and watch" approach due to the adverse effects of radiotherapy. However, the benefits of observation for localized conjunctival MALToma remain unclear. Therefore, we aimed to explore the clinical course of early-stage conjunctival MALToma, distinguish heterogeneity between T1 and T2 patients, and identify prognostic factors. METHODS: This retrospective study involved patients with stage T1-T2 conjunctival MALToma and lasted >6 months. Clinical characteristics were compared between T1 and T2 subjects. Prognostic factors were examined with Cox regression. RESULTS: The research comprised 32 subjects with early-stage conjunctival MALToma, of whom 25% underwent observation. No individuals expired regardless of choosing observation or radiotherapy. The T1 patients were younger (p = 0.002) and more inclined towards observation only (p = 0.035) than the T2 subjects. Despite more of the T1 patients undergoing watchful waiting than the T2 subjects, the T1 patients seemed to have longer systemic relapse-free survival than the T2 subjects (17 vs. 13 years, p = 0.343). CD43 may imply poor prognosis (p = 0.049). CONCLUSIONS: Careful observation may be suggested for early-stage conjunctival MALToma. While more of the T1 individuals were younger and chose observation than the T2 patients, survival seemed longer in the T1 subjects without significance. CD43 may indicate shorter survival in early-stage cases.

The National Landscapes of Gastric Mucosa-Associated Lymphoid Tissue Lymphoma: Stable Trends in Black Populations and Late-Stage Tumors.

BACKGROUND: Helicobacter pylori (H. Pylori) eradication has been the mainstream for preventing and treating gastric mucosa-associated lymphoid tissue (MALT) lymphoma. Prior data showed disparities in eradication rates of H. Pylori between different populations. This can potentially impact the occurrence of gastric MALT lymphoma. There are limited data on the incidence and mortality rates and trends of gastric MALT lymphoma in the US. Therefore, the aim of the current study was to conduct a time-trend analysis of gastric MALT lymphoma incidence and mortality rates in different populations. METHODS: The incidence rates of gastric MALT lymphoma were calculated from the United States Cancer Statistics (USCS) database (which covers nearly 98% of the US population) between 2001-2020 and were age-adjusted to the standard 2000 US population using SEER*Stat software (version 8.4.3, national cancer institute "NCI"). Incidence-based mortality (IBM) rates, also age-adjusted to the standard 2000 US population, were calculated from the Surveillance Epidemiology and End Results (SEER) database. Tumor location was specified using ICD-O-3 codes C 160-C 169 with malignant behavior. Histopathology was specified using the ICD-O-3 code 9699. The rates were categorized by sex, age, race/ethnicity, and tumor stage at diagnosis. Age groups were older adults (aged 55 years or older) and younger adults (aged younger than 55 years). Race/ethnic groups included Non-Hispanic White (White), Non-Hispanic Black (Black), Hispanic, Non-Hispanic Asian/Pacific Islander (API), and Non-Hispanic American Indian/Alaska Native (AI/AN), as reported in the database. Stage at diagnosis included early stage (in situ and localized tumors) and late stage (regional and distant site tumors). Joinpoint Regression Software (version 5.0.2, NCI) using the weighted Bayesian Information Criteria method was used to generate time trends. Trends were reported as annual percentage change (APC) and average APC (AAPC). Parametric estimations were used with a two-sided t-test to evaluate the trends with a p-value cutoff at 0.05. RESULTS: There were 21,625 patients diagnosed with gastric MALT lymphoma in the US between 2001 and 2020. Overall, incidence rates were significantly decreasing over the study period (AAPC = -1.93). This decrease was seen in males (AAPC = -1.67) and in females (AAPC = -1.66) (Figure). When categorized by age groups, older adults also experienced a significant decrease in gastric MALT lymphoma incidence rates (AAPC = -1.66). While this was also seen in younger adults, the rates were decreasing at a slower pace (AAPC = -1.38). When categorizing the trends by race/ethnicity, incidence rates were significantly decreasing in White (AAPC = -2.09), Hispanic (AAPC = -1.61), and API (AAPC = -3.92) populations. However, the rates were stable among Blacks. While early-stage tumors experienced a significant decrease (AAPC = -1.10), the rates were stable for late-stage tumors. When evaluating mortality, there were 11,036 patients whose death was attributed to gastric MALT lymphoma between 2000 and 2020. IBM rates were decreasing in males (AAPC = -1.47), older adults (AAPC = -1.55), Whites (AAPC = -1.23), Hispanics (AAPC = -1.73), APIs (AAPC = -2.30), and early-stage tumors (AAPC = -1.08). On the other hand, IBM rates were stable in females, younger adults, Blacks, and late-stage tumors. DISCUSSION: An extensive nationwide data analysis encompassing nearly 98% of patients diagnosed with gastric MALT lymphoma in the US unveils a declining trend in the incidence of cancer overall over the past two decades. This decline is observed in both sexes and various age groups. When stratifying by race and ethnicity, this incidence has been decreasing in all populations except among Black individuals. While early-stage tumors have also demonstrated a significant decrease in incidence rates, late-stage tumors have shown no parallel decline. Mortality evaluation also revealed an improvement in most of the US population except among females, younger adults, Black individuals, and late-stage tumors. While the cause of our findings is unclear, it could be driven by disproportionate exposure to risk factors, including H. Pylori, and disparities in screening, management, and outcomes. Future studies are warranted to investigate factors contributing to worse outcomes of gastric MALT lymphoma, especially in the Black population.

A Case of Pulmonary MALToma Camouflaged Within Invasive Pulmonary Aspergillosis.

A 59-year-old non-smoking male, with a known case of COPD (chronic obstructive pulmonary disease), treated pulmonary tuberculosis with Category 1 antitubercular drugs (six-month regimen) and was admitted with repeated bouts of moderate haemoptysis (~60 mL/day) for three days. The patient had a history of self-limiting occasional mild haemoptysis (~20 mL) over three years. An HRCT chest revealed a left upper lobe fibro-cavitary lesion with an intracavitary mass (air crescent sign), adjacent pleural thickening and fibrosis. Bronchoalveolar lavage (BAL) was positive for galactomannan and negative for Mycobacterium tuberculosis GeneXpert®. With the above clinical factors, host factors, and microbiological factors, the case was diagnosed as 'probable' invasive pulmonary aspergillosis and was treated with voriconazole. However, given relapsing haemoptysis despite adequate antifungal treatment, a left upper lobectomy was done. The resected left upper lobe specimen culture demonstrated Aspergillus fumigatus with histopathology confirming hyphae invading lung tissues confirming 'proven' invasive aspergillosis. Resected tissue also showed florid lymphoid tissue hyperplasia with Immunohistochemistry confirming the presence of a peculiar malignancy; MALT lymphoma/MALToma in the resected lobe. The association of a rare malignancy such as MALToma with invasive pulmonary aspergilloma (IPA) has been identified and reported for the first time. This could be because of a chronic inflammatory reaction elicited by the Aspergillus antigen. Long-standing fibro-cavitary disease and aspergillosis are partners in crime, augmenting the damages inflicted by one another. In such a scenario, early surgical intervention may be warranted if haemoptysis is moderate to severe or relapsing, following conservative medical management. Surgical resection may lead to the identification of unexpected diseases as in our case.

Perplexity of Preoperative Multimodal Investigations in a Case of Parotid MALToma.

Mucosa-associated lymphoid tissue(MALT) lymphoma is a distinct subtype of lymphoma, presenting as a diffuse gland involvement or a discrete mass. Pre-operative diagnosis is a challenge as Fine Needle Aspiration Cytology is often inconclusive and presently no radiological investigation is confirmatory, therefore final diagnosis is made after surgical resection and immunohistopathology. We report a case of MALT lymphoma, which clinically presented as a unilateral large diffuse swelling of the parotid gland with a diagnostic dilemma eventually underwent total parotidectomy to be finally diagnosed as MALT lymphoma of parotid gland and received field radiotherapy with complete cure and no recurrence in a 2-year follow-up.

Primary appendiceal MALT lymphoma clinically masquerading as chronic appendicitis: A case report.

INTRODUCTION AND IMPORTANCE: Mucosal associated lymphoid tissue (MALT) lymphomas are a type of extranodal indolent lymphoma. They appear in areas ordinarily devoid of lymphoid tissues and are frequently preceded by chronic antigenic stimulation. Primary MALT lymphoma is an extremely rare variant in the appendix. CASE PRESENTATION: A 22-year-old man presented with recurrent abdominal pain of three months. CT scan of the abdomen showed appendiceal wall thickening with ileo-colic lymphadenopathy. The patient was managed with right hemicolectomy and the histopathological examination showed MALT lymphoma. CLINICAL DISCUSSION: MALT lymphomas of the appendix are extremely rare. Chronic appendicitis is an uncommon but possible clinical presentation. Although imaging techniques are essential for making a diagnosis, histological analysis is what leads to a final diagnosis. While there are no specific recommendations for treating appendiceal MALTomas, prior case reports indicate that appendectomy and surveillance may be sufficient. CONCLUSION: Primary MALT lymphoma is extremely uncommon in the appendix. It is indolent in nature and can manifest clinically as chronic appendicitis. The management for localized disease is surgery or radiotherapy. The prognosis is excellent regardless of the initial treatment modality.

Multiple Synchronous Mucosa-Associated Lymphoid Lymphomas Involving in the Stomach, Duodenum, Ileum, and Sigmoid.

Gastric MALToma is an extra-nodal marginal-type B-cell lymphoma. MALToma may occur secondary to chronic inflammation and autoimmunity. The most common gastrointestinal (GI) site of MALToma is the stomach, with approximately 50% of lesions occurring there. Synchronous upper and lower GI MALTomas rarely occur, with few cases reported. We present the case of a 67-year-old patient who presented asymptomatic and was found to have synchronous multifocal upper and lower GI MALTomas in the stomach, duodenum, terminal ileum, and sigmoid, which did not respond to H. Pylori eradication therapy.

Helicobacter pylori-Negative Gastric Mucosa-Associated Lymphoid Tissue Lymphoma Presenting as Massive Gastrointestinal Bleed.

This case reports a patient that represents the minority of patients with gastric mucosa-associated lymphoid tissue (MALT) lymphoma who do not have underlying Helicobacter pylori gastritis. Gastric MALT lymphoma is a type of primary gastric lymphoma (PGL), which are extremely rare gastric malignancies characterized by proliferation of B-cells and infiltration of lymphoid tissue leading to destruction of gastric glands. Development of gastric MALT lymphoma is associated with H. pylori gastritis. Patients typically present with a wide range of symptoms including but not limited to epigastric pain, weight loss, gastrointestinal bleeding and gastric wall perforation. Gastric MALT lymphoma presenting as a massive gastrointestinal bleed is quite rare and only a few cases have been documented. Our case demonstrates that it is important to recognize that acute presentations of this disease may also occur.

Primary pulmonary mucosa-associated lymphoid tissue lymphoma: A case report.

Primary pulmonary lymphoma (PPL) is a rare entity with the most common presentation as mediastinal lymphadenopathy. The most common form of PPL is Mucosa-Associated Lymphoid Tissue Lymphoma (MALToma) which is an extranodal B-cell lymphoma originating from the mucosal layers involving different organs such as the gastrointestinal tract as well as the lung. Herein, we present a case of a 51-year-old woman with progressive dyspnea for 6 months and no prior medical history. The computed tomography (CT scan) revealed bilateral multifocal consolidation and ground-glass opacities as well as interlobular septal thickening. Bronchoscopy was normal and CT-guided biopsy of lung consolidations was conclusive of MALToma. Complete extrapulmonary evaluations inducing bone marrow aspiration were unremarkable. The primary pulmonary MALToma is an extremely rare entity that presents with non-specific symptoms and a wide variety of CT findings such as mediastinal, hilar lymphadenopathy, and single or multiple lung nodules ranging from 2 to 8 cm. the disease has a favorable prognosis, so prompt diagnosis is essential.

Magnetic Resonance Imaging-Guided Radiation Therapy for Early-Stage Gastric Mucosa-Associated Lymphoid Tissue Lymphoma.

Lymphoid neoplasia derived from mucosa-associated lymphoid tissue (MALT; also abbreviated as MALToma) is most commonly seen in the stomach. Radiotherapy (RT) is indicated in early-stage disease as a standard of care. With the advent of RT techniques, large field irradiation was replaced by involved site and involved field approaches. Magnetic resonance imaging-guided online adaptive RT (MRgRT) has the advantage of better soft tissue visualization, adaptive planning before each fraction, and online tumor tracking during treatment; hence, it could be a safe and effective choice for gastric MALToma patients. Herein, we investigated the interfractional changes in target and the impact of MRgRT on daily dosimetry in a gastric MALToma case. A patient diagnosed with MALToma who failed to respond to antibacterial treatment was referred to our clinic for RT. He was found to be suitable for MRgRT. We treated the patient with MRgRT in 20 fractions to a total dose of 30 Gy. Reoptimized adaptive plans were generated before each fraction since the coverages of the original plan were inadequate in each fraction. The patient showed good compliance and tolerated the treatment well. To our knowledge, this is the first documented case of a gastric MALToma treated with MRgRT. MRgRT is safe and feasible for this patient group with improved target coverage using small planning target volume margins. Without online adaptive planning, the target coverages would be inadequate and we would risk surrounding tissues to get higher doses.

Pulmonary mucosa-associated lymphoid tissue (MALT) lymphoma presented as cystic lung disease - a case report in Tuen Mun Hospital Hong Kong.

We report a rare case of pulmonary mucosa-associated lymphoid tissue lymphoma (MALToma) in a 52-year-old woman presented as cystic lung disease together with ground-glass lesion on computed tomography (CT) of the thorax incidentally found as part of workup for organ donation.

Pyogenic Hepatic Abscess: A Case Report and Literature Review on a Rare Complication of Gastric Sleeve Surgery.

Gastric sleeve surgery is a frequently performed procedure. Although it is one of the safest bariatric procedures, it is like any other operation that carries significant risks and complications. Moreover, the hepatic abscess is an infrequent complication of laparoscopic gastric sleeve surgery, the infected late gastric leakage is a rare etiology of the hepatic abscess. We present a case of liver abscess developed one month after sleeve gastrectomy secondary to infected walled-off late-gastric leak. The case highlights this rare complication of gastric sleeve surgery. Moreover, early treatment of liver abscesses with imaging-guided drainage and intravenous antibiotics can prevent life-threatening outcomes.

Ileal mucosa-associated lymphoid tissue lymphoma diagnosed after emergency surgery: A case report and literature review.

INTRODUCTION AND IMPORTANCE: Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue, also called MALT lymphoma, is one of the entities of marginal zone lymphomas. These lymphomas are originated from indolent B-cell lymphomas and involve many organs such as the gastrointestinal tract, salivary gland, skin, lung, thyroid or breast. Ileal MALT lymphoma is relatively rare and clinical symptoms are usually atypical. CASE PRESENTATION: We report a case of a 99-year-old man who admitted to the emergency department with increasing and colicky periumbilical pain, vomiting and constipation. Non-contrast-enhanced computed tomography suggested small bowel obstruction due to phytobezoar. Intraoperatively, surgeon discovered the tumor at the site of phytobezoar. Histologically, there was a diffuse infiltration comprised of small to medium sized lymphocytes with monocytoid features. Immunohistochemical result confirmed CD20 positive B-lymphocytes and the Ki-67 proliferation index was 10%. Ileal mucosa-associated lymphoid tissue lymphoma was diagnosed based on histological findings and immunohistochemistry. DISCUSSION: MALToma of the gastrointestinal tract is related to chronic antigenic, inflammatory bowel disease and malabsorption syndromes. However, the etiology of ileal MALToma is unclear. Moreover, symptom of ileal MALToma is really not typical and overleaped in the context of small intestinal obstruction. It should be differentiated small intestinal MALToma from immunoproliferative small intestinal disease and an alpha heavy chain disease. CONCLUSION: Ileal MALT lymphoma remains little known in many previous studies. It is really difficult to preoperatively diagnose. The combination of clinical presentation, postoperative histology and immunohistochemistry contribute to diagnosis and carry out appropriate management.

Obesity Is a Risk Factor Associated with H. pylori-negative MALT Lymphoma of Stomach.

OBJECTIVE: More than 90% of gastric mucosa-associated lymphoid tissue (MALT) lymphomas are attributed to Helicobacter pylori infections. However, the pathogenesis of H. pylori-negative MALT lymphomas is controversial, and additional etiologies need to be investigated. MATERIALS: A retrospective study of gastric MALT lymphoma cases over a 15-year period revealed 56 cases. The H. pylori status, clinical information, and body mass index (BMI) data were collected. The results of the urea breath test, serology, stool antigen, and previous biopsy results were documented. RESULTS: The 56 cases had an average height of 166.57 cm (range, 147.3-190.5), weight of 83.98 kg (range 55-153.1), and body mass index (BMI) of 30.34 kg/m2 (range, 17.96-49.77). Twenty-one cases were H. pylori-positive (37.5%), with a mean BMI of 27.36 kg/m2 (range, 17.96-47.25), and BMI>30 kg/m2 in 5 (23.8%) patients. Thirty-five cases were H. pylori-negative, with a mean BMI of 31.90 kg/m2 (range, 18.17-49.77), and 20 (57.1%) having BMI>30 kg/m2. A Fisher's exact test and two-tailed test showed a statistically significant difference between the two groups. CONCLUSION: Obesity leads to a baseline state of chronic inflammation and increased production of pro-inflammatory cytokines that can stimulate the lymphocytes, leading to lymphomatous proliferation. Our study suggests a potential correlation between obesity and the risk of development of primary gastric MALT lymphoma.

Primary pulmonary marginal zone lymphoma: an unusual cause of pulmonary infiltrates.

Primary pulmonary extra-nodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma), also known as bronchus-associated lymphoid tissue (BALT lymphoma), is the most common primary pulmonary lymphoma but is rare (<1%) among all non-Hodgkin lymphomas and among pulmonary neoplasms in general. We herein report the case of a 59-year-old male who presented with stable exertional dyspnoea and persistent lung infiltrates who was referred to our hospital for further assessment. A computed tomography (CT)-guided core biopsy was performed showing a dense lymphoid infiltrate, with further testing revealing the diagnosis of pulmonary MALT lymphoma. This uncommon lung tumour is usually seen in older adults and typically associated with a relatively indolent course. Rituximab, an anti-CD20 antibody, has been shown to be effective in up to 70% of cases.

Right Middle Lobe Single Pulmonary Opacity With Air Bronchogram: A Problem-Solving Case Report.

Marginal zone lymphoma (MZL) is a relatively uncommon subtype of non-Hodgkin lymphoma consisting of extranodal, nodal, and splenic MZL. Mucosa-associated lymphoma constitutes the majority of extranodal MZL including but not limited to the stomach, lung, salivary gland, ocular adnexa, skin, and thyroid. Depending on the site of origin, maltoma may present with different symptoms. Here we present a patient who presented to the pulmonary clinic for further evaluation of a right middle lobe consolidation. She was treated with a course of antibiotics empirically with no interval change in imaging. She underwent bronchoscopy with biopsy. Pathology was remarkable for extensive lymphoid infiltrates consisting of mixed B and T lymphocytes. Positron emission tomography (PET) CT demonstrated mild uniform uptake in consolidation with no evidence of PET avid distant metastasis. CT-guided biopsy was consistent with extranodal marginal zone lymphoma. She underwent right middle lobectomy with no complications. As mentioned in our case, bronchoscopy is usually nondiagnostic and a lung biopsy is needed in pulmonary maltoma. Treatment is based on the tumor location and extent of the disease. Prognosis is good with an 86-95% five-year survival.

Maltoma veiled in the lung-a rare case of pulmonary and gastric maltoma.

Mucosa associated lymphoid tissue (MALT) is a type of B-cell lymphoma that is commonly observed in the gastrointestinal site, most frequently occurring in the stomach. However, the incidence of this type of lymphoma in the respiratory tract is very uncommon. We report a case of this rare clinical entity in a patient who presented with non-symptomatology and was diagnosed with pulmonary MALT lymphoma (pMALToma).

Rituximab-induced Ileocolitis in a Patient with Gastric MALToma: A Case Report and Literature Review.

Rituximab (RTX) is effective for treating cancer, but reports of RTX-associated enterocolitis are limited. We herein report the case of a 65-year-old man who developed RTX-induced ileocolitis. He was diagnosed with gastric mucosa-associated lymphoid tissue lymphoma (MALToma) and treated with RTX. He complained of bloody diarrhea after RTX. Mucosal inflammation on colonoscopy indicated RTX-induced ileocolitis. He was treated with corticosteroids, and his symptoms improved. We reviewed the RTX-associated gastrointestinal adverse events and classified the features into ulcerative colitis, Crohn's disease, microscopic colitis, and ileocolitis. To our knowledge, this is the first case of a Japanese patient who developed RTX-induced ileocolitis.

DLBCL of bilateral submandibular glands and MALToma of thyroid-A rare coexistence.

Involvement of the bilateral submandibular glands and thyroid by the same lymphoma simultaneously has been reported in the literature. However, two different types of lymphomas presenting simultaneously at different sites have never been reported. This case report highlights this rare occurrence. A 65-year-old female, a known case of Hashimoto's thyroiditis with raised anti-TPO antibodies, presented with thyroid swelling for 1 year and bilateral submandibular swelling for 3 years. FNAC and flow cytometry showed features of mucosa-associated lymphoid tissue lymphoma in the thyroid gland, whereas the bilateral submandibular glands showed features of diffuse large B cell lymphoma. Histopathology and immunohistochemistry from the submandibular swelling led to similar diagnoses as the flow cytometry.

Child With Extranodal Marginal Zone B-cell Lymphoma of Mucosa-associated Lymphoid Tissue.

Many patients present to our clinic with Helicobacter pylori (H. pylori) infection. Most have nonspecific symptoms that cannot be immediately attributed to H. pylori. The joint European Society for Paediatric Gastroenterology, Hepatology, and Nutrition (ESPGHAN)/North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition (NASPGHAN) guidelines recommend upper gastroscopy to detect the cause of such symptoms. Herein, we present the case of a 9-year-old girl diagnosed with gastric mucosa-associated lymphoid tissue lymphoma associated with H. pylori infection using upper gastroscopy. We believe that a patient of such a young age with this serious condition secondary to H. pylori will highlight the importance of upper gastrointestinal endoscopy in such cases.