The 2015 World Health Organization Classification of Lung Tumors

Travis, 2015 Fritz, 2000 Travis, 2004 World Health Organization, 1967 World Health Organization, 1981 Travis, 1999 Travis, 2011, The new IASLC/ATS/ERS international multidisciplinary lung adenocarcinoma classification, J Thoracic Oncol, 6, 244, 10.1097/JTO.0b013e318206a221 2015, Nivolumab approved for lung cancer, Cancer Discov, 5, OF1, 10.1158/2159-8290.CD-NB2015-042 Lindeman, 2013, J Thorac Oncol, 8, 823, 10.1097/JTO.0b013e318290868f Leighl, 2014, J Clin Oncol, 32, 3673, 10.1200/JCO.2014.57.3055 Travis, 2013, Diagnosis of lung cancer in small biopsies and cytology: implications of the 2011 International Association for the Study of Lung Cancer/American Thoracic Society/European Respiratory Society classification, Arch Pathol Lab Med, 137, 668, 10.5858/arpa.2012-0263-RA Ou, 2009, Carcinoma NOS is a common histologic diagnosis and is increasing in proportion among non-small cell lung cancer histologies, J Thorac Oncol, 4, 1202, 10.1097/JTO.0b013e3181b28fb9 Loo, 2010, Subtyping of undifferentiated non-small cell carcinomas in bronchial biopsy specimens, J Thorac Oncol, 5, 442, 10.1097/JTO.0b013e3181d40fac Nicholson, 2010, Refining the diagnosis and EGFR status of non-small cell lung carcinoma in biopsy and cytologic material, using a panel of mucin staining, TTF-1, cytokeratin 5/6, and P63, and EGFR mutation analysis, J Thorac Oncol, 5, 436, 10.1097/JTO.0b013e3181c6ed9b Edwards, 2000, Preoperative histological classification of primary lung cancer: accuracy of diagnosis and use of the non-small cell category, J Clin Pathol, 53, 537, 10.1136/jcp.53.7.537 Travis, 2010, Pathologic diagnosis of advanced lung cancer based on small biopsies and cytology: a paradigm shift, J Thorac Oncol, 5, 411, 10.1097/JTO.0b013e3181d57f6e Turner, 2012, Napsin A, a new marker for lung adenocarcinoma, is complementary and more sensitive and specific than thyroid transcription factor 1 in the differential diagnosis of primary pulmonary carcinoma: evaluation of 1674 cases by tissue microarray, Arch Pathol Lab Med, 136, 163, 10.5858/arpa.2011-0320-OA Bishop, 2012, p40 (ΔNp63) is superior to p63 for the diagnosis of pulmonary squamous cell carcinoma, Mod Pathol, 25, 405, 10.1038/modpathol.2011.173 Pelosi, 2012, ΔN63 (p40) and thyroid transcription factor-1 immunoreactivity on small biopsies or cellblocks for typing non-small cell lung cancer: a novel two-hit, sparing-material approach, J Thorac Oncol, 7, 281, 10.1097/JTO.0b013e31823815d3 Nonaka, 2012, A study of ΔN63 expression in lung non-small cell carcinomas, Am J Surg Pathol, 36, 895, 10.1097/PAS.0b013e3182498f2b Rekhtman, 2013, Distinct profile of driver mutations and clinical features in immunomarker-defined subsets of pulmonary large-cell carcinoma, Mod Pathol, 26, 511, 10.1038/modpathol.2012.195 Travis, 2013, New pathologic classification of lung cancer: relevance for clinical practice and clinical trials, J Clin Oncol, 31, 992, 10.1200/JCO.2012.46.9270 Cancer Genome Atlas Research Network, 2012, Comprehensive genomic characterization of squamous cell lung cancers, Nature, 489, 519, 10.1038/nature11404 Cancer Genome Atlas Research Network, 2014, Comprehensive molecular profiling of lung adenocarcinoma, Nature, 511, 543, 10.1038/nature13385 Clinical Lung Cancer Genome Project (CLCGP), 2013, A genomics-based classification of human lung tumors, Sci Transl Med, 5, 209ra153 Barletta, 2010, Prognostic significance of grading in lung adenocarcinoma, Cancer, 116, 659, 10.1002/cncr.24831 Kadota, 2012, A grading system combining architectural features and mitotic count predicts recurrence in stage I lung adenocarcinoma, Mod Pathol, 25, 1117, 10.1038/modpathol.2012.58 Nakazato, 2013, Interobserver agreement in the nuclear grading of primary pulmonary adenocarcinoma, J Thorac Oncol, 8, 736, 10.1097/JTO.0b013e318288dbd8 Nakazato, 2010, Nuclear grading of primary pulmonary adenocarcinomas: correlation between nuclear size and prognosis, Cancer, 116, 2011, 10.1002/cncr.24948 Sica, 2010, A grading system of lung adenocarcinomas based on histologic pattern is predictive of disease recurrence in stage I tumors, Am J Surg Pathol, 34, 1155, 10.1097/PAS.0b013e3181e4ee32 Yoshizawa, 2011, Impact of proposed IASLC/ATS/ERS classification of lung adenocarcinoma: prognostic subgroups and implications for further revision of staging based on analysis of 514 stage I cases, Mod Pathol, 24, 653, 10.1038/modpathol.2010.232 Warth, 2012, The novel histologic International Association for the Study of Lung Cancer/American Thoracic Society/European Respiratory Society classification system of lung adenocarcinoma is a stage-independent predictor of survival, J Clin Oncol, 30, 1438, 10.1200/JCO.2011.37.2185 Duhig, 2015, Mitosis trumps T stage and proposed international association for the study of lung cancer/American thoracic society/European respiratory society classification for prognostic value in resected stage 1 lung adenocarcinoma, J Thorac Oncol, 10, 673, 10.1097/JTO.0000000000000446 von der Thüsen, 2013, Prognostic significance of predominant histologic pattern and nuclear grade in resected adenocarcinoma of the lung: potential parameters for a grading system, J Thorac Oncol, 8, 37, 10.1097/JTO.0b013e318276274e Kadota, 2014, Comprehensive pathological analyses in lung squamous cell carcinoma: single cell invasion, nuclear diameter, and tumor budding are independent prognostic factors for worse outcomes, J Thorac Oncol, 9, 1126, 10.1097/JTO.0000000000000253 Sigel, 2012, Predicting pulmonary adenocarcinoma outcome based on a cytology grading system, Cancer Cytopathol, 120, 35, 10.1002/cncy.20185 Kadota, 2015, Tumor budding correlates with protumor immune microenvironment and is an independent prognostic factor for recurrence of stage I lung adenocarcinoma, Chest, 10.1378/chest.14-3005 Yamaguchi, 2010, Histopathologic features of the tumor budding in adenocarcinoma of the lung: tumor budding as an index to predict the potential aggressiveness, J Thorac Oncol, 5, 1361, 10.1097/JTO.0b013e3181eaf2f3 Taira, 2012, Characterization of the immunophenotype of the tumor budding and its prognostic implications in squamous cell carcinoma of the lung, Lung Cancer, 76, 423, 10.1016/j.lungcan.2011.11.010 Travis, 2013, Diagnosis of lung adenocarcinoma in resected specimens: implications of the 2011 International Association for the Study of Lung Cancer/American Thoracic Society/European Respiratory Society classification, Arch Pathol Lab Med, 137, 685, 10.5858/arpa.2012-0264-RA Kadota, 2015, Reevaluation and reclassification of resected lung carcinomas originally diagnosed as squamous cell carcinoma using immunohistochemical analysis, Am J Surg Pathol, 10.1097/PAS.0000000000000439 Rekhtman, 2012, Clarifying the spectrum of driver oncogene mutations in biomarker-verified squamous carcinoma of lung: lack of EGFR/KRAS and presence of PIK3CA/AKT1 mutations, Clin Cancer Res, 18, 1167, 10.1158/1078-0432.CCR-11-2109 Kadota, 2014, Prognostic significance of adenocarcinoma in situ, minimally invasive adenocarcinoma, and nonmucinous lepidic predominant invasive adenocarcinoma of the lung in patients with stage I disease, Am J Surg Pathol, 38, 448, 10.1097/PAS.0000000000000134 Lim, 2013, Persistent pure ground-glass opacity lung nodules ≥ 10 mm in diameter at CT scan: histopathologic comparisons and prognostic implications, Chest, 144, 1291, 10.1378/chest.12-2987 Lee, 2014, Pure ground-glass opacity neoplastic lung nodules: histopathology, imaging, and management, AJR Am J Roentgenol, 202, W224, 10.2214/AJR.13.11819 Isaka, 2014, Comparison between CT tumor size and pathological tumor size in frozen section examinations of lung adenocarcinoma, Lung Cancer, 85, 40, 10.1016/j.lungcan.2014.03.023 Naidich, 2013, Recommendations for the management of subsolid pulmonary nodules detected at CT: a statement from the Fleischner Society, Radiology, 266, 304, 10.1148/radiol.12120628 Girard, 2009, Comprehensive histologic assessment helps to differentiate multiple lung primary non-small cell carcinomas from metastases, Am J Surg Pathol, 33, 1752, 10.1097/PAS.0b013e3181b8cf03 Kadota, 2014, The cribriform pattern identifies a subset of acinar predominant tumors with poor prognosis in patients with stage I lung adenocarcinoma: a conceptual proposal to classify cribriform predominant tumors as a distinct histologic subtype, Mod Pathol, 27, 690, 10.1038/modpathol.2013.188 Mackinnon, 2014, Cribriform adenocarcinoma of the lung: clinicopathologic, immunohistochemical, and molecular analysis of 15 cases of a distinctive morphologic subtype of lung adenocarcinoma, Mod Pathol, 27, 1063, 10.1038/modpathol.2013.227 Warth, 2015, Prognostic impact and clinicopathological correlations of the cribriform pattern in pulmonary adenocarcinoma, J Thorac Oncol, 10, 638, 10.1097/JTO.0000000000000490 Thunnissen, 2012, Reproducibility of histopathological subtypes and invasion in pulmonary adenocarcinoma. An international interobserver study, Mod Pathol, 25, 1574, 10.1038/modpathol.2012.106 Warth, 2012, Training increases concordance in classifying pulmonary adenocarcinomas according to the novel IASLC/ATS/ERS classification, Virchows Arch, 461, 185, 10.1007/s00428-012-1263-6 Nitadori, 2013, Impact of micropapillary histologic subtype in selecting limited resection vs lobectomy for lung adenocarcinoma of 2cm or smaller, J Natl Cancer Inst, 105, 1212, 10.1093/jnci/djt166 Onozato, 2013, Tumor islands in resected early-stage lung adenocarcinomas are associated with unique clinicopathologic and molecular characteristics and worse prognosis, Am J Surg Pathol, 37, 287, 10.1097/PAS.0b013e31826885fb Kadota, 2015, Tumor spread through air spaces is an important pattern of invasion and impacts the frequency and location of recurrences after limited resection for small stage I lung adenocarcinomas, J Thorac Oncol, 10, 806, 10.1097/JTO.0000000000000486 Warth, 2015, Prognostic impact of intraalveolar tumor spread in pulmonary adenocarcinoma, Am J Surg Pathol, 39, 793, 10.1097/PAS.0000000000000409 Loukeri, 2015, Metachronous and synchronous primary lung cancers: diagnostic aspects, surgical treatment, and prognosis, Clin Lung Cancer, 16, 15, 10.1016/j.cllc.2014.07.001 Klempner, 2015, The clinical use of genomic profiling to distinguish intrapulmonary metastases from synchronous primaries in non-small-cell lung cancer: a mini-review, Clin Lung Cancer, 10.1016/j.cllc.2015.03.004 Murphy, 2014, Identification of independent primary tumors and intrapulmonary metastases using DNA rearrangements in non-small-cell lung cancer, J Clin Oncol, 32, 4050, 10.1200/JCO.2014.56.7644 Girard, 2010, Use of epidermal growth factor receptor/Kirsten rat sarcoma 2 viral oncogene homolog mutation testing to define clonal relationships among multiple lung adenocarcinomas: comparison with clinical guidelines, Chest, 137, 46, 10.1378/chest.09-0325 Girard, 2009, Genomic and mutational profiling to assess clonal relationships between multiple non-small cell lung cancers, Clin Cancer Res, 15, 5184, 10.1158/1078-0432.CCR-09-0594 Xue, 2013, Diagnosis of multiple primary lung cancer: a systematic review, J Int Med Res, 41, 1779, 10.1177/0300060513504707 Song, 2013, Prognostic value of the IASLC/ATS/ERS classification in stage I lung adenocarcinoma patients–based on a hospital study in China, Eur J Surg Oncol, 39, 1262, 10.1016/j.ejso.2013.08.026 Takahashi, 2014, Tumor invasiveness as defined by the newly proposed IASLC/ATS/ERS classification has prognostic significance for pathologic stage IA lung adenocarcinoma and can be predicted by radiologic parameters, J Thorac Cardiovasc Surg, 147, 54, 10.1016/j.jtcvs.2013.08.058 Tsuta, 2013, The utility of the proposed IASLC/ATS/ERS lung adenocarcinoma subtypes for disease prognosis and correlation of driver gene alterations, Lung Cancer, 81, 371, 10.1016/j.lungcan.2013.06.012 Yanagawa, 2013, New IASLC/ATS/ERS classification and invasive tumor size are predictive of disease recurrence in stage I lung adenocarcinoma, J Thorac Oncol, 8, 612, 10.1097/JTO.0b013e318287c3eb Yanagawa, 2014, The correlation of the International Association for the Study of Lung Cancer (IASLC)/American Thoracic Society (ATS)/European Respiratory Society (ERS) classification with prognosis and EGFR mutation in lung adenocarcinoma, Ann Thorac Surg, 98, 453, 10.1016/j.athoracsur.2014.04.108 Yoshizawa, 2013, Validation of the IASLC/ATS/ERS lung adenocarcinoma classification for prognosis and association with EGFR and KRAS gene mutations: analysis of 440 Japanese patients, J Thorac Oncol, 8, 52, 10.1097/JTO.0b013e3182769aa8 Russell, 2011, J Thorac Oncol, 6, 1496, 10.1097/JTO.0b013e318221f701 Woo, 2012, Prognostic value of the IASLC/ATS/ERS classification of lung adenocarcinoma in stage I disease of Japanese cases, Pathol Int, 62, 785, 10.1111/pin.12016 Cha, 2014, Micropapillary and solid subtypes of invasive lung adenocarcinoma: clinical predictors of histopathology and outcome, J Thorac Cardiovasc Surg, 147, 921, 10.1016/j.jtcvs.2013.09.045 Nitadori, 2013, Impact of micropapillary histologic subtype in selecting limited resection vs lobectomy for lung adenocarcinoma of 2 cm or smaller, J Natl Cancer Inst, 105, 1212, 10.1093/jnci/djt166 Ujiie, 2015, Solid predominant histologic subtype in resected stage I lung adenocarcinoma is an independent predictor of early, extrathoracic, multisite recurrence and of poor postrecurrence survival, J Clin Oncol, 10.1200/JCO.2015.60.9818 Tsao, 2015, Subtype classification of lung adenocarcinoma predicts benefit from adjuvant chemotherapy in patients undergoing complete resection, J Clin Oncol, 10.1200/JCO.2014.58.8335 Brambilla, 2014, Lung squamous cell carcinomas with basaloid histology represent a specific molecular entity, Clin Cancer Res, 20, 5777, 10.1158/1078-0432.CCR-14-0459 Barnes, 2005 Brambilla, 1992, Basal cell (basaloid) carcinoma of the lung: a new morphologic and phenotypic entity with separate prognostic significance, Hum Pathol, 23, 993, 10.1016/0046-8177(92)90260-A Moro-Sibilot, 2008, Lung carcinomas with a basaloid pattern: a study of 90 cases focusing on their poor prognosis, Eur Respir J, 31, 854, 10.1183/09031936.00058507 Kim, 2003, Basaloid carcinoma of the lung: a really dismal histologic variant?, Ann Thorac Surg, 76, 1833, 10.1016/S0003-4975(03)01296-7 Wang, 2011, Analysis on the clinical features of 22 basaloid squamous cell carcinoma of the lung, J Cardiothorac Surg, 6, 10, 10.1186/1749-8090-6-10 Herbst, 2015, Lung master protocol (Lung-MAP)-A biomarker-driven protocol for accelerating development of therapies for squamous cell lung cancer: SWOG S1400, Clin Cancer Res, 21, 1514, 10.1158/1078-0432.CCR-13-3473 Hwang, 2014, Pulmonary large cell carcinoma lacking squamous differentiation is clinicopathologically indistinguishable from solid-subtype adenocarcinoma, Arch Pathol Lab Med, 138, 626, 10.5858/arpa.2013-0179-OA Rossi, 2014, Large cell carcinoma of the lung: clinically oriented classification integrating immunohistochemistry and molecular biology, Virchows Arch, 464, 61, 10.1007/s00428-013-1501-6 2013, (NGM) CLCGPCNGM. A genomics-based classification of human lung tumors, Sci Transl Med, 5, 209ra153 Lewis, 2014, US lung cancer trends by histologic type, Cancer, 120, 2883, 10.1002/cncr.28749 Caplin, 2015, Pulmonary neuroendocrine (carcinoid) tumors: European neuroendocrine tumor society expert consensus and recommendations for best practice for typical and atypical pulmonary carcinoid, Ann Oncol, 26, 1604, 10.1093/annonc/mdv041 Fernandez-Cuesta, 2014, Frequent mutations in chromatin-remodelling genes in pulmonary carcinoids, Nat Commun, 5, 3518, 10.1038/ncomms4518 Pelosi, 2014, Ki-67 antigen in lung neuroendocrine tumors: unraveling a role in clinical practice, J Thorac Oncol, 9, 273, 10.1097/JTO.0000000000000092 Pelosi, 2005, Typical and atypical pulmonary carcinoid tumor overdiagnosed as small-cell carcinoma on biopsy specimens: a major pitfall in the management of lung cancer patients, Am J Surg Pathol, 29, 179, 10.1097/01.pas.0000149690.75462.29 Walts, 2012, Limited role of Ki-67 proliferative index in predicting overall short-term survival in patients with typical and atypical pulmonary carcinoid tumors, Mod Pathol, 25, 1258, 10.1038/modpathol.2012.81 Travis, 1998, Survival analysis of 200 pulmonary neuroendocrine tumors with clarification of criteria for atypical carcinoid and its separation from typical carcinoid, Am J Surg Pathol, 22, 934, 10.1097/00000478-199808000-00003 Yendamuri, 2012, Outcomes of sarcomatoid carcinoma of the lung: a surveillance, epidemiology, and end results database analysis, Surgery, 152, 397, 10.1016/j.surg.2012.05.007 Italiano, 2009, EGFR and KRAS status of primary sarcomatoid carcinomas of the lung: implications for anti-EGFR treatment of a rare lung malignancy, Int J Cancer, 125, 2479, 10.1002/ijc.24610 Pelosi, 2004, K-ras gene mutational analysis supports a monoclonal origin of biphasic pleomorphic carcinoma of the lung, Mod Pathol, 17, 538, 10.1038/modpathol.3800058 Chang, 2011, EGFR and p53 status of pulmonary pleomorphic carcinoma: implications for EGFR tyrosine kinase inhibitors therapy of an aggressive lung malignancy, Ann Surg Oncol, 18, 2952, 10.1245/s10434-011-1621-7 Kaira, 2010, Pulmonary pleomorphic carcinoma: a clinicopathological study including EGFR mutation analysis, J Thorac Oncol, 5, 460, 10.1097/JTO.0b013e3181ce3e3c Leone, 2011, Identification of EGFR mutations in lung sarcomatoid carcinoma, Int J Cancer, 128, 732, 10.1002/ijc.25335 Lee, 2011, Molecular profiles of EGFR, K-ras, c-met, and FGFR in pulmonary pleomorphic carcinoma, a rare lung malignancy, J Cancer Res Clin Oncol, 137, 1203, 10.1007/s00432-011-0986-0 Dacic, 2002, Molecular pathogenesis of pulmonary carcinosarcoma as determined by microdis-section-based allelotyping, Am J Surg Pathol, 26, 510, 10.1097/00000478-200204000-00015 Holst, 1997, p53 and K-ras mutational genotyping in pulmonary carcinosarcoma, spindle cell carcinoma, and pulmonary blastoma: implications for histogenesis, Am J Surg Pathol, 21, 801, 10.1097/00000478-199707000-00008 Pardo, 2008, Primary mixed squamous carcinoma and osteosarcoma (carcinosarcomas) of the lung have a CGH mapping similar to primitive squamous carcinomas and osteosarcomas, Diagn Mol Pathol, 17, 151, 10.1097/PDM.0b013e31815d05b3 Pelosi, 2012, Multiparametric molecular characterization of pulmonary sarcomatoid carcinoma reveals a nonrandom amplification of anaplastic lymphoma kinase (ALK) gene, Lung Cancer, 77, 507, 10.1016/j.lungcan.2012.05.093 Toyokawa, 2013, The first case of lung carcinosarcoma harboring in-frame deletions at exon19 in the EGFR gene, Lung Cancer, 81, 491, 10.1016/j.lungcan.2013.06.013 Macher-Goeppinger, 2011, Expression and mutation analysis of EGFR, c-KIT, and β-catenin in pulmonary blastoma, J Clin Pathol, 64, 349, 10.1136/jcp.2010.085696 Nakatani, 2002, Aberrant nuclear localization and gene mutation of beta-catenin in low-grade adenocarcinoma of fetal lung type: up-regulation of the Wnt signaling pathway may be a common denominator for the development of tumors that form morules, Mod Pathol, 15, 617, 10.1038/modpathol.3880575 Sekine, 2003, Beta-catenin mutations in pulmonary blastomas: association with morule formation, J Pathol, 200, 214, 10.1002/path.1352 Bodner, 1996, Mutations in the p53 gene in pulmonary blastomas: immunohistochemical and molecular studies, Hum Pathol, 27, 1117, 10.1016/S0046-8177(96)90302-0 Bauer, 2012, Clinicopathologic features and long-term outcomes of NUT midline carcinoma, Clin Cancer Res, 18, 5773, 10.1158/1078-0432.CCR-12-1153 French, 2004, Midline carcinoma of children and young adults with NUT rearrangement, J Clin Oncol, 22, 4135, 10.1200/JCO.2004.02.107 French, 2010, Demystified molecular pathology of NUT midline carcinomas, J Clin Pathol, 63, 492, 10.1136/jcp.2007.052902 Haack, 2009, Diagnosis of NUT midline carcinoma using a NUT-specific monoclonal antibody, Am J Surg Pathol, 33, 984, 10.1097/PAS.0b013e318198d666 Schwartz, 2011, Differentiation of NUT midline carcinoma by epigenomic reprogramming, Cancer Res, 71, 2686, 10.1158/0008-5472.CAN-10-3513 Devouassoux-Shisheboran, 2000, A clinicopathologic study of 100 cases of pulmonary sclerosing hemangioma with immunohistochemical studies: TTF-1 is expressed in both round and surface cells, suggesting an origin from primitive respiratory epithelium, Am J Surg Pathol, 24, 906, 10.1097/00000478-200007000-00002 Chan, 2000, Pulmonary sclerosing hemangioma consistently expresses thyroid transcription factor-1 (TTF-1): a new clue to its histogenesis, Am J Surg Pathol, 24, 1531, 10.1097/00000478-200011000-00009 Niho, 1998, Monoclonality of both pale cells and cuboidal cells of sclerosing hemangioma of the lung, Am J Pathol, 152, 1065 Dal Cin, 1993, Rearrangement of 12q14–15 in pulmonary chondroid hamartoma, Genes Chromosomes Cancer, 8, 131, 10.1002/gcc.2870080211 Fletcher, 1995, Cytogenetic and histologic findings in 17 pulmonary chondroid hamartomas: evidence for a pathogenetic relationship with lipomas and leiomyomas, Genes Chromosomes Cancer, 12, 220, 10.1002/gcc.2870120310 Xiao, 1997, HMGI(Y) activation by chromosome 6p21 rearrangements in multilineage mesenchymal cells from pulmonary hamartoma, Am J Pathol, 150, 901 Tomashefski, 1982, Benign endobronchial mesenchymal tumors: their relationship to parenchymal pulmonary hamartomas, Am J Surg Pathol, 6, 531, 10.1097/00000478-198209000-00005 Carsillo, 2000, Mutations in the tuberous sclerosis complex gene TSC2 are a cause of sporadic pulmonary lymphangioleiomyomatosis, Proc Natl Acad Sci U S A, 97, 6085, 10.1073/pnas.97.11.6085 Martignoni, 2010, Molecular pathology of lymphangioleiomyomatosis and other perivascular epithelioid cell tumors, Arch Pathol Lab Med, 134, 33, 10.5858/2008-0542-RAR1.1 D’Armiento, 2007, Identification of the benign mesenchymal tumor gene HMGA2 in lymphangiomyomatosis, Cancer Res, 67, 1902, 10.1158/0008-5472.CAN-06-1122 McCormack, 2012, Lymphangioleiomyomatosis: calling it what it is: a low-grade, destructive, metastasizing neoplasm, Am J Respir Crit Care Med, 186, 1210, 10.1164/rccm.201205-0848OE Flieder, 1997, Clear cell “sugar” tumor of the lung: association with lymphangioleiomyomatosis and multifocal micronodular pneumocyte hyperplasia in a patient with tuberous sclerosis, Am J Surg Pathol, 21, 1242, 10.1097/00000478-199710000-00017 Pileri, 2004, Clear-cell proliferation of the lung with lymphangioleiomyomatosis-like change, Histopathology, 44, 156, 10.1111/j.1365-2559.2004.01788.x Lawson, 2012, Successful treatment of progressive diffuse PEComatosis, Eur Respir J, 40, 1578, 10.1183/09031936.00076411 Maruyama, 2001, Multifocal micronodular pneumocyte hyperplasia and lymphangioleiomyomatosis in tuberous sclerosis with a TSC2 gene, Mod Pathol, 14, 609, 10.1038/modpathol.3880359 Gaffey, 1997, Clear cell tumors of the lower respiratory tract, Semin Diagn Pathol, 14, 222 Chen, 2014, Clear cell tumor of the lung: a retrospective analysis, Am J Med Sci, 347, 50, 10.1097/MAJ.0b013e318295c821 Gaffey, 1991, Clear cell tumor of the lung. Immunohistochemical and ultrastructural evidence of melanogenesis, Am J Surg Pathol, 15, 644, 10.1097/00000478-199107000-00005 Lantuejoul, 1997, Clear cell tumor of the lung: an immunohistochemical and ultrastructural study supporting a pericytic differentiation, Mod Pathol, 10, 1001 Inoki, 2005, Dysregulation of the TSC-mTOR pathway in human disease, Nat Genet, 37, 19, 10.1038/ng1494 Anderson, 2015, Thoracic epithelioid malignant vascular tumors: a clinicopathologic study of 52 cases with emphasis on pathologic grading and molecular studies of WWTR1–CAMTA1 fusions, Am J Surg Pathol, 39, 132, 10.1097/PAS.0000000000000346 Lau, 2011, Clinical patterns and outcome in epithelioid hemangioendothelioma with or without pulmonary involvement: insights from an internet registry in the study of a rare cancer, Chest, 140, 1312, 10.1378/chest.11-0039 Mendlick, 2001, Translocation t(1;3) (p36.3;q25) is a nonrandom aberration in epithelioid hemangioendothelioma, Am J Surg Pathol, 25, 684, 10.1097/00000478-200105000-00019 Errani, 2011, A novel WWTR1–CAMTA1 gene fusion is a consistent abnormality in epithelioid hemangioendothelioma of different anatomic sites, Genes Chromosomes Cancer, 50, 644, 10.1002/gcc.20886 Patel, 2015, Molecular characterization of epithelioid haemangioendotheliomas identifies novel WWTR1–CAMTA1 fusion variants, Histopathology, 10.1111/his.12697 Tanas, 2011, Identification of a disease-defining gene fusion in epithelioid hemangioendothelioma, Sci Transl Med, 3, 98ra82, 10.1126/scitranslmed.3002409 Antonescu, 2013, Novel YAP1-TFE3 fusion defines a distinct subset of epithelioid hemangioendothelioma, Genes Chromosomes Cancer, 52, 775, 10.1002/gcc.22073 Bagan, 2006, Prognostic factors and surgical indications of pulmonary epithelioid hemangioendothelioma: a review of the literature, Ann Thorac Surg, 82, 2010, 10.1016/j.athoracsur.2006.06.068 Nicholson, 1999, Malignant myxoid endobronchial tumour: a report of two cases with a unique histological pattern, Histopathology, 35, 313, 10.1046/j.1365-2559.1999.00724.x Thway, 2011, Primary pulmonary myxoid sarcoma with EWSR1-CREB1 fusion: a new tumor entity, Am J Surg Pathol, 35, 1722, 10.1097/PAS.0b013e318227e4d2 Thway, 2012, Endobronchial pulmonary angiomatoid fibrous histiocytoma: two cases with EWSR1-CREB1 and EWSR1-ATF1 fusions, Am J Surg Pathol, 36, 883, 10.1097/PAS.0b013e31824b1ee0 Thway, 2012, Tumors with EWSR1-CREB1 and EWSR1-ATF1 fusions: the current status, Am J Surg Pathol, 36, e1, 10.1097/PAS.0b013e31825485c5 Fletcher, 2013, Myoepithelioma/myoepithelial carcinoma/mixed tumor, 208 Hornick, 2003, Myoepithelial tumors of soft tissue: a clinicopathologic and immunohistochemical study of 101 cases with evaluation of prognostic parameters, Am J Surg Pathol, 27, 1183, 10.1097/00000478-200309000-00001 Hysi, 2011, Primary pulmonary myoepithelial carcinoma, Interact Cardiovasc Thorac Surg, 13, 226, 10.1510/icvts.2011.270934 Kourda, 2010, Benign myoepithelioma of the lung - a case report and review of the literature, Cases J, 3, 25, 10.1186/1757-1626-3-25 Masuya, 2005, Myoepithelial carcinoma of the lung, Eur J Cardiothorac Surg, 28, 775, 10.1016/j.ejcts.2005.08.003 Sarkaria, 2011, Primary myoepithelial carcinoma of the lung: a rare entity treated with parenchymal sparing resection, J Cardiothorac Surg, 6, 27, 10.1186/1749-8090-6-27 Antonescu, 2010, EWSR1-POU5F1 fusion in soft tissue myoepithelial tumors. A molecular analysis of sixty-six cases, including soft tissue, bone, and visceral lesions, showing common involvement of the EWSR1 gene, Genes Chromosomes Cancer, 49, 1114, 10.1002/gcc.20819 Cavalli, 2013, The multifaceted clinical presentations and manifestations of Erdheim–Chester disease: comprehensive review of the literature and of 10 new cases, Ann Rheum Dis, 72, 1691, 10.1136/annrheumdis-2012-202542 Diamond, 2014, Consensus guidelines for the diagnosis and clinical management of Erdheim–Chester disease, Blood, 124, 483, 10.1182/blood-2014-03-561381 Arnaud, 2010, Pulmonary involvement in Erdheim–Chester disease: a single-center study of thirty-four patients and a review of the literature, Arthritis Rheum, 62, 3504, 10.1002/art.27672 Haroche, 2014, Erdheim–Chester disease, Curr Rheumatol Rep, 16, 412, 10.1007/s11926-014-0412-0 Brun, 2010, Erdheim–Chester disease: CT findings of thoracic involvement, Eur Radiol, 20, 2579, 10.1007/s00330-010-1830-7 Rush, 2000, Pulmonary pathology of Erdheim–Chester disease [In Process Citation], ModPathol, 13, 747 Haroche, 2012, High prevalence of BRAF V600E mutations in Erdheim–Chester disease but not in other non-Langerhans cell histiocytoses, Blood, 120, 2700, 10.1182/blood-2012-05-430140 Chetritt, 1999, Chester–Erdheim disease: a neoplastic disorder, Hum Pathol, 30, 1093, 10.1016/S0046-8177(99)90228-9 Vencio, 2007, Clonal cytogenetic abnormalities in Erdheim–Chester disease, Am J Surg Pathol, 31, 319, 10.1097/01.pas.0000213366.33627.a0 Gong, 2009, Clonal status and clinicopathological feature of Erdheim–Chester disease, Pathol Res Pract, 205, 601, 10.1016/j.prp.2009.02.004 Tzoulis, 2015, Excellent response of intramedullary Erdheim–Chester disease to vemurafenib: a case report, BMC Res Notes, 8, 171, 10.1186/s13104-015-1135-7 Haroche, 2015, Reproducible and sustained efficacy of targeted therapy with vemurafenib in patients with BRAF(V600E)-mutated Erdheim–Chester disease, J Clin Oncol, 33, 411, 10.1200/JCO.2014.57.1950