Primary pulmonary sarcomas – diagnosis, treatment, prognostic factors
Janusz Gołota 1  
,   Monika Rucińska 2  
,   Aleksandra Sejda 3  
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Department of Thoracic Surgery, Municipal Polyclinical Hospital in Olsztyn, Poland
Department of Oncology, School of Medicine, Collegium Medicum, University of Warmia and Mazury in Olsztyn, Poland
Department of Pathomorphology, Provincial Specialist Hospital in Olsztyn, Poland
Janusz Gołota   

Department of Thoracic Surgery, Municipal Polyclinical Hospital in Olsztyn, Niepodległości 44, 10-044 Olsztyn, Poland.
Submission date: 2018-04-21
Final revision date: 2018-09-17
Acceptance date: 2018-09-17
Online publication date: 2018-09-28
Publication date: 2019-11-01
Pol. Ann. Med. 2019;26(1):66–72
Primary pulmonary sarcomas (PPS) are rare types of non-epithelial malignant tumours of the lungs. PPS can originate from mesenchymal elements of bronchial wall, vessels or pulmonary stroma. Since the introduction of immunohistochemical and molecular diagnosis, a significant improvement in description and classification of sarcomas has been achieved.

The present report was aimed at describing the current state of knowledge concerning diagnosis and treatment of PPS.

Material and methods:
A literature review was conducted in context of PPS.

Results and discussion:
The majority of available literature concerning PPS is limited to reports based on single cases or small series. PPS not only are devoid of typical clinical symptoms, but also their pathomorphological diagnosis is difficult. Identification and differentiation of sarcomas is increasingly based on molecular diagnosis. The most efficient method of treatment is a radical surgical resection. Neoadjuvant and adjuvant therapy is applied according to the soft-tissue sarcoma guidelines, however, due to the rarity of PPS cases, it is impossible to assess its impact on overall survival.

Early diagnosis of PPS is difficult. Prognosis of PPS is poor, because surgery – the most efficient method of PPS treatment is not possible in many cases. Prognostic factors in PPS include the size of the tumour, histological grading of malignancy and clinical staging according to the lung cancer TNM.

The authors declare no conflict of interest.
The article is not funded.
Shields TW, LoCicero J III, Ponn RL, eds. General thoracic surgery. Philadelphia, PA: Lippincott Williams & Wilkins; 2000.
Dail DH, ed. Pulmonary pathology: Tumors. New York: Springer; 1995.
Suster S. Primary sarcomas of the lung. Semin Diagn Pathol. 1995;12(2):140–157.
Fletcher CD, Hogendoorn P, Mertens F, Bridge J. WHO classification of tumours of soft tissue and bone. 4th ed. Lyon, France: IARC Press; 2013.
Martini N, Hajdu SI, Beattie EJ. Primary sarcoma of the lung. J Thorac Cardiovascular Surg. 1971;61(1):33–38.
Régnard JF, Icard P, Guibert L, Thomas de Montpreville V, Magdalenat P, Levasseur P. Prognostic factors and results after surgical treatment of primary sarcomas of the lung. Ann Thorac Surg. 1999;68(1):227–231.
Bacha EA, Wright CD, Grillo HC, et al. Surgical treatment of pulmonary sarcomas. Eur J Cardiothorac Surg. 1999;15(4):456–460.
Porte HI, Metois DG, Leroy X, Conti M, Gosselin B, Wurtz A. Surgical treatment of primary sarcoma of the lung. Eur J Cardiothorac Surg. 2000;18(2):136–142.
Janssen JP, Mulder JJS, Wagenaar SS, Elbers HRJ, Van Den Bosch JMM. Primary sarcoma of the lung: a clinical study with long term follow-up. Ann Thorac Surg. 1994;58:1151–1155.
Lee JT, Shelburne JD, Linder J. Primary malignant fibrous histiocytoma of the lung. Cancer. 1984;53(5):1124–1130.<1124::AID-CNCR2820530518>3.0.CO;2-V.
Weiss S, Goldblum J, Folpe A, eds. Enzinger and Weiss’s Soft Tissue Tumors. Philadelphia, PA: Saunders Elsevier; 2013.
Panicek DM, Gatsonis C, Rosenthal DI, et al. CT and MR imaging in the local staging of primary malignant musculoskeletal neoplasms: report of the Radiology Diagnostic Oncology Group. Radiology. 1997;202(1):237–246.
Park JS, Chung JH, Jheon S, et al. EBUS-TBNA in the differential diagnosis of pulmonary artery sarcoma and thromboembolism. Eur Respir J. 2011;38(6):1480–1482.
Aga P, Singh R, Parihar A, Parashari U. Imaging spectrum in soft tissue sarcomas. Indian J Surg Oncol. 2011;2(4):271–279.
Knapp EL, Kransdorf MJ, Letson GD. Diagnostic Imaging Update: Soft Tissue Sarcomas. Cancer Control. 2005;12(1):22–26.
Völker T, Denecke T, Steffen I, et al. Positron emission tomography for staging of pediatric sarcoma patients: results of a prospective multicenter trial. J Clin Oncol. 2007;25(34):5435–5441.
Van der Woude HJ, Verstraete KL, Hogendoorn PCW, Taminiau AHM, Hermans J, Bloem JL. Musculoskeletal tumors: does fast dynamic contrast-enhanced subtraction MR imaging contribute to the characterization? Radiology. 1998;208(3):821–828.
Wang CK, Li CW, Hseih TJ, Chein SH, Liu GC, Tsai KB. Characterization of bone and soft tisuue tumours with in vivo 1H MR spectroscopy. Radiology. 2004;232(2):599–605.
Schulte M, Brecht-Krauss D, Heymer B, et al. Grading of tumors and tumorlike lesions of bone: evaluation by FDG PET. J Nucl Med. 2000;41(10):1695–1701.
Piperkova E, Mikhaeil M, Mousavi A, et al. Impact of PET and CT in PET/CT studies for staging and evaluating treatment response in bone and soft tissue sarcomas. Clin Nucl Med. 2009;34(3):146–150.
Franzius C, Sciuk J, Daldrup-Link HE, et al. FDG PET for detection of osseous metastases from malignant primary bone tumours: comparison with bone scintigraphy. Eur J Nucl Med. 2000;27(9):1305–1311.
Schuetze SM, Rubin BP, Vernon C, et al. Use of positron emission tomography in localized extremity soft tissue sarcoma treated with neoadjuvant chemotherapy. Cancer. 2005;103(2):339–348.
Johnson GR, Zhuang H, Khan J, et al. Roles of positron emission tomography with fluorine-18-deoxyglucose in the detection of local recurrent and distant metastatic sarcoma. Clin Nucl Med. 2003;28(10):815–820.
Eary JF, O’Sullivan F, O’Sullivan J, et al. Spatial heterogeneity in sarcoma 18F-FDG uptake as a predictor of patient outcome. J Nucl Med. 2008;49(12):1973–1979.
Quak E, van de Luijtgaarden AC, de Geus-Oei LF, et al. Clinical Applications of Positron Emission Tomography in Sarcoma Management. Expert Rev Anticancer Ther. 2011;11(2):195–204.
Caraway NP, Salina D, Deavers MT, et al. Pulmonary artery intimal sarcoma diagnosed using endobronchial ultrasound-guided transbronchial needle aspiration. Cytojournal. 2015;12(1):11–16.
Fletcher CDM, Unni KK, Mertens F, eds. World Health Organization Classification of Tumours, Pathology and Genetics of Tumours of Soft Tissue and Bone. Lyon: IARC Press; 2002.
Gibbs J, Henderson-Jackson E, Bui MM. Bone and soft tissue pathology. Diagnostic and prognostic implications. Surg Clin North Am. 2016;96(5):915–962.
Fisher C. Immunohistochemistry in diagnosis of soft tissue tumours. Histopathology. 2010;58(7):1001–1012.
Egas-Bejar D, Anderson PM, Agarwal R, et al. Theranostic profiling for actionable aberrations in advanced high risk osteosarcoma with aggressive biology reveals high molecular diversity: the human fingerprint hypothesis. Oncoscience. 2014;1(2):167–179.
Chibon F, Lagarde P, Salas S, et al. Validated prediction of clinical outcome in sarcomas and multiple types of cancer on the basis of a gene expression signature related to genome complexity. Nat Med. 2010;16(7):781–787.
Coindre JM, Chibon F. Grading sarcomas: histologic and molecular approaches. Diagn Histopathol. 2011;17(8):325–332.
Robinson DR, Wu YM, Kalyana-Sundaram S, et al. Identification of recurrent NAB2 STAT6 gene fusions in solitary fibrous tumor by integrative sequencing. Nat Genet. 2013;45(2):180–185.
Chmielecki J, Crago AM, Rosenberg M, et al. Whole-exome sequencing identifies a recurrent NAB2-STAT6 fusion in solitary fibrous tumors. Nat Genet. 2013;45(2):131–132.
Mohajeri A, Tayebwa J, Collin A, et al. Comprehensive genetic analysis identifies a pathognomonic NAB2/STAT6 fusion gene, nonrandom secondary genomic imbalances, and a characteristic gene expression profile in solitary fibrous tumor. Genes Chromosomes Cancer. 2013;52(10):873–886.
Dennison S, Weppler E, Giocoppe G. Primary pulmonary synovial sarcoma: a case report and review of current diagnostic and therapeutic standards. Oncologist. 2004;9(3):339–342.
Bridge JA, Cushman-Vokoun A. Molecular diagnostics of soft tissue tumors. Arch Pathol Lab Med. 2011;135(5):588–601.
Bridge JA. Advantages and limitations of cytogenetic, molecular cytogenetic, and molecular diagnostic testing in mesenchymal neoplasms. J Orthop Sci. 2008;13(3):273–282.
Dei Tos AP. A current perspective on the role for molecular studies in soft tissue tumor pathology. Semin Diagn Pathol. 2013;30(4):375–381.
Thway K, Wang J, Mubako T, et al. Histopathological diagnostic discrepancies in soft tissue tumours referred to a specialist centre: reassessment in the era of ancillary molecular diagnosis. Sarcoma. 2014;2014:686902. doi: 10.1155/2014/686902.
Rutkowski P, ed. [Soft tissue sarcomas]. Gdańsk: Via Medica; 2015 [in Polish].
Raut CP, George S, Hornick JL, et al. High rates of histopathologic discordance in sarcoma with implications for clinical care. J Clin Oncol. 2011;29(15 Suppl):10065.
Gan H, Zhang J, Feng L, et al. [Diagnosis and surgical treatment of pulmonary artery sarcoma]. Zhonghua Yi Xue Za Zhi. 2014;94(16):1252–1254 [in Chinese].
Wong HH, Gounaris I, McCormack A, et al. Presentation and management of pulmonary artery sarcoma. Clin Sarcoma Res. 2015;5:3.
Nascimento AG, Unni KK, Bernatz PE. Sarcomas of the lung. Mayo Clin Proc. 1982;57(6): 355–359.
Martini N, Vogt-Moykopf I, eds. Thoracic Surgery: Frontiers and Uncommon Neoplasms. St. Louis: Mosby; 1989.
Wu YC, Wang LS, Chen W, wet al. Primary pulmonary malignant hemangiopericytoma associated with coagulopathy. Ann Thorac Surg. 1997;64(3):841–843.
Tawbi HA, Burgess M, Bolejak V. Pembrolizumab in advanced soft-tissue sarcoma and bone sarcoma (SARC028): a multicentre, two-cohort, single-arm, open-label, phase 2 trial. Lancet Oncol. 2017;18(11):1493–1501.