Bronchus-associated Lymphoid Tissue Lymphoma Research Articles

Long-term survival outcomes of 'watch and wait' in patients with bronchus-associated lymphoid tissue lymphoma: a multicenter real-world data analysis in Korea.

Bronchus-associated lymphoid tissue (BALT) is a rare cause of extranodal marginal zone lymphoma (MZL). Although most patients with BALT lymphoma (BALToma) show an indolent clinical course and are monitored without treatment, there are limited real-world data on the long-term outcome of "watch-and-wait' strategy in comparison with other treatments.The survival outcomes of patients newly diagnosed with BALToma at three tertiary hospitals in Korea undergoing two treatment strategies were analyzed: group A, patients who were monitored without any treatment or received only radiotherapy after diagnosis; and group B, patients receiving any kind of systemic chemotherapy after diagnosis, regardless of their history of any local treatment such as surgery or radiotherapy.Of the 67 patients included in our analysis, the 10-year progression-free survival (PFS) and 10-year overall survival (OS) rates were 65.3% and 83.2%, respectively. The 10-year PFS rates for observation or localized treatment and systemic chemotherapy were 78.7% and 56.9%, respectively (p = 0.044). Ten-year OS rates for observation or localized treatment and systemic chemotherapy were 100% and 71.7%, respectively (p = 0.016). Multivariate analysis showed that bilateral lung (HR 2.462, p = 0.047) and extrapulmonary organ (HR 4.485, p = 0.004) involvement were the only significant factors associated with poor PFS. Prognostic factor analysis for OS did not yield significant results.Patients with BALToma show a favorable prognosis, suggesting that observation or localized therapy alone may be effective for patient management. However, patients with bilateral lung or extrapulmonary involvement may require careful monitoring for disease progression and more aggressive treatment approaches.

Treatment outcome, toxicity, and quality of life of patients with bronchus-associated lymphoid tissue lymphoma

The disease failure patterns and optimal treatment of bronchus-associated lymphoid tissue (BALT) lymphoma are unknown. This retrospective study involved 71 patients with primary BALT lymphoma who had received radiotherapy (RT), surgery, immunochemotherapy (IC), or observation. The median follow-up time was 66 months. The 5-year overall survival and lymphoma-specific survival were 91.2% and 96.1%, respectively, and were not significantly different among treatments. The 5-year cumulative incidence of overall failure for RT, surgery, IC, and observation was 0%, 9.7% (p = .160), 30.8% (p = .017), and 31.3% (p = .039). There was no grade ≥3 toxicity in RT group according to the CTCAE 5.0 reporting system. Quality of life (QoL) was at similarly good levels among the treatment groups. BALT lymphoma had a favorable prognosis but persistent risk of relapse after IC or observation. Given the very low disease failure risk and good QoL, RT remains an effective initial treatment for BALT lymphoma.

Real-World Data of Outcome of Patients with Newly Diagnosed Bronchial-Associated Lymphoid Tissue Lymphoma: A Multi-Center Retrospective Analysis with Long-Term Survivors

Introduction Extranodal marginal-zone lymphoma (MZL) of mucosa-associated lymphoid tissue (MALT) is an indolent type of non-Hodgkin's lymphoma. Although the lung is the second most common site, it accounts for less than 1% of all lymphomas. Extranodal MZL of MALT type of the lungs originates from bronchial-associated lymphoid tissue (BALT) and is clinically referred to as “BALT lymphoma” or “BALToma”. The indolent nature of BALTomas results in none or mild symptoms and their slow growth pattern allows for various treatment approaches. Depending on the case, active surveillance may be considered, while others may benefit from surgery, radiotherapy or chemotherapy. Despite some existing reviews on this lymphoma, comprehensive characterization of optimal management and prognostic factors remains inadequately defined. Limited case reports and small-scale retrospective data analyses have been conducted, but the number of patients included in these studies was highly restricted. In this study, we conducted a retrospective analysis of clinical data from 67 BALToma patients in South Korea. Methods 67 patients were included in our analysis, all of whom were diagnosed with BALToma between September 1996 and January 2023 at three institutions in South Korea. The criteria for case inclusion was histologically confirmed diagnosis of extranodal MZL of pulmonary origin in accordance with WHO classifications, and availability of clinical data. Extranodal MZL was diagnosed based on WHO classifications, along with the findings from immunohistochemical staining for CD20 and CD3. If needed, CD5, CD10, CD23, cyclin D1, BCL6, and Ki-67 was also stained. Gene rearrangement studies targeting the IgH gene were conducted using PCR analysis. Patients were treated according to the stage and location of the disease. The patients were analyzed into two groups; observation or localized treatment and systemic chemotherapy. Localized treatment included surgery or radiotherapy. Systemic chemotherapy regimens used were mainly (R)-CVP and (R)-CHOP. Baseline characteristics was analyzed with Fisher's exact test. Overall survival (OS) and Progression Free Survival (PFS) were estimated using the Kaplan-Meier method. PFS was calculated from the date of diagnosis to the date on which progression was noted, the date of the last follow-up, or the date of death. OS was measured from the date of diagnosis to the date of death or the last follow-up visit. Survival rates were compared for statistical differences via Generalized Wilcoxon analyses. Prognostic factor analysis and hazard ratio was calculated using univariate and multivariate Cox Regression method. All statistical tests were two-sided, and p < 0.05 was considered statistically significant. All data was analyzed using SPSS Statistics for Windows, Version 27.0. Results The clinical characteristics of patients according to treatment modality was analyzed (Table 1). Gender, age, performance status was not significantly different between each group. Notably, involvement of single or multiple lobe (p=0.045), involvement of bilateral lung (p<0.001), nodal involvement (p=0.033), and involvement of extrapulmonary organ (p=0.010) was significantly different between each group. The PFS and OS according to treatment method are provided in Figure 1 and 2. 10-year PFS for observation or localized treatment was 78.7% whereas chemotherapy was 56.9% (p=0.044). 10-year OS for observation or localized treatment was 100%, whereas chemotherapy was 71.7% (p=0.016). The prognostic factors for progression of disease were significant only for the involvement of extrapulmonary organ in both univariate and multivariate (hazard ratio 3.615, p=0.023) analysis (Table 2). Prognostic factor analysis for overall survival did not yield any significant result. Conclusion This study concludes that the most important prognostic factor for the progression of disease is the involvement of extrapulmonary organ. This study implies that the feasibility of simply monitoring patients whose disease is confined exclusively to the lung is a viable strategy. However, in the case of extrapulmonary involvement, the implementation of systemic chemotherapy ought to be deliberated. Given the heightened potential for disease progression, this underscores the necessity for meticulous consideration during the development of therapeutic plans.

Very low-dose radiotherapy for extranodal marginal zone lymphoma of bronchus-associated lymphoid tissue

Extranodal marginal zone lymphoma of bronchus-associated lymphoid tissue (BALT) is a rare cancer for which optimal treatment strategies are undefined. Retrospective analyses suggest excellent outcomes with surgical resection for localized BALT lymphoma; however, the role of radiotherapy remains underexplored. We report the largest-to-date single-center analysis of 13 primary BALT lymphoma patients treated with radiotherapy. Of 15 treated lesions, we report a 100% response rate with complete response (CR) achieved in 67% of lesions. Among 10 lesions treated with very low-dose radiotherapy (VLDRT; 4 Gray [Gy]), 6 (60%) achieved a CR; among 5 lesions treated with full-dose radiotherapy (24–36 Gy), 4 (80%) achieved a CR. There were no local recurrences. Only one patient, treated with 30 Gy, developed an acute grade 3/4 toxic effect. There were no events of radiation-induced secondary malignancies. Our institutional experience indicates that radiotherapy, including VLDRT, is a safe and effective treatment for primary BALT lymphoma.

A single‐center experience of low‐dose radiotherapy for primary extranodal marginal zone lymphoma of bronchus‐associated lymphoid tissue (BALT)

Introduction: Extranodal marginal zone lymphoma of bronchus-associated lymphoid tissue (BALT) is a rare cancer for which optimal treatment strategies are not well defined. Retrospective analyses show equivalent or superior outcomes with surgical resection compared to systemic therapy or active surveillance. Despite a well established role for radiotherapy in the management of other types of extranodal marginal zone lymphoma (MZL), its role in BALT lymphoma is underexplored. Methods: We screened 153 patients with a diagnosis of primary BALT lymphoma treated at a single institution from 1995 to 2022. Inclusion criteria comprised patients aged 18 years or older, histologically confirmed BALT lymphoma, and receipt of radiotherapy at initial presentation or disease progression/recurrence. We excluded patients with synchronous evidence of lymphoma involving non-thoracic extranodal sites and/or lymphadenopathy outside of the mediastinum and hilum, as these patients might represent MZL with secondary lung involvement as opposed to primary BALT lymphoma. All patients underwent baseline PET/CT staging. We extracted patient, tumor, imaging, and treatment characteristics from the electronic medical record. We defined active surveillance as at least 3 months of follow up prior to initiating active therapy combined with documentation of this plan in the medical record. We assessed treatment response using the RECIL 2017 criteria. Results: We report the largest to date single-center retrospective analysis of 13 patients (median age 67 years, range 33–84 years) with localized BALT lymphoma treated with radiotherapy. The median tumor size was 3.6 cm (range 0.8–8.2 cm) with a median SUV of 3.9 (range 1.3–11.4). Most patients (n = 8, 62%) received radiotherapy for BALT lymphoma after failing first-line treatment, including active surveillance (n = 3), surgical resection (n = 2), or systemic therapy (n = 3). Of 15 irradiated lesions, most (n = 10, 67%) received very low-dose radiotherapy (VLRDT) of 4 Gy (range 4–36 Gy). With a median follow-up of >50 months, only 3 patients experienced progression of disease, none within the irradiated field (1 following VLDRT and 2 following full-dose RT). Among all treated lesions, the overall response rate was 100% (n = 5, 33% partial response [PR] and n = 10, 67% complete response [CR]). Among 10 lesions treated with VLDRT, 6 (60%) achieved CR; among 5 lesions treated with full-dose RT, 4 (80%) achieved CR. There were no events of secondary lung cancers within or in proximity to the irradiated field. Conclusions: Radiotherapy, including VLDRT, is a feasible, well tolerated, non-invasive and effective treatment strategy for primary BALT lymphoma that can be considered in both the upfront and recurrent treatment settings. The research was funded by: National Cancer Institute (P30 CA008748) Keywords: Extranodal non-Hodgkin lymphoma, Indolent non-Hodgkin lymphoma No conflicts of interests pertinent to the abstract.

The Development of an Ontology Platform to Detect Serine Protease Involvement in COVID-19-Induced Co-Morbid BALT Lymphoma

Mucosal-associated lymphoid tissue (MALT) lymphoma is a rare, low-grade non-Hodgkin lymphoma that develops from B cells and often is diagnosed incidentally. Although it possesses diverse subvariants, such as bronchial-associated lymphoid tissue (BALT), its pathogenesis remains unclear. Currently, little consensus exists on a standard treatment approach partially due to their rare occurrences and heterogeneities, but also due to good prognosis. Studies over the past decades have identified an association between coronaviral infection and BALT formation, whereby mutations in serine proteases increase the risk of BALT lymphoma. The aim of this study was to use a computational approach to validate and further elucidate this relationship specifically between SARS-CoV-2 and BALT lymphoma development. To address this, ontologies for serine proteolytic homeostasis were constructed utilizing keywords and abstracts from PubMed, which were then used with parsing and logical "OR" functions to generate search inputs for the European Bioinformatics Institute (EBI) COVID-19 ontology and ClinicalTrials.gov (as in Table 1), specifically for and between "BALT", "lymphoma", "serine protease", and "heat shock protein" as a representative type of serine protease inhibitor (SerPIn) class. The Heat Shock Protein 70 family and its more specific subtype Binding immunoglobulin protein (BiP) (a.k.a. GRP-78, HSPA5, and Byun1), were also manually identified as an alternative results marker for efficacy comparison. Results showed that this approach successfully demonstrated a literary relationship between serine proteases (SERPINE1) with heat shock proteins (HS90) and COVID-19, with aryl hydrocarbon (Row 4 of Table 1) potentially acting as this bridge between BALT lymphomas, transmembrane bound serine proteases, and SARS-CoV-2. This demonstrates a viable approach to automate the development of future ontologies to detail the pathogenesis between SARS-CoV-2, BALT lymphoma, and serine proteases. However, it was limited in its ability to yield positive hit results for HSP70 or GRP-78, likely due to the narrow focus of the EBI COVID-19 ontology and PubMed for covering full text papers. Nonetheless, this platform still offers a wealth of genetic, proteomic, and clinical trials information that can effectively be deployed toward characterizing disease pathogenesis, even in rare hematological malignancies. Forward looking, future studies will continue to investigate the application of serine proteolytic homeostasis ontologies to BALT lymphoma to better characterize its pathogenesis and interplay with other malignancies, which can then be utilized to develop targeted therapeutic solutions for critical BALT and MALT malignancies. Figure 1View largeDownload PPTFigure 1View largeDownload PPT Close modal

NOT YOUR USUAL PULMONARY NODULE: CASE OF PULMONARY MALTOMA

NOT YOUR USUAL PULMONARY NODULE: CASE OF PULMONARY MALTOMA

PO-1170 Low-dose radiotherapy for extranodal marginal zone lymphoma of bronchus-associated lymphoid tissue

PO-1170 Low-dose radiotherapy for extranodal marginal zone lymphoma of bronchus-associated lymphoid tissue

Bronchus-associated lymphoid tissue (BALT) lymphoma and autoimmune disease.

Bronchus-associated lymphoid tissue (BALT) lymphoma and autoimmune disease.

Decision-making in diagnosis of bronchus-associated lymphoid tissue lymphoma

Bronchus-associated lymphoid tissue (BALT) lymphomas of the lung are uncommon, and diagnosis is often delayed due to the indolent clinical course. Often, adequate samples are difficult to obtain by bronchoscopy with transbronchial biopsy alone. This retrospective study reviewed the diagnosis and treatment of BALT lymphoma cases at our institution over the course of 19 years. Most patients were white, women, and >50 years old; the mean Charlson Comorbidity Index at the time of diagnosis was 6. Seven of 12 patients presented with solitary nodules or multiple nodules. For six cases, initial modalities were nondiagnostic; four subsequently underwent surgical biopsy, one underwent computed tomography–guided biopsy, and one underwent navigational bronchoscopy for final diagnosis of BALT lymphoma. Ultimately, 55% of cases were diagnosed with nonsurgical biopsy. One patient suffered a pneumothorax related to the initial diagnostic attempt. Ten patients received chemotherapy, radiation, and/or surgery, and 11 of the 12 are still alive. Our data confirm the previously described indolent behavior of BALT lymphomas and the challenges related to diagnosis. While previous studies have suggested surgical biopsy as the primary modality for obtaining histopathology, navigational bronchoscopy could serve as a safer alternative.

Active surveillance of primary extranodal marginal zone lymphoma of bronchus-associated lymphoid tissue

AbstractAlthough patients with bronchus-associated lymphoid tissue (BALT) lymphoma show an indolent clinical course, appropriate disease management at diagnosis is not well defined. This study aimed to compare 3 treatment strategies for patients with BALT lymphoma: active surveillance, systemic chemotherapy or immunotherapy at diagnosis, or complete surgical resection at diagnosis. We conducted a retrospective study of all patients with new diagnoses of marginal zone lymphoma (MZL) involving the lung who were treated at the Memorial Sloan Kettering Cancer Center between 1995 and 2017. Primary BALT lymphoma was defined as disease confined to the lungs and adjacent lymph nodes. Active surveillance was defined as a documented observation plan and ≥3 months of follow-up before initiating treatment. Overall survival (OS) and event-free survival (EFS) were compared between treatment groups. We reviewed 200 consecutive patients with MZL involving the lung; 123 met the inclusion criteria and were managed by active surveillance (47%), complete surgical resection (41%), or systemic chemotherapy or immunotherapy (11%). With a median follow-up of >60 months, surgical resection was associated with a superior EFS compared with active surveillance and systemic treatment (6-year EFS: 74% vs 65% vs 62%, respectively; P = .013). Larger lesions and thrombocytopenia were associated with shorter EFS. All groups had excellent OS at 6 years (93%), albeit with a slight superiority for surgical resection (100%) over active surveillance (91%) and systemic treatment (76%) (P = .024). BALT lymphoma is an indolent disease that can often be managed expectantly and not require therapy for many years.

Clinical and biological features of B-cell neoplasms with CDK6 translocations: an association with a subgroup of splenic marginal zone lymphomas displaying frequent CD5 expression, prolymphocytic cells, and TP53 abnormalities.

A translocation involving the cyclin-dependent kinase 6 (CDK6) gene [t(CDK6)] is a rare but recurrent abnormality in B-cell neoplasms. To further characterise this aberration, we studied 57 cases; the largest series reported to date. Fluorescence in situ hybridisation analysis confirmed the involvement of CDK6 in all cases, including t(2;7)(p11;q21) immunoglobulin kappa locus (IGK)/CDK6 (n=51), t(7;14)(q21;q32) CDK6/immunoglobulin heavy locus (IGH) (n=2) and the previously undescribed t(7;14)(q21;q11) CDK6/T-cell receptor alpha locus (TRA)/T-cell receptor delta locus (TRD) (n=4). In total, 10 patients were diagnosed with chronic lymphocytic leukaemia, monoclonal B-cell lymphocytosis or small lymphocytic lymphoma, and 47 had small B-cell lymphoma (SmBL) including 36 cases of marginal zone lymphoma (MZL; 34 splenic MZLs, one nodal MZL and one bronchus-associated lymphoid tissue lymphoma). In all, 18 of the 26 cytologically reviewed cases of MZL (69%) had an atypical aspect with prolymphocytic cells. Among the 47 patients with MZL/SmBL, CD5 expression was found in 26 (55%) and the tumour protein p53 (TP53) deletion in 22 (47%). The TP53 gene was mutated in 10/30 (33%); the 7q deletion was detected in only one case, and no Notch receptor 2 (NOTCH2) mutations were found. Immunoglobulin heavy-chain variable-region (IGHV) locus sequencing revealed that none harboured an IGHV1-02*04 gene. Overall survival was 82% at 10years and not influenced by TP53 aberration. Our present findings suggest that most t(CDK6)+ neoplasms correspond to a particular subgroup of indolent marginal zone B-cell lymphomas with distinctive features.

FLIGHT RISK: BRONCHUS-ASSOCIATED LYMPHOID TISSUE IN A HEALTHY AVIATOR

SESSION TITLE: Fellows Lung Cancer Posters SESSION TYPE: Fellow Case Report Posters PRESENTED ON: October 18-21, 2020 INTRODUCTION: Bronchus-associated lymphoid tissue (BALT) lymphoma is a rare marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT). The incidence of MALT worldwide is estimated to be up to 1% of primary pulmonary lymphomas, most commonly endobronchial.(1) Infectious pathogens, amyloid deposition, rheumatological diseases and smoking have all been associated with pulmonary MALT to varying degrees.(2) Treatment is patient-specific, and ranges from observation to chemotherapy, radiation therapy or resection, usually with a favorable prognosis.(3) CASE PRESENTATION: We present a case of an asymptomatic United States Marine Corps F/A-18 fighter jet aviator who was incidentally found to have BALT, first identified as a 5mm lesion during a CT scan for an unrelated pericardial cyst four years earlier. The most recent imaging demonstrated the lesion to be similar in size to previous annual studies. To avoid further unnecessary exposure to radiation for a stable endobronchial lesion, we offered an inspection flexible bronchoscopy which confirmed the presence of a raised flesh-colored, polypoid lesion located in the medial right bronchus intermedius. PET/CT was non-avid. The lesion was easily biopsied and sent for pathological examination. After the diagnosis, the patient successfully underwent involved-site radiation therapy. He went on to receive a waiver from his flight surgeon to return to flight duty without limitations. DISCUSSION: Our case of BALT was diagnosed after it was noted incidentally on CT. Its radiographic appearance was consistent with other cases in the literature.(4, 5) This case underscores the importance of investigating such stable-appearing, non-resolving endobronchial lesions. In this instance, an efficient time course from diagnosis to treatment allowed this aviator to be cleared to fly again. CONCLUSIONS: Bronchus-associated lesions have a wide differential diagnosis.(5) This case highlighted how pursuit of such lesions may have clinical benefit, such as this case, which positively impacted this patient’s military flying career.(4) Reference #1: Takeshi K, Daisuke H, et. al. Endobronchial Mucosa-associated Lymphoid Tissue Lymphoma: A Report of Two Cases and a Review of the Literature. Intern Med. 2018; 57: 2233-2236. Reference #2: William J, Variakojis D, et. al. Lymphoproliferative Disorders. Archives of Pathology & Laboratory Medicine. 2013; 137: 382–391. Reference #3: Hiraishi Y, Iikura M, et. al. Two tracheal BALT lymphoma patients successfully treated with chemotherapy including rituximab. Respiratory Investigations. 2014; 52: 144- 146. DISCLOSURES: No relevant relationships by Neil Partain, source=Web Response

Expectant Management of Extranodal Marginal Zone Lymphoma of Bronchial-Associated Lymphoid Tissue (BALT)

Expectant Management of Extranodal Marginal Zone Lymphoma of Bronchial-Associated Lymphoid Tissue (BALT)

BRONCHIAL-ASSOCIATED LYMPHOID TISSUE LYMPHOMA (BALTOMA) PRESENTING AS A NONRESOLVING PNEUMONIA

SESSION TITLE: Lung Pathology 2 SESSION TYPE: Fellow Case Report Posters PRESENTED ON: 10/09/2018 01:15 PM - 02:15 PM INTRODUCTION: Pulmonary mucosa-associated lymphoid tissue lymphoma (also known as bronchial associated lymphoid tissue lymphoma or BALToma) is a rare type of extranodal marginal zone B-cell lymphoma. Marginal zone lymphomas in other parts of the body have been associated with chronic antigen stimulation, such as in autoimmune disease. Pathogenesis from chronic infection has been hypothesized but a causative organism or antigen has not been identified for the lungs. We present a soldier with initial infectious symptoms who had persistent dense pulmonary consolidation that was found to be a BALToma. CASE PRESENTATION: 48 year old male underwent chest x-ray for cough and dyspnea on exertion that developed shortly after returning from deployment to Afghanistan. He was found to have a right middle lobe consolidation on chest radiograph that was redemonstrated along with multiple bilateral ground glass opacities on confirmatory chest CT. His symptoms resolved after treatment with oral azithromycin and follow-up chest radiograph 3 weeks later showed improvement in the right middle lobe consolidation. Chest imaging two years later while asymptomatic revealed persistent, slightly enlarged right middle lobe consolidation and persistent bilateral ground glass lesions. PET-CT of the chest, abdomen, and pelvis showed hypermetabolism (SUV-m of 3.7) limited to the pulmonary mass without any other hypermetabolic foci. There was a single pathologic-sized right paratracheal lymph node measuring up to 1.5 cm that showed normal FGD activity. Bronchoscopy with bronchial brushing, fine needle aspiration, and transbronchial forceps biopsy of the mass revealed a dense infiltrate composed of small B-cells that tested positive for the MALT1 translocation (t(11;18)), consistent with a BALToma. No infectious organism was isolated. DISCUSSION: Bronchial associated lymphoid tissue lymphoma is a slow growing malignant pulmonary tumor. Clinical manifestations are usually nonspecific and the majority of patients are asymptomatic at diagnosis. Biopsy sample of a BALToma are characterized by presence of a nodular lymphoid infiltrate expanding the marginal zone of reactive lymphoid follicles with tumor cells infiltrating the bronchiolar or the alveolar epithelium. The diagnosis is confirmed via immunohistochemical analysis that excludes lung localization of other lymphomas such as mantle cell and follicular lymphoma. PCR or FISH probes for the MALT1 gene can be supportive. Diagnosis may be missed due to the radiographic similarity to community acquired pneumonia, but appropriate diagnosis is important since this process typically responds well to therapy. CONCLUSIONS: BALToma should be considered in the differential of asymptomatic patients with imagining suggest of persistent nonresolving pneumonia. Reference #1: Borie R, Wislez M, Antoine M, et al. Pulmonary mucosa-associated lymphoid tissue lymphoma revisited. Eur Respir J. 2016 Apr;47(4):1244-60 DISCLOSURES: No relevant relationships by Benjamin Rosen, source=Web Response No relevant relationships by Krystle Salazar, source=Web Response No relevant relationships by John Sherner, source=Web Response

A Case of Bronchus-Associated Lymphoid Tissue Lymphoma Diagnosed by Navigational Bronchoscopy

SESSION TITLE: Bronchoscopy and Associated Procedures SESSION TYPE: Affiliate Case Report Poster PRESENTED ON: Tuesday, October 31, 2017 at 01:30 PM - 02:30 PM INTRODUCTION: Bronchus-associated lymphoid tissue (BALT) lymphomas of the lung are uncommon and diagnosis is often delayed due to indolent clinical course, varied radiographic presentations, inability to obtain adequate sampling by bronchoscopy and eventual need for surgical biopsy. To date, there have been no cases of BALT lymphoma diagnosed by navigational bronchoscopy. We describe the first case, to our knowledge, of BALT lymphoma diagnosed by navigational bronchoscopy. CASE PRESENTATION: A 77 year-old female with a 25 pack-year history of smoking was referred to our Pulmonary clinic after incidental finding of a nodule on computed tomography (CT) of the chest performed at an outside hospital for chest pain. The CT Chest demonstrated a 1.6 cm ground-glass nodule in the left upper lobe. There were no prior CTs for comparison. Due to concern for malignancy, we proceeded with flexible bronchoscopy with transbronchial biopsy but pathology did not yield malignant cells. Given continued concern for malignancy, various options to obtain tissue diagnosis were considered. She was felt to be a poor candidate for CT-guided core biopsy which would likely result in pneumothorax due to diffuse emphysematous changes on CT. She was also not an optimal candidate for surgical biopsy due to severely reduced forced expiratory volume in one second (FEV1). Therefore, attempt at sampling was made by navigational bronchoscopy. Biopsy results yielded histology consistent with marginal zone lymphoma of bronchial associated lymphoid tissue. Since bronchoscopy, the patient has been managed conservatively with serial CT scans due to lack of constitutional symptoms that would necessitate treatment for lymphoma. DISCUSSION: Marginal zone lymphomas of the lung, also known as BALT lymphomas, arise from BALT which are not native to the bronchial tree. BALT is hypothesized to form in response to various antigenic stimuli, such as smoking, inflammatory disorders or autoimmune diseases. BALT lymphomas account for only .5% of all pulmonary malignancies1; most require no treatment due to indolent course. BALToma can be diagnosed by bronchoscopy; however, surgical lung biopsy is often required for optimal histologic yield. The most appropriate biopsy technique can be a challenging clinical risk-benefit decision and peripheral location of lesions in addition to patient co-morbidities including emphysematous changes around the lesion, respiratory function, and the likelihood of malignancy must be taken into account. Since BALT lymphomas frequently have indolent courses, the risks associated with a more aggressive diagnostic modality such as surgical biopsy may not be justifiable. CONCLUSIONS: In using navigational bronchoscopy, we were able to avoid the risks associated with both surgical biopsy and CT-guided biopsy to obtain a diagnosis of BALT lymphoma of the lung. Reference #1: Stefanovic, A et al. Pulmonary marginal zone lymphoma. Leukemia and Lymphoma. 2008 Jul;49(7):1311-20. DISCLOSURE: The following authors have nothing to disclose: Tasnim Lat, Parsa Hodjat, Juan Sanchez, Carl Boethel No Product/Research Disclosure Information

A single center experience: rituximab plus cladribine is an effective and safe first-line therapy for unresectable bronchial-associated lymphoid tissue lymphoma.

Bronchial-associated lymphoid tissue (BALT) lymphoma is a relatively rare form of B-cell non-Hodgkin lymphoma (B-NHL). To date, the standard systemic treatment for this disease is still under debate, and few data are accessible for newly diagnosed unresectable BALT lymphoma presented with advanced disease. The combination of rituximab (R) and cladribine (2-CdA) has shown some activity in indolent B-NHL, but its usage has not been tested in disseminated BALT lymphoma so far. An observational retrospective study was performed on homogeneous data of 8 patients with biopsy-proven stage IV BALT lymphoma to assess the efficacy and the safety of R-2-CdA therapy. All but one of the patients received six courses of R-2-CdA regimen consisted of rituximab 375 mg/m2 IV day 1 and cladribine 0.1 mg/kg IV days 1-4 every 21 days; one patient completed 4 cycles and received additional R maintenance. A high overall response rate (ORR) was observed (100%), with 2 patients (25%) achieved a complete remission (CR), the remaining (75%) a partial response. Improvement of pulmonary function was observed in all tested patients. Grade 3 and 4 toxicities were leukocytopenia and neutropenia in 3 patients (37.5%), diarrhea in one (12.5%). Estimated two-year progression-free survival (PFS) and 2-yr overall survival (OS) were 80.0% (95% CI, 20.3-96.7%) and 100%, respectively. R-2-CdA therapy demonstrated high activity and tolerable toxicity in chemotherapy-naïve patients with unresectable BALT lymphoma of advanced stage. Although further large-scale study is needed for consolidation, R-2-CdA regimen could be a good first-line therapy option for patients with unresectable BALT lymphoma.

びまん性汎細気管支炎に合併し, 自然消退した気管支原発悪性リンパ腫の 1 例

びまん性汎細気管支炎に合併し, 自然消退した気管支原発悪性リンパ腫の 1 例

Bronchus-Associated Lymphoid Tissue Lymphoma Associated with Gain of MALT1

Bronchus-Associated Lymphoid Tissue Lymphoma Associated with Gain of MALT1

Case Report: Bronchial associated lymphoid tissue lymphoma and Mycobacterium chelonae

Bronchial-associated lymphoid tissue (BALT) lymphoma is a rare condition that accounts for only 0.5-1% of all malignant lung tumours. We present the case of a 66-year-old man admitted with pneumonia for further study and therapy. Initially the sputum was positive for Mycobacterium tuberculosis complex using polymerase chain reaction technology and antituberculous therapy was initiated. Due to the lack of imagiological improvement, the patient underwent a pulmonary transthoracic biopsy that revealed BALT lymphoma. Months later, Mycobacterium chelonae was identified and specific therapy was started with clarithromycin and tobramycin, before initiating BALT treatment with cyclophosphamide. There are only a few documented cases of BALT lymphoma associated with Mycobacterium. In this case M. chelonae might have been present before BALT lymphoma, contributing as an immunologic stimulus, or appeared afterwards, in the neoplastic context. BALT has an indolent evolution with a good prognosis and that is the reason why some experts favour a “watchful waiting” option.