open access

Vol 89, No 5 (2021)
Review paper
Submitted: 2021-04-05
Accepted: 2021-04-30
Published online: 2021-10-20
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Pulmonary blastoma: a comprehensive overview of a rare entity

Ioannis Tsamis1, Stavroula - Porfyria Chachali2, Georgia Gomatou1, Ioannis Trontzas1, Maria Mitsogianni1, Nikolaos Syrigos1, Ioannis Vamvakaris3, Elias Kotteas1
DOI: 10.5603/ARM.a2021.0085
·
Pubmed: 34725809
·
Adv Respir Med 2021;89(5):511-519.
Affiliations
  1. Oncology Unit, Third Department of Medicine, Sotiria General Hospital, National and Kapodistrian University of Athens, Messogion, Athens, Greece
  2. 4th Department of Internal Medicine, National and Kapodistrian University of Athens, Medical School, Athens, Greece
  3. Pathology Department, Sotiria General Hospital, Messogion, Athens, Greece

open access

Vol 89, No 5 (2021)
REVIEWS
Submitted: 2021-04-05
Accepted: 2021-04-30
Published online: 2021-10-20

Abstract

Introduction: Pulmonary blastoma is a rare malignancy, accounting for less than 0.5% of primary lung tumors. It belongs to the group of pulmonary sarcomatoid carcinomas, and it is typically characterized by a biphasic pattern of an epithelial and a mesenchymal component. Only a few hundred cases have been reported worldwide. The aim of this study is to review and critically assess the literature regarding pulmonary blastoma.
Material and methods: A narrative literature review of PubMed database from the inception of the database up to January 2021, limited to the English language, was conducted, using combinations of the following keywords: “pulmonary blastoma”, “biphasic pulmonary blastoma”, “sarcomatoid carcinoma”.
Results: Pulmonary blastoma is composed of an epithelial and a mesenchymal malignant component. Regarding pathogenesis, the origin of the biphasic cell population remains elusive. Characteristic immunohistochemical stains are supportive of diagnosis.Clinically, the symptomatology is non-specific, while 40% of the cases are asymptomatic. It is diagnosed at a younger agecompared to other types of lung cancer, and it is often non-metastatic at diagnosis allowing for surgical treatment. Data on management and survival are scarce and mainly come from isolated cases. Advances on targeted therapy may provide novel treatment options. Given the rarity of the cases, multicenter collaboration is needed in order to establish therapeutic guidelines.

Abstract

Introduction: Pulmonary blastoma is a rare malignancy, accounting for less than 0.5% of primary lung tumors. It belongs to the group of pulmonary sarcomatoid carcinomas, and it is typically characterized by a biphasic pattern of an epithelial and a mesenchymal component. Only a few hundred cases have been reported worldwide. The aim of this study is to review and critically assess the literature regarding pulmonary blastoma.
Material and methods: A narrative literature review of PubMed database from the inception of the database up to January 2021, limited to the English language, was conducted, using combinations of the following keywords: “pulmonary blastoma”, “biphasic pulmonary blastoma”, “sarcomatoid carcinoma”.
Results: Pulmonary blastoma is composed of an epithelial and a mesenchymal malignant component. Regarding pathogenesis, the origin of the biphasic cell population remains elusive. Characteristic immunohistochemical stains are supportive of diagnosis.Clinically, the symptomatology is non-specific, while 40% of the cases are asymptomatic. It is diagnosed at a younger agecompared to other types of lung cancer, and it is often non-metastatic at diagnosis allowing for surgical treatment. Data on management and survival are scarce and mainly come from isolated cases. Advances on targeted therapy may provide novel treatment options. Given the rarity of the cases, multicenter collaboration is needed in order to establish therapeutic guidelines.

Get Citation

Keywords

pulmonary blastoma; sarcomatoid lung carcinoma; biphasic pulmonary blastoma

About this article
Title

Pulmonary blastoma: a comprehensive overview of a rare entity

Journal

Advances in Respiratory Medicine

Issue

Vol 89, No 5 (2021)

Article type

Review paper

Pages

511-519

Published online

2021-10-20

DOI

10.5603/ARM.a2021.0085

Pubmed

34725809

Bibliographic record

Adv Respir Med 2021;89(5):511-519.

Keywords

pulmonary blastoma
sarcomatoid lung carcinoma
biphasic pulmonary blastoma

Authors

Ioannis Tsamis
Stavroula - Porfyria Chachali
Georgia Gomatou
Ioannis Trontzas
Maria Mitsogianni
Nikolaos Syrigos
Ioannis Vamvakaris
Elias Kotteas

References (75)
  1. Pelosi G, Sonzogni A, De Pas T, et al. Review article: pulmonary sarcomatoid carcinomas: a practical overview. Int J Surg Pathol. 2010; 18(2): 103–120.
  2. Koss M, Hochholzer L, O'Leary T. Pulmonary blastomas. Cancer. 1991; 67(9): 2368–2381, doi: 10.1002/1097-0142(19910501)67:9<2368::aid-cncr2820670926>3.0.co;2-g.
  3. Knight S, Knight T, Khan A, et al. Current management of pleuropulmonary blastoma: A surgical perspective. Children (Basel). 2019; 6(8).
  4. Brambilla E, Travis WD, Colby TV, et al. The new World Health Organization classification of lung tumours. Eur Respir J. 2001; 18(6): 1059–1068.
  5. Baldovini C, Rossi G, Ciarrocchi A. Approaches to tumor classification in pulmonary sarcomatoid carcinoma. Lung Cancer (Auckl). 2019; 10: 131–149.
  6. Travis WD, Brambilla E, Nicholson AG, et al. WHO Panel. The 2015 World Health Organization classification of lung tumors: impact of genetic, clinical and radiologic advances since the 2004 classification. J Thorac Oncol. 2015; 10(9): 1243–1260.
  7. Zhao YY, Liu L, Zhou T, et al. A retrospective analysis of the clinicopathological and molecular characteristics of pulmonary blastoma. Onco Targets Ther. 2016; 9: 6915–6920.
  8. Brodowska-Kania D, Kotwica E, Paturej A, et al. What do we know about pulmonary blastoma?: review of literature and clinical case report. Nagoya J Med Sci. 2016; 78(4): 507–516.
  9. Bodner SM, Koss MN. Mutations in the p53 gene in pulmonary blastomas: immunohistochemical and molecular studies. Hum Pathol. 1996; 27(11): 1117–1123.
  10. Holst VA, Finkelstein S, Colby TV, et al. p53 and K-ras mutational genotyping in pulmonary carcinosarcoma, spindle cell carcinoma, and pulmonary blastoma: implications for histogenesis. Am J Surg Pathol. 1997; 21(7): 801–811.
  11. Sekine S, Shibata T, Matsuno Y, et al. Beta-catenin mutations in pulmonary blastomas: association with morule formation. J Pathol. 2003; 200(2): 214–221.
  12. Macher-Goeppinger S, Penzel R, Roth W, et al. Expression and mutation analysis of EGFR, c-KIT, and β-catenin in pulmonary blastoma. J Clin Pathol. 2011; 64(4): 349–353.
  13. Thompson L, Chang B, Barsky SH. Monoclonal origins of malignant mixed tumors (carcinosarcomas). Evidence for a divergent histogenesis. Am J Surg Pathol. 1996; 20(3): 277–285.
  14. Hansen T, Bittinger F, Kortsik C, et al. Expression of KIT (CD117) in biphasic pulmonary blastoma. Novel data on histogenesis. Lung. 2003; 181(4): 193–200.
  15. Takahashi K, Kohno T, Matsumoto S, et al. Clonality and heterogeneity of pulmonary blastoma from the viewpoint of genetic alterations: a case report. Lung Cancer. 2007; 57(1): 103–108.
  16. Nemeh F, Kuo AH, Ross J, et al. The radiologic and pathologic diagnosis of biphasic pulmonary blastoma. J Radiol Case Rep. 2017; 11(9): 10–21.
  17. Yousem SA, Wick MR, Randhawa P, et al. Pulmonary blastoma. An immunohistochemical analysis with comparison with fetal lung in its pseudoglandular stage. Am J Clin Pathol. 1990; 93(2): 167–175.
  18. Larsen H, Sørensen JB. Pulmonary blastoma: a review with special emphasis on prognosis and treatment. Cancer Treat Rev. 1996; 22(3): 145–160.
  19. Archie PH, Beasley MB, Ross HJ. Biphasic pulmonary blastoma with germ cell differentiation in a 36-year-old man. J Thorac Oncol. 2008; 3(10): 1185–1187.
  20. Jenkins TM, Morrissette JJD, Kucharczuk JC, et al. ROS1 rearrangement in a case of classic biphasic pulmonary blastoma. Int J Surg Pathol. 2018; 26(4): 360–363.
  21. Nakatani Y, Miyagi Y, Takemura T, et al. Aberrant nuclear/cytoplasmic localization and gene mutation of beta-catenin in classic pulmonary blastoma: beta-catenin immunostaining is useful for distinguishing between classic pulmonary blastoma and a blastomatoid variant of carcinosarcoma. Am J Surg Pathol. 2004; 28(7): 921–927.
  22. Dixit R, Joshi N, Dave L. Biphasic pulmonary blastoma: An unusual presentation with chest wall, rib, and pleural involvement. Lung India. 2014; 31(1): 87–89.
  23. Vassilopoulos PP, Vrettou V, Smerniotis V, et al. Pulmonary blastoma presenting with massive hemothorax. Chest. 1992; 102(2): 649–650.
  24. Ondo K, Ishida T, Yamazaki K, et al. Pulmonary blastoma in an adult. A case with rapid progression. Scand Cardiovasc J. 1998; 32(4): 247–249.
  25. Shadrach BJ, Vedant D, Vishwajeet V, et al. Endobronchial pulmonary blastoma - an unusual presentation of a rare lung malignancy and review of literature. Monaldi Arch Chest Dis. 2020; 90(3).
  26. Luo Z, Cao C, Xu N, et al. Classic biphasic pulmonary blastoma: a case report and review of the literature. J Int Med Res. 2020; 48(10): 300060520962394.
  27. Kim K, Gupta S, Gupta S, et al. Incidental early diagnosis of biphasic pulmonary blastoma in a patient with history of stage IV lung adenocarcinoma. Thorac Cancer. 2020; 11(10): 3029–3033.
  28. Liu Yi, Luo D, Du T, et al. Clinical and pathology analysis of 1 case of adult pleural pulmonary blastoma: A case report. Medicine (Baltimore). 2017; 96(50): e8918.
  29. Van Loo S, Boeykens E, Stappaerts I, et al. Classic biphasic pulmonary blastoma: a case report and review of the literature. Lung Cancer. 2011; 73(2): 127–132.
  30. Yang M, Li B, Zhang C, et al. Classical biphasic pulmonary blastoma in a young woman: case report and review of literature. International journal of clinical and experimental pathology. 2019; 12(12): 4400–4404.
  31. Iwata T, Nishiyama N, Inoue K, et al. Biphasic pulmonary blastoma: report of a case. Ann Thorac Cardiovasc Surg. 2007; 13(1): 40–43.
  32. Robert J, Pache JC, Seium Y, et al. Pulmonary blastoma: report of five cases and identification of clinical features suggestive of the disease. Eur J Cardiothorac Surg. 2002; 22(5): 708–711.
  33. Vila R, McCoy JJ, McCall RE. Pulmonary blastoma, report of a case. Journal of the South Carolina Medical Association. 1973; 69(7): 251–6.
  34. Kennedy A, Prior AL. Pulmonary blastoma: a report of two cases and a review of the literature. Thorax. 1976; 31(6): 776–781.
  35. Meinecke R, Bauer F, Skouras J, et al. Blastomatous tumors of the respiratory tract. Cancer. 1976; 38(2): 818–823, doi: 10.1002/1097-0142(197608)38:2<818::aid-cncr2820380225>3.0.co;2-1.
  36. Kern WH, Stiles QR. Pulmonary blastoma. J Thorac Cardiovasc Surg. 1976; 72(5): 801–808.
  37. Fung C, Lo J, Yonan T, et al. Pulmonary blastoma.An ultrastructural study with a brief review of literature and a discussion of pathogenesis. Cancer. 1977; 39(1): 153–163, doi: 10.1002/1097-0142(197701)39:1<153::aid-cncr2820390126>3.0.co;2-#.
  38. Roth JA, Elguezabal A. Pulmonary blastoma evolving into carcinosarcoma. A case study. Am J Surg Pathol. 1978; 2(4): 407–413.
  39. Jacobsen M, Francis D. Pulmonary Blastoma. Acta Pathologica Microbiologica Scandinavica Section A Pathology. 2009; 88A(1-6): 151–160.
  40. Medbery CA, Bibro MC, Phares JC, et al. Pulmonary blastoma. Case report and literature review of chemotherapy experience. Cancer. 1984; 53(11): 2413–2416, doi: 10.1002/1097-0142(19840601)53:11<2413::aid-cncr2820531108>3.0.co;2-e.
  41. Dienemann D, Hartmann CA, Minck C. Pulmonary blastomas. Immunohistochemical investigations of three cases. Pathol Res Pract. 1989; 184(3): 306–311.
  42. Kliem V, Bügge M, Leimenstoll K, et al. Pulmonary blastoma--a rare tumour. Clin Investig. 1992; 70(10): 927–931.
  43. Ohara N, Tominaga O, Oka T, et al. Pulmonary blastoma: report of a case. Surg Today. 1999; 29(4): 385–388.
  44. Asimakopoulos G, Krausz T, Smith PL. Radical resection of a pulmonary blastoma involving the mediastinum. Thorac Cardiovasc Surg. 1999; 47(3): 197–199.
  45. Walker RI, Suvarna K, Matthews S. Case report: pulmonary blastoma: presentation of two atypical cases and review of the literature. Br J Radiol. 2005; 78(929): 437–440.
  46. Kawano R, Hata E, Ikeda S, et al. Pulmonary blastoma. Jpn J Thorac Cardiovasc Surg. 2005; 53(11): 611–614.
  47. Liman ST, Altinok T, Topcu S, et al. Survival of biphasic pulmonary blastoma. Respir Med. 2006; 100(7): 1174–1179.
  48. Kouvaris JR, Gogou PV, Papacharalampous XN, et al. Solitary brain metastasis from classic biphasic pulmonary blastoma: a case report and review of the literature. Onkologie. 2006; 29(12): 568–570.
  49. Oshika Y, Matsukuma S, Hashimoto H, et al. Biphagic pulmonary blastoma with a lesion of yolk sac tumor. Gen Thorac Cardiovasc Surg. 2007; 55(6): 243–247.
  50. Mulamalla K, Truskinovsky AM, Dudek AZ. Pulmonary blastoma with renal metastasis responds to sorafenib. J Thorac Oncol. 2007; 2(4): 344–347.
  51. Maeda R, Isowa N, Onuma H, et al. Biphasic pulmonary blastoma with rapid progression. Gen Thorac Cardiovasc Surg. 2009; 57(2): 104–107.
  52. Lindet C, Vanhuyse M, Thebaud E, et al. Pulmonary blastoma in adult: dramatic but transient response to doxorubicin plus ifosfamide. Acta Oncol. 2011; 50(1): 156–157.
  53. Nakayama T, Ohtsuka T, Kazama A, et al. Classic pulmonary blastoma: a subtype of biphasic pulmonary blastoma. Ann Thorac Cardiovasc Surg. 2012; 18(2): 125–127.
  54. Sharma A, O'Gorman K, Aman C, et al. A rare occurrence of biphasic pulmonary blastoma in an elderly male. Anticancer research. 2013; 33(9): 3911–3915.
  55. Smyth RJ, Fabre A, Dodd JD, et al. Pulmonary blastoma: a case report and review of the literature. BMC Res Notes. 2014; 7: 294.
  56. Kawasaki K, Yamamoto K, Suzuki Y, et al. Surgery and radiation therapy for brain metastases from classic biphasic pulmonary blastoma. BMJ Case Rep. 2014; 2014.
  57. Muthu P, Unnikrishnan A, Jose WM, et al. A case of biphasic pulmonary blastoma showing good response to preoperative chemotherapy. Indian J Cancer. 2014; 51(4): 510–511.
  58. Sakata S, Saeki S, Hirooka S, et al. A case of biphasic pulmonary blastoma treated with carboplatin and paclitaxel plus bevacizumab. Case Rep Oncol Med. 2015; 2015: 842621.
  59. Bosch-Barrera J, Holguin F, Baldó X, et al. Neoadjuvant Chemoradiotherapy Treatment for a Classic Biphasic Pulmonary Blastoma with High PD-L1 Expression. Anticancer Res. 2015; 35(9): 4871–4875.
  60. Bu X, Liu J, Wei L, et al. Epidemiological features and survival outcomes in patients with malignant pulmonary blastoma: a US population-based analysis. BMC Cancer. 2020; 20(1): 811.
  61. Wang YX, Zhang J, Chu XY, et al. Diagnosis and multi-modality treatment of adult pulmonary plastoma: Analysis of 18 cases and review of literature. Asian Pac J Trop Med. 2014; 7(2): 164–168.
  62. Lewis JA, Petty WJ, Urbanic J, et al. Cure of oligometastatic classic biphasic pulmonary blastoma using aggressive tri-modality treatment: case series and review of the literature. Cureus. 2018; 10(11): e3586.
  63. Cutler CS, Michel RP, Yassa M, et al. Pulmonary blastoma: case report of a patient with a 7-year remission and review of chemotherapy experience in the world literature. Cancer. 1998; 82(3): 462–467, doi: 10.1002/(sici)1097-0142(19980201)82:3<462::aid-cncr6>3.0.co;2-r.
  64. Zagar TM, Blackwell S, Crawford J, et al. Preoperative radiation therapy and chemotherapy for pulmonary blastoma: a case report. J Thorac Oncol. 2010; 5(2): 282–283.
  65. Teixeira A, Vieira C, Sousa N, et al. [Biphasic pulmonary blastoma with germ cell differentiation: a challenge in diagnosis and treatment]. Acta Med Port. 2011; 24 Suppl 3: 685–688.
  66. Meng Z, Chen P, Zang F, et al. A patient with classic biphasic pulmonary blastoma harboring CD74-ROS1 fusion responds to crizotinib. Onco Targets Ther. 2018; 11: 157–161.
  67. Bini A, Ansaloni L, Grani G, et al. Pulmonary blastoma: report of two cases. Surg Today. 2001; 31(5): 438–442.
  68. Zaidi A, Zamvar V, Macbeth F, et al. Pulmonary blastoma: medium-term results from a regional center. Ann Thorac Surg. 2002; 73(5): 1572–1575.
  69. He W, Jiang G, Xie B, et al. Radical resection of a pulmonary blastoma involving the pulmonary artery. Eur J Cardiothorac Surg. 2008; 34(3): 695–696.
  70. Yu L, Li X, Yang W. Pulmonary blastoma metastatic to the ovary. Int J Gynecol Pathol. 2009; 28(1): 59–62.
  71. Schwitter M, Potocnik P, von Moos R, et al. Dyspnoea and a lung mass in a young female 2 weeks after Caesarean delivery. Eur Respir J. 2011; 38(2): 465–467.
  72. Gallo K, Brickman A, Warren WH, et al. Unresectable middle mediastinal biphasic pulmonary blastoma. Anticancer Res. 2015; 35(11): 6325–6327.
  73. Kilic D, Yilmaz C, Tepeoglu M, et al. Biphasic pulmonary blastoma associated with cerebral metastasis. Turk Neurosurg. 2016; 26(1): 169–172.
  74. Le Caer H, Teissier E, Barriere JR, et al. Classic biphasic pulmonary blastoma: A case report and review of the literature. Crit Rev Oncol Hematol. 2018; 125: 48–50.
  75. Vossler JD, Abdul-Ghani A. Pulmonary blastoma in an adult presenting with hemoptysis and hemothorax. Ann Thorac Surg. 2019; 107(5): e345–e347.

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