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Primary Pulmonary Sarcomas


Authors: T. Jakubcová;  P. Jakubec
Authors‘ workplace: Klinika plicních nemocí a tuberkulózy FN a LF UP, Olomouc
Published in: Klin Onkol 2009; 22(4): 139-153
Category: Reviews

Overview

Primary pulmonary sarcomas are rare diseases unlike lung carcinomas. The occurence of these sarcomas is between 0.013–0.40% of all malignant lung tumours. There are malignant mesenchymal tumours. They are flowing from the soft tissue of lung. The pulmonary sarcomas are heterogenic group with various biological behaviour. Their morfologic structure does not digger from the sarcomas of soft tissue. The primary pulmonary sarcomas occur more often in childhood and in young people unlike lung carcinomas. Radiation and some toxic substances are noted risk factors. Some gene mutations, infectious pathoghens and contraception have a possible impact on the origin of some types of the sarcomas. The current hypothesis is, that most of the sarcomas, if not all sarcomas, stem from primitive multipotent mesenchymal cell by malignant transformation in one or more lines. The diagnostic standard is biopsy from tumour with histologic and immunohistochemistry examination of a sample. The basic diagnostic problem is exclusion of a secondary origin of sarcomatic cells in the lung, because pulmonary metastasis of extrapulmonary sarcomas are more often than the primary pulmonary involvement. The optimal treatment is a resection of the tumour. The other therapeutic modalities are radiotherapy and chemotherapy, but results of these modalities are unsatisfactory. There are various chemotherapeutic regimes, monotherapy or combination regimes. The basic cytostatics are doxorubicine, iphosphamide, dacarbazine. Problems of the chemotherapy are high toxicity and relatively low curative effect about 20%. The first studies with biological treatment of the sarcomas of soft tissue have been published recently. This types of drugs could be a part of the complex management of these primary pulmonary tumours in the future. The primary pulmonary sarcomas have mostly aggresive course and often recur. Their prognosis is usually not very good. The survival median is 48 months and 5-years survival ranges between 38 and 48%. Prognostic factors are the size of tumour, histological type, grading, clinical stage and measure of a surgery major.

Key words:
primary pulmonary sarcoma – classification of diseases – disease detection – disease management


Sources

1. Zatloukal P, Petruželka L. Karcinoid plic. 1. vyd. Praha: Grada Publishing 2001.

2. Régnard JF, Icard P, Guibert L et al. Prognostic Factors and Results After Surgical Treatment of Primary Sarcomas of the Lung. Ann Thorac Surg 1999; 68: 227–231.

3. Porte HL, Metois DG, Leroy X et al. Surgical treatment of primary sarcoma of the lung. Eur J Cardiothorac Surg 2000; 18: 136–142.

4. Shidham VB. Benign and Malignant Soft Tissue Tumors. Available from http://www.emedicine.com/orthoped/TOPIC377.HTM. [updated 2006].

5. Gladish GW, Sabloff BM, Munden RF et al. Primary Thoracic Sarcomas. Radiographics 2002; 22: 621–637.

6. Krygier G, Amado A, Salisbury S et al. Primary lung liposarcoma. Lung Cancer 1997; 17: 271–275.

7. Said M, Migaw H, Hafsa C et al. Imaging features of primary pulmonary liposarcoma. Australasian Radiology 2003; 47: 313–317.

8. Sin HL, Shim JJ, Shin JS et al. Primary Endobronchial Leiomyosarcoma. Respiration 2001; 68: 99–102.

9. Dong Shang L, Ko-Huang L, Jang-Ming S et al. Primary Bronchopulmonary Leiomyosarcoma of the Left Main Bronchus in a Child Presenting With Wheezing and Atelectasis of the Left Lung. Pediatric Pulmonology 2002; 33: 318–321.

10. Wyatt JM, Matsubara O. Update in pulmonary vascular diseases. Pathology International 2004; 54: 469–489.

11. Yekeler E, Dursun M, Yildirim A et al. Diffuse pulmonary lymphangiomatosis: imaging findings. Diagn Intervent Radiology 2005; 11: 31–34.

12. Ozcan C, Celik A, Ural Z et al. Primary Pulmonary Rhabdomyosarcoma Arising Within Cystic Adenomatoid Malformation: A Case Report and Review of the Literature. J Pediatric Surg 2001; 36: 1062–1065.

13. Conquest HF, Thornton JL, Massie JR et al. Primary Pulmonary Rhabdomyosarcoma. Ann Surg 1965; 161: 688–692.

14. Shan ND, Diwanji SR. Primary chondrosarcoma of the lung with cutaneous and skeletal metastase. Singapure Med 2007; 48: 196–199.

15. Watanabe A, Ito M, Nomura F. Primary Chondrosarcoma of the Lung – A Case Report with Immunohistochemical Study. Jpn J Med 1990; 29: 616–619.

16. Yamazaki K, Okabayashi K, Hamatake D et al. Primary Osteosarcoma of the Lung: A Case Report. Ann Thorac Cardiovasc Surg 2006; 12: 126–128.

17. Kadowaki T, Hamada H, Yokoyama A. Two Cases of Primary Pulmonary Osteosarcoma. Internal Medicine 2005; 4: 632–637.

18. Sagawa M, Ueda Y, Matsubara F et al. Intrapulmonary Solitary Fibrous Tumor Diagnosed by Immunihistochemical and Genetic Approaches: Report of a Case. Surg Today 2007; 37: 423–425.

19. Kanamori Y, Hashizume K, Sugiyama M et al. Intrapulmonary Solitary Fibrous Tumor in an Eight-Year-Old Male. Pediatr Pulmonol 2005; 40: 261–264.

20. Sakurai H, Hasegawa T, Watanabe S et al. Inflammatory myofibroblastic tumor of the lung. Eur J Card Thorac Surg 2004; 25: 155–159.

21. Melloni G, Carretta A, Ciriaco P et al. Inflammatory Pseudotumor of the Lung in Adults. Ann Thorac Surg 2005; 79: 426–432.

22. Findik S, Erkan ML, Kandemir B. A Case report: Primary Malignant Fibrous Histiocytoma of the Lung. Turkish Respiratory Journal 2001; 2: 44–46.

23. Maeda J, Ohta M, Inoue M et al. Surgical Intervention for Malignant Fibrous Histiocytoma of the Lung: Report of a Case. Surg Today 2007; 37: 316–319.

24. Myung SS, Kang-Jey H. Primary Hemangiopericytoma of Lung: Radiography and Pathology. AJR 1979; 133: 1077–1083.

25. Marec-Bérard P. Malignant Hemangiopericytoma. Available from http://www.orpha.net/data/patho/GB/uk-HCP.pdf.

26. Kim L, Yoon YH, Choi SJ et al. Hyalinizing spindle cell tumor with giant rosettes arising in the lung: Report of a case with FUS-CREB3L2 fusion transcripts. Pathology International 2007; 5: 153–157.

27. Bejarano PA, Padhya TA, Smith R et al. Hyalinizing Spindle Cell Tumor With Giant Rosettes – A Soft Tissue Tumor With Mesenchymal and Neuroendocrine Features. Arch Pathol Labor Med 2000; 124: 1179–1184.

28. Logrono R, Filipowicz EA, Eyzaguirre EJ et al. Diagnosis of primary fibrosarcoma of the lung by fine-needle aspiration and core biopsy: A case report and review of the literature. Arch Pathol Lab Med 1999; 123: 731–735.

29. Savas C, Candir O, Ozguner F. Acute Respiratory Distress Due to Fibrosarcoma of the Carina in a Child. Pediatr Pulmonol 2004; 38: 355–357.

30. McGinnis M, Jacobs G, el-Naggar A et al. Congenital peribronchial myofibroblastic tumor (so-called „congenital leiomyosarcoma“). A distinct neonatal lung lesion associated with nonimmune hydrops fetalis. Mod Pathol 1993; 6: 487–492.

31. Horikoshi T, Kikuchi A, Matsumoto Y et al. Fetal hydrops associated with congenital pulmonary myofibroblastic tumor. J Obstet Gynaecol Res 2005; 31: 552–555.

32. Aboulafia DM. The Epidemiologic, Pathologic and Clinical Features of AIDS-Associated Pulmonary Kaposi’s Sarkoma. Chest 2000; 117: 1128–1145.

33. Snopková S, Chalupa P. Postižení plic u infekce HIV/AIDS. In: Ševčík P, Skřičková J, Šrámek V et al. Záněty plic v intenzivní medicíně. 1. vyd. Praha: Galén 2004.

34. Jakubec P, Jakubcová T, Hutyrová B et al. Epitelioidní hemangioendoteliom. Kazuistiky v alergologii, pneumologii a ORL 2007; 4: 7–14.

35. Atasoy C, Fitoz S, Yigit H et al. Radiographic, CT, and MRI findings in primary pulmonary angiosarcoma. J Clin Imaging 2001; 25: 337–340.

36. Kojima K, Okamoto I, Ushijima S et al. Successful Treatment of Primary Pulmonary Angiosarcoma. Chest 2003; 124: 2397–2400.

37. Colby TV, Koss MN, Travis WD. Tumors of the Lower Respiratory Tract (Atlas of Tumor Patology, series 3, fasc. 13). Washington: Armed Forces Institute of Patology 1995.

38. Matoo A, Fedullo PF, Kapelanski D et al. Pulmonary Artery Sarcoma. A Case Report of Surgical Cure and 5-Year Follow up. Chest 2002; 122: 745–747.

39. Bhatia K, Ellis S. Unusual lung tumours: an illustrated review of CT features suggestive of this diagnosis. Cancer Imaging 2006; 6: 72–82.

40. Uchida A, Tabata M, Kiura K et al. Successful Treatment of Pulmonary Artery Sarcoma by a Two-drug Combination Chemotherapy Consisting of Ifosfamide and Epirubicin. Jpn J Clin Oncol 2005; 35: 417–419.

41. Majer I, Haruštiak S, Janík P et al. Glomus trachey jako neobvyklý prípad dýchavice. Kazuistiky v alergologii, pneumologii a ORL 2007; 4: 4–6.

42. Hishida T, Hasegawa T, Asamura H et al. Malignant glomus tumor of the lung. Pathol Int 2003; 53: 632–636.

43. Chao BH. Intrathoracic Malignant Peripheral Nerve Sheath Tumor in Neurofibromatosis 1. J Clin Oncol 2008; 26: 2216–2218.

44. Tashiro T, Kawakita C, Takai C. Primary pulmonary malignant peripheral nerve sheath tumor: a case report. Acta Cytol 2007; 51: 820–824.

45. Hill DA, Sadeghi S, Schultz MZ et al. Pleuropulmonary Blastoma in an Adult. Cancer. 1999; 85: 2368–2374.

46. Abdelmohsen MH, Amr RS, Shereif LB. Pleuropulmonary blastoma. Available from http://www.ispub.com/journal/the_internet_journal_of_thoracic_and_cardiovascular_surgery.html. Version 6 [ 2004]. ISSN 1524–0724.

47. Niva H, Masuda S, Kobayashi C et al. Pulmonary synovial sarcoma with polypoid endobronchial growth: A case report, immunohistochemical and cytogenetic study. Patology International 2004; 54: 611–615.

48. Hosono T, Hironaka M, Kobayashi A et al. Primary Pulmonary Synovial Sarcoma Confirmed by Molecular Detection of SYT-SSX1 Fusion Gene Transcripts: a Case Report and Review of the Literature. Jpn J Clin Oncol 2005; 35: 274–279.

49. Yoon YL, Do HK, Ji HL et al. Primary Pulmonary Ewing’s Sarcoma/Primitive Neuroectodermal Tumor in a 67-year-old Man. J Korean Med Sci 2007; 22: 59–63.

50. Wang Z, Xiao W, Zheng S. Desmoplastic small round cell tumor of the lung: case report. Chin Med J 2007; 120: 2327–2328.

51. Stuart-Buttle CE, Smart CJ, Pritchard S. et al. Desmoplastic small round cell tumour: a review of literature and treatment options. Surg Oncol 2008; 17: 107–112.

52. Jiang M, Anderson T, Nwogu C et al. Pulmonary malignant granular cell tumor. World J Surg Oncol 2003; 1: 22–26.

53. Nakajima J, Morota T, Matsumoto J et al. Pulmonary Intimal Sarcoma Treated by a Left Pneumonectomy with Pulmonary Arterioplasty Under Cardiopulmonary Bypass: Report of a Case Surg Today 2007; 37: 496–499.

54. Deslée G, Guillou PJ, Baehrel B et al. Malignant mesenchymoma of the pleura. Interac Cardiovasc Thorac Surg 2003; 2: 376–378.

55. Adachi T, Oda Y, Sakamoto A. Prognostic factors in the so-called malignant mesenchymoma: a clinicopathological and immunohistochemical analysis. Oncol Rep 2003; 10: 803–811.

56. Mastroianni BE, Falchero L, Chalabreysse L et al. Primary sarcomas of the lung. A clinicopathologic study of 12 cases. Lung Cancer 2002; 38: 283–289.

57. Česká onkologická společnost ČLS JEP. Kapitola 22: Zhoubné nádory měkkých tkání. In: Zásady cytostatické léčby maligních onkologických onemocnění. 6. vyd. Brno: 2008. Dostupné z http://www.linkos.cz/info_praxe/standardy_htlm.php?t=26.

58. Maki RG, Wathen JK, Patel SR et al. Randomized phase II study of gemcitabine and docetaxel compared with gemcitabine alone in patients with metastatic soft tissue sarcomas: results of sarcoma aliance for research through collaboration study 002 /corrected/. J Clin Oncol 2007; 25: 2755–2763.

59. Le Cesne A, Domont J, Cioffi A. The new era of trabectedin in soft tissue sarcomas. EJHP Practice 2008; 14: 72–75.

60. Schoffski P, Dumez H, Wolter P et al. Clinical impact of trabectedin (ecteinascidin 743) in advanced/metastatic soft tissue sarcoma. Expert Opin Pharmacother 2008; 9: 1609–1618.

61. Ray-Coquard I, Blay JY. Adult soft tissue sarcomas. EJHP Practice 2008; 14: 68–70.

62. Petrov DB, Vlasov VI, Kalaydjiev GT et al. Primary pulmonary sarcomas and carcinosarcomas – postoperative results and comparative survival analysis. Eur J Cardiothorac Surg 2003; 23: 461–466.

63. Zatloukal P. Nádory plic. In: Zatloukal P, Fiala P, Votruba J et al. Vnitřní lékařství, díl IIIa Pneumologie. 1. vyd. Praha: Galén 2001.

64. Travis WD, Colby TV, Corrin B et al. In Collaboration with Sobin LH and Pathologists from 14 Countries. World Health Organization International Histological Classification of Tumours. Histological Typing of Lung and Pleural Tumours. 3rd ed. Berlin: Springer-Verlag 1999.

65. 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.

66. Kotilingam D, Lev DC, Lazar AJ et al. Staging Soft Tissue Sarcoma: Evolution and Change. CA Cancer J Clin 2006; 56(5): 282–291.

67. Fleming ID, Cooper JS, Henson DE et al (eds). AJCC Cancer Staging Manual. 5th ed. Philadelphia: Lippincott-Raven 1997.

68. Greene FL, Page DL, Fleming ID et al (eds). AJCC Cancer Staging Manual. 6th ed. New York: Springer 2002.

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