Sarcomatoid carcinoma of lung


Poorly differentiated non-small cell carcinoma with a sarcoma or sarcoma-like component0,9. Loss of heterozygocity studies indicate that both epithelial and mesenchymal components are derived from the same clone12.


Up to 1.3% of all lung carcinomas are of sarcomatoid type. It is four times more common in men than in women1,10,13,14,16,19,21,22. There is a strong association with smoking10,13,21,22.



Expression of epithelial markers by the spindle or giant cell component is not a requirement, provided that an overtly epithelial component is present. If negative for cytokeratins, differentiation of pure spindle cell carcinoma from sarcoma is problematic. There is often co-expression of cytokeratins, vimentin, CEA and SMA.



Variable4, reduced compared to better differentiated carcinomas5, 37/3719, 18/2120, positive28












Reduced compared to better differentiated carcinomas5, positive28 , 13/1930

MAb A80












Often strong paranuclear in giant cell carcinoma5, 9/1011



beta-human chorionic gonadotrophin

May be positive in giant cell carcinoma6. Positive in 21% of squamous carcinomas, 60% of adenocarcinomas and 93% of large cell carcinomas, showing no association with tumour giant cells8.


Positive in 28% of squamous carcinomas, 10% of adenocarcinomas and 56% of large cell carcinomas, showing no association with tumour giant cells8.


pregnancy-specific beta-1 glycoprotein

Positive in 64% of squamous carcinomas, 80% of adenocarcinomas and 93% of large cell carcinomas, showing no association with tumour giant cells8.


Collagen IV

positive in the sarcomatous component26.



The immunoreactivity of the sarcomatous component is often determined by the nature of the coexistent epithelial component:

specific components present are shown in brackets








pure spindle cell carcinoma





pure giant cell carcinoma


2/31, 0/329


2/31, 2/329

pleomorphic carcinoma (giant cell carcinoma/spindle cell carcinoma)


5/71, 1/329


5/71, 1/329

Pleomorphic carcinoma (adenocarcinoma)

12/141, 3/429

11/141, 1/429

14/141, 3/429

12/141, 2/429

Pleomorphic carcinoma (squamous cell carcinoma)

0/121, 0/329

0/121, 0/229


2/121, 1/329

Pleomorphic carcinoma (large cell carcinoma)

13/181, 2/829

7/181, 0/629

14/181, 6/829

13/181, 6/829

Pleomorphic carcinoma(adenocarcinoma/large cell carcinoma)





Pleomorphic carcinoma(squamous cell carcinoma/adenocarcinoma)










Pulmonary blastoma

1/11, 1/13

0/11, 0/13









CK20 was negative in all components in all the above tumours. Surfactant protein A showed a low sensitivity (39% for epithelial component, 6% for sarcomatoid component).

Carcinosarcoma: the epithelial component is positive for cytokeratins; any chondrosarcomatous component is positive for S-100 and any rhabdomyosarcomatous component is positive for muscle markers.

Pulmonary blastoma: the epithelial component is positive for cytokeratins, EMA and CEA, variably for neuroendocrine markers and specific hormones. Staining for alpha-fetoprotein is rare. The stromal component is positive for vimentin and muscle-specific actin. Any striated muscle is positive for desmin and any cartilage for S-100. Although usually segregated, sometimes the epithelium is positive for vimentin and the stroma for cytokeratins.

The correlation in the level of p53 expression in the epithelial and sarcomatoid components of biphasic tumours argues that the two components share a common pathway of tumourogenesis2.


There may be dense paranuclear aggregation of intermediate filaments in giant cell carcinoma5. Spindle cell squamous carcinomas show intercellular junctions and thick bundles of tonofilaments25.

Differential diagnosis


These are generally considered highly aggressive tumours13,14,16,17,21,23,26, which may metastasis to unusual sites10. Others have found no difference in prognosis from non-sarcomatoid carcinomas19. There may be a higher rate of gastrointestinal tract involvement than for other non-small cell carcinomas15. Giant cells constituting less than 10% of the tumour may be found in squamous cell carcinomas and adenocarcinomas and do not worsen the prognosis6.


0Tumours of the Lung, Pleura, Thymus and Heart. WHO Classification of Tumours. IARC Press 2004.

1 Rossi, G., Cavazza, A., Sturm, N., Migaldi, M., Facciolongo, N., Longo, L., Maiorana, A. and Brambilla, E. Pulmonary carcinomas with pleomorphic, sarcomatoid, or sarcomatous elements: a clinicopathologic and immunohistochemical study of 75 cases. Am J Surg Pathol 2003;27:311-24.

2 Ansari-Lari, M. A., M. O. Hoque, et al. (2002). "Immunohistochemical p53 expression patterns in sarcomatoid carcinomas of the upper respiratory tract." Am J Surg Pathol 26(8): 1024-31.

3 Garcia-Escudero, A., R. Gonzalez-Campora, et al. (2004). "Thyroid transcription factor-1 expression in pulmonary blastoma." Histopathology 44(5): 507-8.

4 Addis BJ,Corrin B Pulmonary blastoma, carcinosarcoma and spindle-cell carcinoma: an immunohistochemical study of keratin intermediate filaments. J Pathol 1985; 147:291-301

5 Addis BJ, Dewar A,Thurlow NP Giant cell carcinoma of the lung--immunohistochemical and ultrastructural evidence of dedifferentiation. J Pathol 1988; 155:231-40

6 Attanoos RL, Papagiannis A, Suttinont P, et al. Pulmonary giant cell carcinoma: pathological entity or morphological phenotype? Histopathology 1998; 32:225-31

7 Bocklage TJ, Dail D,Colby TV Primary lung tumors infiltrated by osteoclast-like giant cells. Ann Diagn Pathol 1998; 2:229-40

8 Boucher LD,Yoneda K The expression of trophoblastic cell markers by lung carcinomas. Hum Pathol 1995; 26:1201-6

9 Brambilla E, Travis WD, Colby TV, et al. The new World Health Organization classification of lung tumours. Eur Respir J 2001; 18:1059-68

10 Chang YL, Lee YC, Shih JY, et al. Pulmonary pleomorphic (spindle) cell carcinoma: peculiar clinicopathologic manifestations different from ordinary non-small cell carcinoma. Lung Cancer 2001; 34:91-7

11 Chejfec G, Candel A, Jansson DS, et al. Immunohistochemical features of giant cell carcinoma of the lung: patterns of expression of cytokeratins, vimentin, and the mucinous glycoprotein recognized by monoclonal antibody A-80. Ultrastruct Pathol 1991; 15:131-8

12 Dacic S, Finkelstein SD, Sasatomi E, et al. Molecular pathogenesis of pulmonary carcinosarcoma as determined by microdissection-based allelotyping. Am J Surg Pathol 2002; 26:510-6

13 Davis MP, Eagan RT, Weiland LH, et al. Carcinosarcoma of the lung: Mayo Clinic experience and response to chemotherapy. Mayo Clin Proc 1984; 59:598-603

14 Fishback NF, Travis WD, Moran CA, et al. Pleomorphic (spindle/giant cell) carcinoma of the lung. A clinicopathologic correlation of 78 cases. Cancer 1994; 73:2936-45

15 Ginsberg SS, Buzaid AC, Stern H, et al. Giant cell carcinoma of the lung. Cancer 1992; 70:606-10

16 Koss MN, Hochholzer L,Frommelt RA Carcinosarcomas of the lung: a clinicopathologic study of 66 patients. Am J Surg Pathol 1999; 23:1514-26

17 Koss MN, Hochholzer L,O'Leary T Pulmonary blastomas. Cancer 1991; 67:2368-81

18 Matsui K,Kitagawa M Spindle cell carcinoma of the lung. A clinicopathologic study of three cases. Cancer 1991; 67:2361-7

19 Nakajima M, Kasai T, Hashimoto H, et al. Sarcomatoid carcinoma of the lung: a clinicopathologic study of 37 cases. Cancer 1999; 86:608-16

20 Nappi O, Glasner SD, Swanson PE, et al. Biphasic and monophasic sarcomatoid carcinomas of the lung. A reappraisal of 'carcinosarcomas' and 'spindle-cell carcinomas'. Am J Clin Pathol 1994; 102:331-40

21 Nappi O,Wick MR Sarcomatoid neoplasms of the respiratory tract. Semin Diagn Pathol 1993; 10:137-47

22 Ro JY, Chen JL, Lee JS, et al. Sarcomatoid carcinoma of the lung. Immunohistochemical and ultrastructural studies of 14 cases. Cancer 1992; 69:376-86

23 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; 22:708-11

24 Siegel RJ, Bueso-Ramos C, Cohen C, et al. Pulmonary blastoma with germ cell (yolk sac) differentiation: report of two cases. Mod Pathol 1991; 4:566-70

25 Suster S, Huszar M,Herczeg E Spindle cell squamous carcinoma of the lung. Immunocytochemical and ultrastructural study of a case. Histopathology 1987; 11:871-8

26 Wick MR, Ritter JH,Humphrey PA Sarcomatoid carcinomas of the lung: a clinicopathologic review. Am J Clin Pathol 1997; 108:40-53

27 Wick MR, Ritter JH,Nappi O. Inflammatory sarcomatoid carcinoma of the lung: report of three cases and clinicopathologic comparison with inflammatory pseudotumors in adult patients. Hum Pathol 1995; 26:1014-21

28 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; 93:167-75

29 Yoo SH, Han J, Kim TJ, et al. Expression of CD99 in pleomorphic carcinomas of the lung. J Korean Med Sci 2005; 20:50-55 FULL TEXT

30 Lewis JS, Ritter JH,El-Mofty S Alternative epithelial markers in sarcomatoid carcinomas of the head and neck, lung, and bladder-p63, MOC-31, and TTF-1. Mod Pathol 2005; 18:1471-81 FULL TEXT

This page last revised 18.4.2005.


©SMUHT/PW Bishop