Search

We report a case of primary pulmonary anaplastic lymphoma kinase-positive anaplastic large cell lymphoma in a 38-year-old woman with a smoking and vaping history. The patient presented with hypoxemia and a history of shortness of breath, cough, and intermittent fevers. Initial imaging and pleural fluid studies suggested possible empyema. Despite being given antibiotics, her respiratory status continued to deteriorate and she was put on extracorporeal membrane oxygenation. Repeat imaging showed increased size of intrathoracic lymph nodes and perilymphatic pulmonary nodules. IV steroids were initiated after bronchoalveolar lavage revealed lipophages suggestive of e-cigarette, or vaping, product use-associated lung injury. A laboratory workup revealed no signs of rheumatologic disease, and negative cultures ruled out a bacterial or fungal cause of the disease. Because of these laboratory results and because the patient did not show clinical signs of improvement, a biopsy of the left lower lobe lymph node was performed. The patient was diagnosed with anaplastic lymphoma kinase-positive anaplastic large cell lymphoma based on the results of the biopsy. This case highlights the importance of suspecting pulmonary lymphoma in patients with a history of B-symptoms and compatible imaging findings, despite its rarity.

Primary pulmonary lymphoma is a rare neoplasm characterized by the proliferation of lymphoid tissue affecting the lungs. The most common subtype is marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT). Rarely, a MALT lymphoma transforms into a diffuse large B-cell lymphoma (DLBCL). Treatment options include chemotherapy, radiotherapy, immunotherapy, and surgery. Here, we describe a patient with a primary pulmonary MALT lymphoma transforming into DLBCL. The purpose of this case report is to raise awareness of the relevant clinical and imaging features and to emphasize the need for a multidisciplinary approach to optimal management. In addition, we screened the PubMed and Embase databases for similar reports with a confirmed presence of transforming lymphoma within the lungs. [ABSTRACT FROM AUTHOR]

Primary pulmonary lymphoma is a rare neoplasm characterized by the proliferation of lymphoid tissue affecting the lungs. The most common subtype is marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT). Rarely, a MALT lymphoma transforms into a diffuse large B-cell lymphoma (DLBCL). Treatment options include chemotherapy, radiotherapy, immunotherapy, and surgery. Here, we describe a patient with a primary pulmonary MALT lymphoma transforming into DLBCL. The purpose of this case report is to raise awareness of the relevant clinical and imaging features and to emphasize the need for a multidisciplinary approach to optimal management. In addition, we screened the PubMed and Embase databases for similar reports with a confirmed presence of transforming lymphoma within the lungs.

Key message Ultrasound‐guided transthoracic needle biopsy is a relatively safe procedure diagnosing subpleural pulmonary mass and has high sensitivity in the diagnosis of lung cancer. However, the usefulness in other rare malignancies is unknown. This case shows the effectiveness in diagnosing not only lung cancer but also rare malignancies including primary pulmonary lymphoma.

Primary pulmonary diffuse large B‐cell lymphoma is a rare entity. We describe a case of pulmonary lymphoma with multiple nodules mimicking metastases in a treated patient with rheumatoid arthritis. A 73‐year‐old man was diagnosed with rheumatoid arthritis at the age of 30. He was treated with leflunomide. He was followed up for a nontuberculous mycobacterial infection. He underwent percutaneous coronary intervention for acute myocardial infarction at the age of 70. In April 2022, routine follow‐up revealed new‐onset multiple nodules on chest computed tomography (CT). A position emission tomography/CT scan with 18F‐fluorodeoxyglucose showed a low‐high maximum standardized uptake value by multiple nodules. Pathologic examination of a video‐assisted thoracic surgical biopsy revealed pulmonary diffuse large B‐cell lymphoma. Systemic chemotherapy with rituximab, cyclophosphamide, vincristine, and prednisolone reduced and eliminated multiple nodules. Pulmonary lymphoma should be considered as a differential diagnosis in the case of multiple nodules on a chest CT. [ABSTRACT FROM AUTHOR]

Abstract Primary pulmonary diffuse large B‐cell lymphoma is a rare entity. We describe a case of pulmonary lymphoma with multiple nodules mimicking metastases in a treated patient with rheumatoid arthritis. A 73‐year‐old man was diagnosed with rheumatoid arthritis at the age of 30. He was treated with leflunomide. He was followed up for a nontuberculous mycobacterial infection. He underwent percutaneous coronary intervention for acute myocardial infarction at the age of 70. In April 2022, routine follow‐up revealed new‐onset multiple nodules on chest computed tomography (CT). A position emission tomography/CT scan with 18F‐fluorodeoxyglucose showed a low‐high maximum standardized uptake value by multiple nodules. Pathologic examination of a video‐assisted thoracic surgical biopsy revealed pulmonary diffuse large B‐cell lymphoma. Systemic chemotherapy with rituximab, cyclophosphamide, vincristine, and prednisolone reduced and eliminated multiple nodules. Pulmonary lymphoma should be considered as a differential diagnosis in the case of multiple nodules on a chest CT.

Key message: Ultrasound‐guided transthoracic needle biopsy is a relatively safe procedure diagnosing subpleural pulmonary mass and has high sensitivity in the diagnosis of lung cancer. However, the usefulness in other rare malignancies is unknown. This case shows the effectiveness in diagnosing not only lung cancer but also rare malignancies including primary pulmonary lymphoma. [ABSTRACT FROM AUTHOR]

Background: The risk of gastrointestinal stromal tumor (GIST) in combination with other primary malignancies is high, which occurs before and after the diagnosis of GIST. Primary pulmonary T-cell lymphoma is a rare type of non-Hodgkin lymphoma. Case presentation: We report a 53-year-old male patient who was admitted to our hospital with fever, cough, and expectoration for 2 weeks. Chest computed tomography (CT) showed a cavitary mass in the left lower lobe with multiple nodules in the upper lobes of both lungs. The patient had a history of surgery for small intestinal stromal tumors and was treated with oral imatinib after surgery. Lung biopsy was diagnosed as lymphomatoid granulomatosis, tending to grade 3. The pathological diagnosis was corrected by surgery and genetic testing for lung non-Hodgkin CD8-positive cytotoxic T-cell lymphoma with Epstein-Barr virus (EBV) infection in some cells. After multiple chemotherapies, the CT scan showed a better improvement than before. The patient is still under follow-up, and no tumor recurrence has been found. Conclusion: Patients with a history of GIST should be monitored for other malignancies. The clinical symptoms and imaging examinations of primary pulmonary T-cell lymphoma are not characteristic, and the definite diagnosis still depends on pathological examination. The patient was treated with the CHOP chemotherapy regimen after the operation, the curative effect was good. [ABSTRACT FROM AUTHOR]

Background: The occurrence of primary pulmonary lymphoma (PPL) as a secondary malignancy in patients diagnosed with chronic myeloid leukemia (CML) is extremely rare. As the clinical manifestations are atypical, most patients with PPL tend to be misdiagnosed with pneumonia. When the radiographic features of PPL and pulmonary infection overlap, clinicians can be confused about the diagnosis. Here, we report the first case of coexistence of PPL and opportunistic infections in a patient with CML in chronic phase (CML-CP). Case presentation: A 55-year-old woman presented with three weeks of hemorrhage of the oral mucosa at the Department of Hematology. After undergoing various examinations, she was diagnosed with CML-CP and was started on imatinib (400 mg/daily). Due to sudden respiratory distress, the patient was admitted to the respiratory intensive care unit 11 months later. Chest computed tomography (CT) revealed ground-glass opacities, patchy shadows, and multiple nodules in both lungs and enlarged mediastinal lymph nodes. The combination of biapenem and voriconazole antibiotic treatments was effective. The patient's respiratory distress was relieved, but therewas intermittent coughing. In the following time, the patient developed a fever, and the imaging findings indicated progression of the disease in both lungs. Bronchoalveolar lavage (BAL) identified pathogens of multiple opportunistic infections. The coexistence of lymphomatoid granulomatosis (LYG) was not confirmed in this patient until a second CT-guided biopsy was performed. Ultimately, the patient underwent chemotherapy in time and is currently alive today. Conclusions: When the patient's recurrent respiratory symptoms and imaging findings do not coincide, secondary tumors should be considered in addition to infection as a diagnosis. In these cases, multiple pathological tissue biopsies should be performed. [ABSTRACT FROM AUTHOR]

Primary pulmonary follicular lymphoma is an extremely rare subset of extra-nodal non-Hodgkin's lymphoma. We present a successful work-up and surgical management of this disease entity. The patient is a 74-year-old man who presented with an enlarging ground glass opacity on his computed tomography scan. Percutaneous biopsy was not diagnostic for malignancy, but given the underlying malignant potential, he underwent definitive operative resection rather than additional invasive diagnostic testing. Our case highlights challenges in the management of nondiagnostic preliminary pathology as well as the role of a multidisciplinary approach to treatment of a rare lung pathology., Competing Interests: Informed consent was obtained from the patient. No conflicts of interest, financial ties or funding/support to disclose., (Published by Oxford University Press and JSCR Publishing Ltd. © The Author(s) 2024.)

Primary pulmonary lymphoma (PPL) is an uncommon condition involving the lungs, characterized by the abnormal clonal growth of lymphoid cells. Patients with PPL can be asymptomatic or present with vague clinical signs such as cough, fever, chest discomfort, and shortness of breath. Some may also have underlying immunosuppression or autoimmune conditions. Tuberculosis being more common with a higher incidence in endemic areas, PPL can often be misdiagnosed as tuberculosis. Also, they share common symptoms like cough, fever, fatigue, unexplained weight loss, and an upper lobe predilection. Therefore, diagnosing PPL from other common pulmonary diseases is of utmost importance in early diagnosis and treatment. Generally, small biopsy samples are essential for precise diagnosis and prompt treatment. Therapeutic options include chemotherapy, radiotherapy, immunotherapy, and surgical intervention. Herewith we outline a case initially presented as acanthosis nigricans, referred with respiratory symptoms, pleural effusion, and mediastinal lymphadenopathy, which was primarily thought to be a tuberculous pleural effusion. Apart from clinicoradiological and laboratory analysis, thoracoscopy-guided biopsy and histopathological examination pointed toward further steps in diagnosis. The patient was initiated on chemotherapy with an R-CHOP regimen. This case depicts the call for a multidisciplinary perspective for the definite and expeditious diagnosis and management of a paraneoplastic syndrome associated with diffuse large B cell lymphoma. This discussion also highlights the need for a thorough evaluation of paraneoplastic syndromes, as they are crucial in the early diagnosis of the disease and in identifying relapses., Competing Interests: Human subjects: Consent was obtained or waived by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work., (Copyright © 2024, Jose et al.)

Background: To date, there is no treatment consensus on mucosa-associated lymphoid tissue (MALT) derived primary pulmonary lymphoma (PPL). Methods: We identified patients with early-stage MALT-type PPL from the National Cancer Institute's Surveillance, Epidemiology, and End Results program database. The patients were divided into four groups according to treatment modalities: None of surgery or chemotherapy (None) group, Surgery alone group, Chemotherapy alone (Chemo alone) group, and Surgery plus chemotherapy (Surgery + chemo) group. Overall survival (OS) and cancerspecific survival (CSS) were study endpoints. We performed Cox regression analyses, propensity score-matched analyses (PSM) and Kaplan-Meier (KM) survival curves to compare the survival among different groups. Results: A total of 953 patients were included in our analysis with 302, 403, 175, and 73 cases in the None, Surgery alone, Chemo alone, and Surgery + chemo groups, respectively. In this cohort, the estimated 3-year, 5-year and 10-year OS rates were 86.95%, 78.91%, and 55.89%, respectively. Meanwhile, the estimated 3-year, 5-year and 10-year CSS rates were 96.71%, 93.73%, and 86.84%, respectively. Multivariate Cox regression analyses demonstrated that increasing age, tumors located in the lower lobe, and stage II were significant predictors of poorer OS while increasing age and tumors located in the bilateral lungs were associated with lower CSS. After PSM analyses, the KM survival curves showed no significant differences in OS or CSS among the four groups. Conclusion: Early-stage MALT-type PPL is indolent in nature. Neither surgery, chemotherapy nor a combination of surgery and chemotherapy can improve OS and CSS, suggesting that "watch and wait" may be a reasonable alternative. [ABSTRACT FROM AUTHOR]

Objective: This study aimed at to raise the awareness understanding of primary pulmonary lymphoma (PPL) by analyzing the clinical manifestation, imaging, pathology, diagnosis, treatment, and prognostic features of 50 cases of PPL. Methods: The study of 50 individuals with PPL diagnosed at the First affiliated hospital of Nanchang university between January 2009 and December 2019 was performed. Results: Overall, 27 males and 23 females were enrolled, with an average age of 57.6 ± 15.6 years. The primary symptoms included, cough (n = 37), expectoration (n = 25), sputum with blood (n = 12), and chest pain (n = 12). Two individuals had Hodgkin's lymphoma and 48 patients had non-Hodgkin's lymphoma (NHL). We divided the NHL cases into mucosa-associated lymphoid tissue lymphoma (MALT) (n = 21), diffuse large B-cell lymphoma (n = 12), small lymphocytic lymphoma (n = 2), mantle B-cell lymphoma (n = 2), follicular lymphoma (n = 1), B-cell lymphoma without further classification (n = 8), and T-cell lymphoma (n = 2). The imaging findings revealed that unilateral lung involvement was more common among the patients. The longest follow-up duration up to December 2019 was 123 months with 40 surviving patients. The 5-year overall survival and progression-free survival were 46.7% and 44.4%, respectively. Age was an independent predictive factor for the 5-year survival (hazard ratio, 8.900; P =.038), (P <.05). Conclusion: PPL is a uncommon disease with atypical clinical manifestations and is often misdiagnosed. Immunohistochemistry is currently the standard used in pathologic evaluation of PPL. MALT prognosis is better in contrast with other kinds of PPL. Surgery or radiotherapy can be considered in patients with limited lesions, and chemotherapy is the first treatment option for diffuse lesions. Age of ≥ 60 years was reported as an independent adverse predictive factor. [ABSTRACT FROM AUTHOR]

Background: Primary pulmonary lymphoma (PPL) is a rare clonal lymphoproliferative lung disease. The present study analyzes the clinical features, imaging data, pathologic characteristics, treatment, and prognosis of PPL patients, with the aim to discuss the appropriate diagnosis and therapy of PPL patients in thoracic surgery. Methods: We performed a retrospective analysis on 36 patients with PPL confirmed by postoperative pathology between 2006 and 2020. We divided the patients into low-stage (IE) and high-stage (IIE) groups using modified Ann Arbor staging. The clinical manifestations, imaging findings, treatment modalities, and outcomes were evaluated. Results: The female to male ratio was 1.57:1 and the median age was 55 (31–69) years old. The majority of the patients had stage IE disease (75%; 27 of 36) and 9 patients had stage IIE disease. Patients with advancing stage were more likely to have respiratory symptoms. The imaging findings presented solid nodule or mass, pneumonia-like consolidative pattern, ground-glass opacity, and mixed pattern. There were 31 cases of mucosa-associated lymphoid tissue lymphoma (MALT), 2 diffuse large B-cell lymphoma (DLBCL), 2 nodular sclerosing Hodgkin's lymphoma, and 1 marginal zone B-cell lymphoma. Two patients were diagnosed with PPL and non-small cell lung cancer (NSCLC) synchronously (one AIS and MIS and one lung adenocarcinoma). All the patients received surgery. Nine patients received adjuvant therapy after surgery (five radiotherapy, two chemotherapy, and two chemoradiotherapy). Thirty-four patients had a median follow-up time of 31 months (follow-up range: 7–152 months). Of the 34 patients, 1 patient died of liver metastases and 1 patient died of intestinal metastases. Conclusions: Our retrospective analysis suggested that most PPLs were indolent and had favorable prognosis, but the discrimination of PPL with other lung diseases was difficult. Preoperative biopsy and intraoperative frozen section examination might help in the surgical choice. Limited lung resection was enough for peripherally localized PPL. [ABSTRACT FROM AUTHOR]

BackgroundPrimary pulmonary lymphoma (PPL) is a rare clonal lymphoproliferative lung disease. The present study analyzes the clinical features, imaging data, pathologic characteristics, treatment, and prognosis of PPL patients, with the aim to discuss the appropriate diagnosis and therapy of PPL patients in thoracic surgery.MethodsWe performed a retrospective analysis on 36 patients with PPL confirmed by postoperative pathology between 2006 and 2020. We divided the patients into low-stage (IE) and high-stage (IIE) groups using modified Ann Arbor staging. The clinical manifestations, imaging findings, treatment modalities, and outcomes were evaluated.ResultsThe female to male ratio was 1.57:1 and the median age was 55 (31–69) years old. The majority of the patients had stage IE disease (75%; 27 of 36) and 9 patients had stage IIE disease. Patients with advancing stage were more likely to have respiratory symptoms. The imaging findings presented solid nodule or mass, pneumonia-like consolidative pattern, ground-glass opacity, and mixed pattern. There were 31 cases of mucosa-associated lymphoid tissue lymphoma (MALT), 2 diffuse large B-cell lymphoma (DLBCL), 2 nodular sclerosing Hodgkin’s lymphoma, and 1 marginal zone B-cell lymphoma. Two patients were diagnosed with PPL and non-small cell lung cancer (NSCLC) synchronously (one AIS and MIS and one lung adenocarcinoma). All the patients received surgery. Nine patients received adjuvant therapy after surgery (five radiotherapy, two chemotherapy, and two chemoradiotherapy). Thirty-four patients had a median follow-up time of 31 months (follow-up range: 7–152 months). Of the 34 patients, 1 patient died of liver metastases and 1 patient died of intestinal metastases.ConclusionsOur retrospective analysis suggested that most PPLs were indolent and had favorable prognosis, but the discrimination of PPL with other lung diseases was difficult. Preoperative biopsy and intraoperative frozen section examination might help in the surgical choice. Limited lung resection was enough for peripherally localized PPL.

It is extremely unusual for primary pulmonary lymphoma, an uncommon occurrence in any form, to be of T-cell origin and to manifest as an endobronchial lesion. Each of these characteristics is rare individually, so cases that combine them are exceptional. We report a patient in whom primary pulmonary anaplastic large cell lymphoma, a T-cell neoplasm, presented with obstruction of the left upper lobe bronchus, resulting in left upper lobe atelectasis and creating the radiographic luftsichel sign. We briefly discuss anaplastic large cell lymphoma as a whole, place our case in the context of previously published literature on endobronchial anaplastic large cell lymphoma, and review the genesis of the luftsichel sign. [ABSTRACT FROM AUTHOR]

Abstract Background Primary pulmonary lymphoma (PPL) is a rare disease, especially in children. We analyse the clinical features of PPL in 4 children to strengthen a understanding of it. Results We reported a case series of 4 pediatric patients with PPLs including three diffuse large B-cell lymphomas and one natural killer-T cell lymphoma. All patients presented with unknown fever and cough as well as weight loss and fatigue. The white blood cell count was reduced in three patients and increased in the other one. The level of C-reactive protein was increased in all patients. The procalcitonin concentrations and bone marrow specimens were normal. Multiple or single pulmonary nodules with halo signs were found in all patients and air bronchograms found in 3 of them on chest computed tomography scan. Primary immunodeficiency was diagnosed in two patients who was performed genetic analysis. Conclusions When a patient presents with long-term fever, high C-reactive protein level, leukopenia/leukocytosis, and multiple or single pulmonary nodules with a “halo sign” and air bronchogram on computed tomography, a possibility of PPL should be considered. A co-existance of immunodeficiency needs to be further investigated in patients with PPL.

It is extremely unusual for primary pulmonary lymphoma, an uncommon occurrence in any form, to be of T-cell origin and to manifest as an endobronchial lesion. Each of these characteristics is rare individually, so cases that combine them are exceptional. We report a patient in whom primary pulmonary anaplastic large cell lymphoma, a T-cell neoplasm, presented with obstruction of the left upper lobe bronchus, resulting in left upper lobe atelectasis and creating the radiographic luftsichel sign. We briefly discuss anaplastic large cell lymphoma as a whole, place our case in the context of previously published literature on endobronchial anaplastic large cell lymphoma, and review the genesis of the luftsichel sign.

Primary pulmonary lymphoma (PPL) is a rare neoplasm. We report a case of 45-year-old men who was admitted to hospital for multiple nodules and masses in both lungs. The contrast enhanced chest CT scans revealed multiple nodules and masses of varying sizes in right upper and middle lobes and both lower lobes. CT-guided percutaneous transthoracic needle biopsy was performed. The diagnosis of PPL was confirmed by histopathological examination and Immunohistochemical staining. PPL should be included in the differential diagnoses in symptomless patients with multiple pulmonary nodules and masses.

Primary pulmonary lymphoma (PPL) is a rare clonal proliferation of lymphoid tissue involving one or both lungs. It is of two types, B-cell and T-cell lymphomas among which T-cell lymphoma is a rare entity and it is sparsely considered as a differential diagnosis in neoplastic lesions of the lung. Here, we are reporting a case of primary pulmonary T-cell lymphoma. PPL is a rare disease and can present with nonspecific symptoms. Radiologically, it can easily be confused with more common malignancies such as bronchogenic carcinoma with or without metastases. PPL carries different therapeutic and prognostic implications. Therefore, physicians should make every effort to achieve histopathological diagnosis before prognosticating a patient presenting with lung cancer. [ABSTRACT FROM AUTHOR]

Abstract Primary pulmonary lymphoma is an uncommon disease, and extranodal marginal zone lymphoma of mucosa‐associated lymphoid tissue (MALT) is the most common type of pulmonary lymphoma. The most frequent pattern observed in chest computed tomography (CT) is consolidation, followed by nodules and mass. The differentiation of pulmonary MALT lymphoma from other lung diseases is critical for disease management and treatment. However, pulmonary MALT lymphoma with isolated endobronchial manifestation has seldomly been reported. Here, we report a case of an elderly woman who presented with a four‐month history of cough, dyspnoea, and haemoptysis. Chest CT scan revealed left main bronchus narrowing without lung parenchymal lesion. Bronchoscopic examination was performed, and the diagnosis of primary pulmonary MALT lymphoma with isolated endobronchial involvement was made. She has been successfully treated with rituximab.

Abstract Background The primary pulmonary lymphoma (PPL), with a low incidence, was highly misdiagnosed in clinic. The present study analyzes the clinical features, laboratory and imaging data, pathologic characteristics, and summarizes misdiagnosis reasons of PPL cases, aims to provide a better understanding and increase the accuracy of early diagnosis and minimize the misdiagnosis of PPL. Methods The clinical data of 19 cases were collected from the first affiliated hospital of Wenzhou medical university (PRC) from April 2010 to May 2016. All cases were confirmed by pathology. The process of misdiagnosis was described. This study retrospectively analyzed the incidence, clinical presentation, laboratory examination, Chest CT scan and diagnosis of the cases. Results The symptoms of the 19 cases were dyspnea, fever, hemoptysis, chest pain or physical findings without obvious symptoms. Five patients were pneumonia-like, nine patients had lung single nodule or mass and four patients got pleural effusion, which were reported by computed tomography (HRCT) scan. There were 2 cases of Hodgkin lymphoma (HL), and 17 cases of non-Hodgkin lymphoma (NHL). In NHL cases, 12 cases were confirmed mucosa associated lymphoid tissue B lymphoma type, 3 cases were confirmed diffuse large B-cell lymphoma, angioimmunoblastic T-cell lymphoma and ALK positive anaplastic large cell lymphoma were one case separately. Clinical and imaging manifestation of PPL is untypical, but there are still some hints: 1) Fuzzy shadow at the edge of lung mass with air bronchogram; 2) Lung mass shadow stable for a long time; 3) Pneumonia-like changing without infections clinical and lab manifestation. Thirteen patients (68.4%) were misdiagnosed as pneumonia, lung cancer and tuberculosis initially. The term between initial diagnosis and final diagnosis lasted for half a month up to 2 years, with median time of 6 months. Two cases were misdiagnosed as tuberculosis. One case was misdiagnosed as small cell lung cancer. Conclusion Clinical and imaging manifestation of PPL is untypical. Biopsies should be taken actively if the imaging findings don’t match the symptoms or the anti-infection treatments to “lung infection” don’t work. Accurate diagnosis requires adequate tissue sampling with appropriate ancillary pathologic studies. If clinical manifestation and the diagnosis don’t match, repeated biopsy should be ordered.

Pulmonary lymphoproliferative neoplasms are rare lung tumors and account for

Primary pulmonary lymphoma is an uncommon disease, and extranodal marginal zone lymphoma of mucosa‐associated lymphoid tissue (MALT) is the most common type of pulmonary lymphoma. The most frequent pattern observed in chest computed tomography (CT) is consolidation, followed by nodules and mass. The differentiation of pulmonary MALT lymphoma from other lung diseases is critical for disease management and treatment. However, pulmonary MALT lymphoma with isolated endobronchial manifestation has seldomly been reported. Here, we report a case of an elderly woman who presented with a four‐month history of cough, dyspnoea, and haemoptysis. Chest CT scan revealed left main bronchus narrowing without lung parenchymal lesion. Bronchoscopic examination was performed, and the diagnosis of primary pulmonary MALT lymphoma with isolated endobronchial involvement was made. She has been successfully treated with rituximab.We describe a case of primary pulmonary mucosa‐associated lymphoid tissue (MALT) lymphoma presented as an endobronchial tumor without lung parenchymal involvement. [ABSTRACT FROM AUTHOR]

Primary pulmonary Hodgkins lymphoma is a rare and enigmatic neoplasm commonly seen in females. The aim of this case report is to emphasize that pulmonary lymphoma should be considered as a differential diagnosis in a young patient presenting with a lung mass and B symptoms. Extensive clinical details, imaging should be done to rule out the secondary involvement of lung by other lymphomas. [ABSTRACT FROM AUTHOR]

Primary pulmonary diffuse large B cell lymphoma (PPDLBCL) is extremely rare, with fewer than 40 cases reported to date and a lack of systemic analysis. Herein, we present a case of PPDLBCL mimicking metastasis in a heavily treated patient with breast cancer. To our knowledge, this is the first reported case of PPDLBCL in a patient with breast cancer. A 66-year-old Chinese female diagnosed with breast cancer 7.5 years previously and multiple bone metastases 31 months later presented with a new-onset subpleural nodule in the inferior lobe of left lung detected by routine follow-up in November 2017. A 18F-fluorodeoxyglucose (FDG) positron emission tomography-computed tomography scan showed that the pulmonary nodule was hypermetabolic with a maximum standard uptake value of 14.9, consistent with lung metastasis in view of her history of breast cancer and multiple bone involvement. Surprisingly, pathologic investigation revealed primary lung DLBCL, staged IEA. Systemic chemotherapy with R-CDOP (rituximab, cyclophosphamide, vindesine, doxorubicin liposome, and prednisone) achieved complete remission with mild side effects. At the latest follow-up in August 2019, the patient had disease-free survival of 21 months. The findings from this case indicate that primary pulmonary lymphoma should be included in the differential diagnostic checklist of pulmonary occupancy, even in solid tumor patients treated with multiple modalities. When a newly developed lung nodule is identified in such patients, clinicians should not take for granted that it is lung metastasis. Pathology results are a prerequisite for making a correct diagnosis, choosing appropriate treatment, and improving patient prognosis. [ABSTRACT FROM AUTHOR]

Primary pulmonary lymphomas (PPLs) are rare lymphoproliferative malignancies arising from the lungs. The prognostic factors and optimal management of PPL have not been clearly defined due to its rarity. This study sought to characterize the significant prognostic factors and develop a validated nomogram for individualized prediction of survival outcomes in patients with PPL. A total of 2325 patients diagnosed with PPL between 1983 and 2010 were identified using the Surveillance, Epidemiology, and End Results (SEER) database. Older age at diagnosis, males, Hispanic race, non-marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue histology, Ann Arbor stage IV were significantly associated with worse OS on multivariable analysis. All treatment modalities, including chemotherapy, surgery, and radiotherapy were independent predictors of survival on univariable analysis. The nomogram built demonstrated good discriminative ability and calibration, with the C-index of 0.690 and 0.730 in the training and validation cohorts, respectively. [ABSTRACT FROM AUTHOR]

A 20-year-old female patient was admitted to hospital with complaints of chest and back pain in September 2018. There was a cavitary lesion in the upper zone of the left lung in the chest X-ray. Thorax CT revealed an irregular contoured and shaped mass with 87x67x79 mm sizes, in the upper lobe of the left lung lying to paramediastinal area. Since there was a doubt about malignancy, positron emission tomography (PET) was performed; there was a cavitary lesion in the left upper lobe with high FDG uptake (SUVmax: 23.2). Bronchoscopic examination revealed an endobronchial lesion with nearly complete occlusion in the apicoposterior segment of the left upper lobe. Bronchoalveolar lavage (BAL) performed in this session for acid-fast bacilli (AFB) was negative. The patient was diagnosed as primary pulmonary diffuse large B-cell lymphoma (DLBCL) by histopathological and immunohistochemical evaluation of endobronchial biopsy specimens. Following the final diagnosis of Bronchus-Associated Lymphoid Tissue Lymphoma (BALTOMA), the patient was referred to the department of haematology, and chemotherapy was planned for therapy. Since DLBCL is extremely rare, and uncommonly presenting with an endobronchial lesion, we want to present this patient as the youngest adult case of primary endobronchial BALT lymphoma in the literature. [ABSTRACT FROM AUTHOR]

Background: Primary pulmonary lymphoma (PPL) is a rare disease, especially in children. We analyse the clinical features of PPL in 4 children to strengthen a understanding of it.Results: We reported a case series of 4 pediatric patients with PPLs including three diffuse large B-cell lymphomas and one natural killer-T cell lymphoma. All patients presented with unknown fever and cough as well as weight loss and fatigue. The white blood cell count was reduced in three patients and increased in the other one. The level of C-reactive protein was increased in all patients. The procalcitonin concentrations and bone marrow specimens were normal. Multiple or single pulmonary nodules with halo signs were found in all patients and air bronchograms found in 3 of them on chest computed tomography scan. Primary immunodeficiency was diagnosed in two patients who was performed genetic analysis.Conclusions: When a patient presents with long-term fever, high C-reactive protein level, leukopenia/leukocytosis, and multiple or single pulmonary nodules with a "halo sign" and air bronchogram on computed tomography, a possibility of PPL should be considered. A co-existance of immunodeficiency needs to be further investigated in patients with PPL. [ABSTRACT FROM AUTHOR]

Abstract Primary pulmonary mucosa-associated lymphoid tissue (MALT) lymphoma is extremely rare. MALT lymphoma patients usually show no clinical symptoms or physical signs. Chest radiograph or computed tomography (CT) may confuse MALT lymphoma with other pulmonary diseases, which would lead to misdiagnosis or a delayed diagnosis. In the present study, a 33-year-old male patient had cough and fever. Chest CT showed consolidation on both sides. Those clinical symptoms disappeared after he had been misdiagnosed and treated for community-acquired pneumonia for three weeks. However, further chest CT still showed the consolidation without any change. Then an ultrasonic guided transthoracic needle biopsy was performed. Morphological changes indicated the diagnosis of extranodal marginal Zone B cell lymphoma of MALT. The patient was then treated with chemotherapy and rituximab. After this line of treatment, the consolidation decreased. [ABSTRACT FROM AUTHOR]

Extranodal natural killer (NK)/T-cell lymphoma (ENKTL) is an aggressive disease with poor prognosis. The lung is a relatively rare site of involvement. The current study presents a case of primary pulmonary ENKTL with fever and dyspnea, mimicking pneumonia and initially treated with empirical antibiotics. The patient demonstrated rapid deterioration and died shortly following diagnosis. To the best of our knowledge, large-scale investigations referring to primary pulmonary ENKTL are not available. As a result, the exact incidence and clinical features of primary pulmonary ENKTL are unknown. In the current report, a literature review is presented to discuss the clinical characteristics, diagnosis, treatment, and prognosis factors of this malignant disease. [ABSTRACT FROM AUTHOR]

Pulmonary lymphoproliferative neoplasms are rare lung tumors and account for <1% of all lung tumors. Among them, primary pulmonary lymphomas (PPL) constitute the majority, which include Non-Hodgkin's lymphoma (NHL) that comprise of mucosa-associated lymphoid tissue lymphoma, diffuse large B-cell lymphomas and other rare types of NHL and lymphomatoid granulomatosis. HL, which arises secondary to contiguous spread from the mediastinum, is the rarest type of PPL. Other entities described within the umbrella of pulmonary lymphoproliferative neoplasms include pleural lymphomas and posttransplant lymphoproliferative disorders (PTLD) - which occurs in the poststem cell and organ transplant patients. These neoplasms although rare, have a favorable prognosis, which does not depend on disease resectability. Moreover, with its nonspecific presentation, diagnosis is challenging, which often leads to delayed diagnosis or misdiagnosis in many cases. Therefore, knowledge of this entity is important for the practicing pulmonologist. This review article aims to describe the clinical presentation, diagnosis and management of primarily the entities within PPL, as well as pleural lymphomas and PTLD. [ABSTRACT FROM AUTHOR]

Primary lung lymphomas are rare tumors representing less than 1% of malignant tumors of the lung. The most frequent form is the mucosaassociated lymphoid tissue (MALT). Ocular involvement is also rare and it is mostly located in the lachrymal glands. We report the case of a patient with pulmonary MALT lymphoma associated with synchronous involvement of the lachrymal glands. This study illustrates the nonspecific clinical, radiological and evolutionary features of this disease. [ABSTRACT FROM AUTHOR]

Primary pulmonary lymphoma (PPL) is an extranodally original disorder, which is involving the lung parenchyma and (or) bronchial with clonal lymph cell abnormal proliferation. PPL is very rare in clinics, accounting for only 0.5%-1% of primary lung tumors, and can easily be misdiagnosed or missed due to the nonspecifically clinical features and imaging findings. Although the treatment of this disease has controversial, surgery and postoperative adjuvant chemotherapy are used for most patients in present. This review will report a rare case of primary pulmonary lymphoma, and discuss its diagnosis and treatment.

The aim of the present study was to analyze the differences in imaging and clinical features between primary pulmonary lymphoma (PPL) and secondary pulmonary lymphoma (SPL) to provide insight into pulmonary lymphoma for an improved clinical diagnosis. A retrospective study of 38 patients with pulmonary lymphoma (19 PPL and 19 SPL) treated between September 1, 2006, and December 31, 2015, was performed. The clinical manifestations, and computed tomography (CT) and positron emission tomography-CT images of each case were collected. Χ² and Fisher's exact tests were applied to assess statistically significant differences between PPL and SPL in terms of clinical and imaging features. The significant variables were further applied to canonical discriminate analysis. The CT results revealed that the occurrence of a >3-cm mass (P=0.007), peripheral location (lower than the segmental bronchi) (P=0.027), cavitation (P=0.008) and consolidation (P=0.027) were associated with PPL, while peripheral and hilar location (P=0.003) or mediastinal and hilar lymph node engagement (P=0.044) were predominantly observed in SPL. However, no significant differences between clinical manifestations and the maximum standard uptake value of pulmonary lesions in PPL and SPL were identified (all P>0.05). A function derived from discriminate analysis was generated that may predict the affiliation to PPL or SPL radiographically, with an overall accuracy of ≤92.1%. The results of the present study revealed that PPL and SPL exhibit distinctive features on CT images due to distinct molecular mechanisms and growth patterns. Careful observation of CT features may be useful in the diagnosis of PPL and SPL regarding the tumor morphology, location and lymph node involvement. [ABSTRACT FROM AUTHOR]

Opinion Statement: Lung carcinosarcoma (PCS) and other histological subtypes of non-small cell lung cancer, such as primary pulmonary lymphoma (PPL), pulmonary carcinoid (PC), and primary pulmonary lymphoepithelioma-like carcinoma (LELC), are rare. For their low incidence, the diagnosis and treatment are still controversial. Some patients only need surgery, while others may need chemotherapy, radiotherapy, or targeted therapy. In this paper, we retrospectively reviewed the literature of some rare histological subtype of NSCLC for the recent 20 years, and try to get some conclusions. [ABSTRACT FROM AUTHOR]

This review aims to describe some of the most frequent lymphoproliferative disorders arising from the lung: pulmonary mucosa-associated lymphoid tissue (MALT) lymphoma, lymphomatoid granulomatosis (LG), multicentric Castleman disease (MCD), primary effusion lymphoma (PEL), and nodular lymphoid hyperplasia (NLH). Primary pulmonary lymphoma is defined as a clonal lymphoproliferative disorder affecting one or both lungs, without extrapulmonary involvement 3 months after diagnosis, and includes pulmonary MALT lymphoma and LG. MALT lymphoma is the most common pulmonary lymphoma. The disease is slow growing, most often asymptomatic, and revealed by chronic alveolar opacity on radiography. The diagnosis should involve minimally invasive techniques, and the prognosis is typically excellent. LG is a rare B-cell lymphoma driven by Epstein-Barr virus infection. The disease may mimic pulmonary vasculitis, often revealed by systemic signs. The diagnosis usually requires surgical lung biopsy. Its evolution is unpredictable, but median survival is poor and chemotherapy is usually proposed. MCD and PEL are both driven by Human herpesvirus 8 infection. Patients with MCD present with fever and lymphadenopathy associated with interstitial lung disease. PEL provokes a febrile, lymphocytic-exudative pleural effusion, without any pleural mass on CT. Specific chemotherapy is urgent for both MCD and PEL. NLH is a benign lymphoproliferative disorder of the lung that is usually asymptomatic and revealed by a single nodular opacity. The prognosis is good, without recurrence after surgical resection. [ABSTRACT FROM AUTHOR]

Primary pulmonary lymphoma (PPL) is an uncommon entity of non-Hodgkin lymphoma, which accounts for <1% of all cases of lymphoma. We present two rare cases of PPL of diffuse large B-cell lymphoma, which underwent 18fluorine fluoro-deoxy-glucose positron emission tomography-computed tomography for initial staging and response evaluation after chemotherapy. [ABSTRACT FROM AUTHOR]