Hematolymphoid tumors of the lung
2024-10-30 Paola Dal Cin, PhD Affiliation1.Brigham and Women's Hospital , Harvard Medical School, Boston , MA (USA)
Keywords
Extranodal MALT lymphoma,Primary pulmonary DLBC, Lymphomatoid granulomatosis,Erdheim-Chester disease ,Langerhans cell histiocytosisClassification
Definition
Secondary involvement of hematolymphoid disorders is much more common than primary involvement in the lung. Primary lymphoid proliferations of the lung are of B-cell lineage e.g. pulmonary MALT lymphoma, pulmonary DLBCL and lymphomatoid granulomatosis. 1-3 Extranodal MALT lymphoma represents the most common type of primary lymphoma of the lung, but Helicobacter pylori has not been identified in pulmonary MALT lymphomas. 4 Primary pulmonary DLBCL are rare, and no specific genetic alterations were reported, so far. Lymphomatoid granulomatosis is an EBV-associated B-cell lymphoproliferative disorder. Vemurafenib had prolonged efficacy in patients with pulmonary Erdheim-Chester disease and Langerhans cell histiocytosis since both carrying BRAF V600–mutant.5
| Hematolymphoid tumors of the lung | |
|---|---|
| Pulmonary MALT lymphoma | Extranodal MALT lymphoma is the most common type of primary lymphoma of the lung, but Helicobacter pylori has not been identified in pulmonary MALT. 4 The most common cytogenetic abnormality in the lung is the t(11;18)(q21;q21)/ BIRC3::MALT1, follow by t(14;18)(q32;q21)/IGH::MALT1 ; t(1;14)(p22;q32)/ IGH::BCL10 and trisomies 3 and 18 (even not diagnostic ). Mutations of POT1,TET2, TNFAIP3, TNFRSF14 and TBL1XR1 , and rarely MYD88 were also observed in the lung .6 |
| Primary pulmonary DLBCL | Litle is known about other genetic abnormalities occurring specifically in primary pulmonary DLBCL. |
| Lymphomatoid granulomatosis | EBV-associated B-cell lymphoproliferative disorder. 7 |
| Intravascular large B-cell lymphoma of the lung (IVLBCL) | The most frequent mutation was PIM1 (60%) , follow by MYD88 L265P and CD79B,with both later mutations detected concomitantly. Other less recurrent mutations were in IRF4 , TMEM30A , BTG2 , and ETV6. 8 |
| Pulmonary Langerhans Cell Histiocytosis (LCH) | The BRAF V 600E , MAP2K1 mutation/deletion and BRAF deletions are the main MAPK pathway alterations in these lesions. Other other MAPK molecular alterations have been also identiied e.g.,NRAS,KRAS,ARAF , MAP3K ,and MAP3K8. 9,10 |
| Pulmonary Erdheim-Chester disease(ECD) | The BRAF V 600E muatition is also the main MAPK pathway alteration in these lesions , followed by MAP2K1, KRAS or NRAS mutations, and very rare BRAF deletions, MAP3K1amp, ARAF mutations. 11 Activating PIK3CA mutations have been also found.12 |
Article Bibliography
| Reference Number | Pubmed ID | Last Year | Title | Authors |
|---|---|---|---|---|
| 1 | 28609772 | 2017 | Lymphoproliferative Disorders of the Lung. | Borie R et al |
| 2 | 32444246 | 2020 | Primary Pulmonary B-cell Lymphoma. | Kelemen K et al |
| 3 | 33499258 | 2021 | Primary Pulmonary B-Cell Lymphoma: A Review and Update. | Sanguedolce F et al |
| 4 | 26152851 | 2016 | A retrospective international study on primary extranodal marginal zone lymphoma of the lung (BALT lymphoma) on behalf of International Extranodal Lymphoma Study Group (IELSG). | Sammassimo S et al |
| 5 | 29188284 | 2018 | Vemurafenib for BRAF V600-Mutant Erdheim-Chester Disease and Langerhans Cell Histiocytosis: Analysis of Data From the Histology-Independent, Phase 2, Open-label VE-BASKET Study. | Diamond EL et al |
| 6 | 35008340 | 2021 | Recent Advances in the Genetic of MALT Lymphomas. | Rodríguez-Sevilla JJ et al |
| 7 | 32107539 | 2020 | Pathobiology and treatment of lymphomatoid granulomatosis, a rare EBV-driven disorder. | Melani C et al |
| 8 | 36221796 | 2023 | Intravascular Large B-Cell Lymphoma Genomic Profile Is Characterized by Alterations in Genes Regulating NF-κB and Immune Checkpoints. | Gonzalez-Farre B et al |
| 9 | 33763431 | 2020 | Update on Pulmonary Langerhans Cell Histiocytosis. | Radzikowska E et al |
| 10 | 37410483 | 2023 | Pulmonary Langerhans cell histiocytosis - an update on pathogenesis and treatment. | Jouenne F et al |
| 11 | 32107533 | 2020 | Erdheim-Chester disease. | Haroche J et al |
| 12 | 25150293 | 2014 | Recurrent RAS and PIK3CA mutations in Erdheim-Chester disease. | Emile JF et al |
Citation
Paola Dal Cin, PhD
Hematolymphoid tumors of the lung
Atlas Genet Cytogenet Oncol Haematol. 2024-10-30
Online version: http://atlasgeneticsoncology.org/solid-tumor/209282/hematolymphoid-tumors-of-the-lung
