TY - JOUR T1 - CLINICAL AND RADIOLOGICAL EVALUATION IN RARE PRIMARY MALIGNANT TUMORS OF THE LUNG TT - AKCİĞERİN NADİR PRİMER MALİGN TÜMÖRLERİNDE KLİNİK VE RADYOLOJİK DEĞERLENDİRME AU - Öz, Gürhan AU - Özdemir, Çiğdem AU - Aydın, Suphi AU - Dumanlı, Ahmet AU - Günay, Ersin AU - Çilekar, Şule AU - Günay, Sibel AU - Gencer, Adem AU - Öztürk, Düriye AU - Demirağ, Funda PY - 2021 DA - December Y2 - 2020 DO - 10.17343/sdutfd.753812 JF - Medical Journal of Süleyman Demirel University JO - Med J SDU PB - Süleyman Demirel University WT - DergiPark SN - 1300-7416 SP - 551 EP - 558 VL - 28 IS - 4 LA - en AB - ObjectiveThe most common primary malignant tumors of thelung are squamous cell carcinoma, small cell carcinomaand adenocarcinoma. However, some rare malignantprimary lung tumors can also affect the lungand cause difficulties in diagnosis and treatment.Conventional imaging methods do not help the diagnosisin most cases, and moreover, preoperativetissue samples may fail to establish a diagnosis. Incases with endobronchial lesions, small samples orlack of transthoracic biopsy in central tumors withoutendobronchial lesions can make diagnosis difficult.The definitive diagnosis can only be made after largerexaminations with larger tissue samples taken afterthe operation. In addition, failure to differentiate benign-malignant in frozen examination may negativelyaffect the resection of the surgeon. It can cause incompleteor unnecessary resection.The aim of this study was to evaluate the clinical radiologicaland histopathological features of these tumors,which have been rarely reported in the literature,and to contribute to the diagnosis and treatment ofthese tumors.Material and MethodsThe study included 10 patients with rare malignantprimary lung tumor who were operated on in our clinicbetween 2010 and 2019. All patients were retrospectivelyevaluated in respect of age, gender, symptoms,preoperative imaging methods and invasive diagnosticmethods. Tumor localization, tumor size, type ofsurgical operation and survival were recorded.ResultsThe 10 patients included in the study comprised 6males and 4 females. Postoperative histopathologicaldiagnoses of the patients were reported as 2 carcinosarcomas,2 large cell carcinomas, 2 epithelioidhemangioendothelioma, 1 glomangiosarcoma, 1 primarypulmonary leiomyosarcoma, 1 mucoepidermoidcarcinoma, and 1 synovial sarcoma.ConclusionIt can be difficult to diagnose in rare primary malignantlung tumors by preoperative imaging and preoperativeinvasive diagnostic methods. CT-guided fine needlebiopsy and tru-cut biopsy, endobronchial biopsyand frozen samples performed before surgery may beinsufficient in diagnosis, which may mislead the surgeonabout lung resection. KW - Rare Primary Lung Tumors KW - Malignant KW - Glomangiosarcoma KW - Epiteloid Hemangioendothelioma N2 - AmaçAkciğerin en sık görülen primer malign tümörleriniyassı hücreli karsinom, küçük hücreli karsinom veadenokarsinom oluşturur. Ancak, nadir görülen bazımalign primer akciğer tümörleri de akciğeri etkileyebilir,tanı ve tedavide zorluklara neden olabilir. Gelenekselgörüntüleme yöntemleri olguların birçoğundatanıda yeterince yardımcı olmaz hatta preoperatifalınan doku örnekleri tanı koymada yetersiz kalabilir.Endobronşial lezyonu olan vakalarda örneklerin küçükolması veya endobronşial lezyon olmayan santraltümörlerde transtorasik biyopsi yapılamaması tanıyızorlaştırabilir. Kesin tanı ancak operasyon sonrasıalınan daha büyük doku örnekleri ile ayrıntılı incelemelersonunda konabilir. Ayrıca frozen incelemesindebenign-malign ayrımı yapılamaması cerrahın yapacağırezeksiyonu olumsuz yönde etkileyebilir. Eksik yada gereksiz rezeksiyona neden olabilir. Çalışmamızınamacı literatürde çok az bildirilen bu tümörlerin klinikradyolojik ve histopatolojik görünümlerini değerlendirerektanı ve tedavilerine katkıda bulunmaktır.Gereç ve Yöntem2010-2019 yılları arasında kliniğimizde opere edilenoldukça nadir 10 malign primer akciğer tümörü hastaçalışmaya dahil edildi. Tüm hastalar, yaş, cinsiyet,semptomlar, preoperatif görüntüleme yöntemleri veinvazif tanı yöntemleri ile retrospektif olarak incelendi.Tümör lokalizasyonu, tümör boyutları, yapılan cerrahioperasyon tipi ve yaşam süreleri kaydedildi.BulgularÇalışmamıza 10 hasta dahil edildi. Hastaların 6 tanesierkek, 4 tanesi kadındı. Yaş ortalamaları 53.4idi. 3 hastaya sol alt lobektomi, 2 hastaya sol pnömonektomi,3 hastaya wedge rezeksiyon, 1 hastayasol üst lobektomi, 1 hastaya orta lobektomi yapıldı.Hastaların postoperatif histopatolojik tanıları 2 hastadakarsinosarkom, 2 hastada büyük hücreli nöroendokrinkarsinom, 2 hastada epiteloid hemanjioendotelyoma,1 hastada glomanjiosarkom, 1 hastadaprimer pulmoner leiomyosarkom, 1 hastada mukoepidermoidkarsinom, 1 hastada sinovyal sarkom olarakraporlandı.SonuçAkciğerin nadir görülen primer malign tümörlerinepreoperatif görüntüleme ve invaziv yöntemler ile tanıkoymak zor olabilir. Ameliyat öncesi yapılan tomografieşliğinde ince iğne biyopsi, tru-cut biyopsi, bronkoskopikbiyopsi örnekleri ve frozen incelemeleri tanıkoymakta yetersiz kalabilir. Bu durum, operasyonuyapacak cerrahı yapılacak akciğer rezeksiyonu konusundayanlış yönlendirebilir. CR - 1. Fraser RS, Muller NL, Coleman N, Pare PD. Diagnosis of Diseases of the Chest, 4th edn. Philadelphia, PA: W B Saunders, 1999 CR - 2. Travis WD, Brambilla E, Müller-Hermelink HK, Harris CC, eds. World Health science Organization Classification of Tumours, Pathology and Genetics of Tumours of the Lung, Pleura, Thymus and Heart. IARC Pres, Lyon, 2004:12 CR - 3. Fasano M, Della Corte CM, Papaccio F, Ciardiello F, Morgillo F Pulmonary Large-Cell Neuroendocrine Carcinoma: From Epidemiology to Therapy.J Thorac Oncol. 2015 Aug;10(8):1133- 41. doi: 10.1097/JTO. CR - 4. Fernandez FG, Battafarano RJ. Large-cell neuroendocrine carcinoma of the lung: an aggressive neuroendocrine lung cancer. Semin Thorac Cardiovasc Surg 2006;18:206–210. CR - 5. Sánchezde Cos Escuín J. Diagnosis and treatment of neuroendocrinelung tumors. Arch Bronconeumol 2014;50:392–396. CR - 6. Battafarano RJ, Fernandez FG, Ritter J, Meyers BF, Guthrie TJ, Cooper JD et al. Large cell neuroendocrinecarcinoma: an aggressive form of non-small cell lung cancer. J ThoracCardiovasc Surg 2005;130:166–172. CR - 7. Odate S, Nakamura K, Onishi H, Kojima M, Uchiyama A, Nakano K et al. TrkB/BDNF signaling pathway is a potential therapeutic target for pulmonary large cell neuroendocrinecarcinoma. Lung Cancer 2013;79:205–214. CR - 8. Doğan C, Cömert SŞ, Çağlayan B, Salepçi B, Sağmen SB, Fidan A et al. A Rare Tumor of the Lung: Sarcomatoid Carcinoma South. Clin. Ist. Euras. 2017;28(2):135-138 CR - 9. Kim K, Flint JDA, Müller NL. Pulmonary carcinosarcoma: Radiologic and pathologic findings in three patients. AJR 1997; 169: 691 – 694 CR - 10. Travis WD, Brambilla E, Müller-Hermelink HC, Harris CC .Pathology and genetics. Tumours of the lung, plevra, thymus and heart. World Health science Organisation Classification of Tumours. IARC pres, Lyon, 2004, 53 – 58 CR - 11. Robinson PG, Shields TW. Uncommon primary malignant tumour of the lung. In.Shields TW, Lo Cicero III J,Pom Rb, Rusch VW (Eds). General thoracic surgery. 6.th edition, Lippincott Williams &Wilkins ,Philadelphia 2005,1810-12 CR - 12. Koss MN, Hochholzer L, Frommelt RA. Carcinosarcomas of the lung: a clinicopathologic study of 66 patients. Am J Surg Pathol. 1999 Dec;23(12):1514-26. CR - 13. S. W. Weiss, K. G. Ishak, D. H. Dail, D. E. Sweet, and F.M. Enzinger, “Epithelioid hemangioendothelioma and related lesions,” Semin Diagn Pathol, vol. 3, no. 4, pp. 259–287, 1986, http://www.ncbi.nlm.nih.gov/pubmed/3303234. CR - 14. Mesquita RD, Sousa M, Trinidad C, Pinto E, Badiola IA. New Insights about Pulmonary Epithelioid Hemangioendothelioma: Review of the Literature and Two Case Reports. Case Rep Radiol. 2017;2017:5972940. doi: 10.1155/2017/5972940. CR - 15. Carter EJ, Bradburne RM, Jhung JW, Ettensohn DB . Alveolar haemorrhage with epithelioid haemangioendothelioma. Am Rev Respir Dis 1990; 142: 700–701. CR - 16. Kradin RL, Mark EJ. Hemoptysis in a 20-year-old man with multiple pulmonary nodules. Massachusetts General Hospital Case Records, case 6-2000. N Engl J Med 2000; 342:572–578. CR - 17. Mata JM, Ca´ceres J, Prat J, Lo´pez JI, Velilla O. Intravascular bronchio-alveolar tumor: radio-graphic findings. Eur J Radiol 1991; 12:95–97. CR - 18. Buggage RR, Soudi N, Olson JL, C.T.(A.S.C.P.), C.T.(I.A.C.), Jean L et al. Epithelioid haemangioendothelioma of the lung. Diagn Cytopathol 1995; 13:54–60. CR - 19. K. Eguchi and M. Sawafuji, “Surgical management of a patient with bilateral multiple pulmonary epithelioid hemangioendothelioma: report of a case,” Surgery Today, vol. 45, no. 7, pp. 904–906, 2014. CR - 20. Y.Mizuno, H. Iwata, K. Shirahashi, Y.Hirose, and H. Takemura,“ Pulmonary epithelioid hemangioendothelioma,” General Thoracic and Cardiovascular Surgery, vol. 59, no. 4, pp. 297– 300,2011. CR - 21. Yousem SA, Hochholzer L. Mucoepidermoid tumors of the lung. Cancer 1987; 60:1346-52. CR - 22. Ishizumi T, Tateishi U, Watanabe S, Matsuno Y. Mucoepidermoid carcinoma of the lung: high-resolution CT and histopathologic findings in five cases. Lung Cancer 2008; 60:125-31. CR - 23. Li X, Yi W, Zeng Q. CT features and differential diagnosis of primary pulmonary mucoepidermoid carcinoma and pulmonary adenoid cystic carcinoma. J Thorac Dis. 2018 Dec;10(12):6501- 6508. doi: 10.21037/jtd.2018.11.71. CR - 24. Han X, Zhang J, Fan J, Cao Y, Gu J, Shi H. Radiological and Clinical Features and Outcomes of Patients with Primary Pulmonary Salivary Gland-Type Tumors. Can Respir J. 2019 Apr 1;2019:1475024. doi: 10.1155/2019/1475024. CR - 25. Zhou X, Zhang M, Yan X, Zhong Y, Li S, Liu J. Challenges in diagnosis of pulmonary mucoepidermoid carcinoma Medicine (Baltimore). 2019 Nov;98(44):e17684. doi: 10.1097/ MD.0000000000017684. CR - 26. Pozgain Z, Dulic G, Kristek J, Rajc J, Bogović S, Rimac M et al. Giant primary pleomorphic adenoma of the lung presenting as a post-traumatic pulmonary hematoma: a case report. J Thorac Cardiovasc Surg 2016;11:18. CR - 27. Roden AC, Garcia JJ, Wehrs RN, Colby TV, Khoor A, Leslie KO et al. Histopathologic, immunophenotypicand cytogenetic features of pulmonary mucoepidermoid carcinoma. Mod Pathol 2014;27:1479–88. CR - 28. Wang S, Ding C, Tu J Malignant glomus tumor of the lung with multiple metastasis: a rare case report. World J Surg Oncol. 2015 Feb 7;13:22. doi: 10.1186/s12957-014-0423-3. CR - 29. Abu-Zaid A, Azzam A, Amin T, Mohammed S. Malignant glomus tumor(glomangiosarcoma) of intestinal ileum: a rare case report. Case Reports Pathol. 2013;2013:305321. CR - 30. Milia M E, Turri L, Beldi D, Deantonio L, Pareschi R, Krengli M. Multidisciplinary approach in the treatment of malignant paraganglioma of the glomus vagale: a case report. Tumori. 2011;97:225–8. CR - 31. Attanoos R.L, Appleton M.A, A.R. Gibbs, Primary sarcomas of the lung: a clinicopathological and immunohistochemical study of 14 cases, Histopathology 29 (1996) 29–36. CR - 32. Frances R.L , J.A. Royo Prats. Pulmonary artery leiomyosarcoma diagnosed by magnetic resonance, PET-CT and EBUS-TBNA, Arch. Bronconeumol. 53 (2017) 522–523. CR - 33. J.S. Woo, O.L. Reddy, M. Koo, Y. Xiong, F. Li, H. Xu, Application of immunohistochemistry in the diagnosis of pulmonary and pleural neoplasms, Arch. Pathol. Lab Med. 141 (2017) 1195–1213. CR - 34. Suurmeijer et al. (2013) Synovial sarcoma. In: Fletcher DM et al.(eds) WHO classification of tumors of soft tissue and bone, 4thedn. IARC, Lyon CR - 35. Okamoto S, Hisaoka M, Daa T, Hatakeyama K, Iwamasa T,- Hashimoto HA et al. (2004) Primary pulmonary synovial sarcoma: a clinicopathologic, immunohistochemical, and molecular study of11 cases. Hum Pathol 35:850–856 CR - 36. Essary LR, Vargas SO, Fletcher CD (2002) Primary pleuropulmonary synovial sarcoma: reappraisal of a recently described anatomic subset. Cancer 94:459–469 CR - 37. Dennison S, Weppler E, Giacoppe G (2004) Primary pulmonary synovial sarcoma: a case report and review of current diagnostic and therapeutic standards. Oncologist 9:339–342 UR - https://doi.org/10.17343/sdutfd.753812 L1 - https://dergipark.org.tr/en/download/article-file/1154592 ER -