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CASE REPORT
Year : 2021  |  Volume : 38  |  Issue : 3  |  Page : 266-268  

Sarcomatoid carcinoma of the lung


1 Department of Pulmonary Medicine, All India Institute of Medical Sciences, Patna, Bihar, India
2 Department of Respiratory Medicine, Government Medical College, Srinagar, Jammu & Kashmir, India

Date of Submission01-Feb-2020
Date of Acceptance30-Mar-2020
Date of Web Publication30-Apr-2021

Correspondence Address:
Dr. Saurabh Karmakar
Room No. 330, 3rd Floor, Pulmonary Medicine OPD, New OPD Building, All India Institute of Medical Sciences, Phulwari Sharif, Patna - 801 505, Bihar
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/lungindia.lungindia_67_20

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   Abstract 


Sarcomatoid carcinoma (SC) of the lung is a rare histological subtype of nonsmall cell lung cancer and comprises a diagnostically and therapeutically challenging group of tumors. We report a patient of SC of the lung in an elderly patient who presented with a lung mass. Computed tomography-guided biopsy of the lung mass was done, which clinched the diagnosis. We present this case because of the rarity of this histological subtype and to expand understanding regarding this rare cancer.

Keywords: Carcinoma, lung, sarcoma


How to cite this article:
Karmakar S, Ansari MH, Thakur S, Rai DK. Sarcomatoid carcinoma of the lung. Lung India 2021;38:266-8

How to cite this URL:
Karmakar S, Ansari MH, Thakur S, Rai DK. Sarcomatoid carcinoma of the lung. Lung India [serial online] 2021 [cited 2021 May 8];38:266-8. Available from: https://www.lungindia.com/text.asp?2021/38/3/266/315305




   Introduction Top


Pulmonary sarcomatoid carcinoma (PSC) is defined as poorly differentiated nonsmall cell lung carcinoma (NSCLC) that contains a component of sarcoma or sarcoma-like elements (at least 10% spindle and/or giant cells) or a carcinoma consisting only of spindle and giant cells.[1] Sarcomatoid carcinomas (SCs) of the lung pose a significant challenge due to their rare occurrence, heterogeneous histology, and unclear histogenesis. Since pathological sub-classifications are also important for treatment decisions in lung cancer, clinicians and pathologists should be aware of the rarer tumors.


   Case Report Top


A 75-year-old male, a retired teacher and ex-smoker and a known case of chronic obstructive pulmonary disease for the past 20 years, presented with chief complaints of progressively increasing nonproductive cough and dyspnea for 8 months, loss of weight for 4 months, chest pain for 2 months, and hemoptysis for 1 month. On systemic examination, the patient was hemodynamically stable, had pallor and respiratory failure with a SpO2 of 91% on room air. On local examination, breath sounds were diminished in the left infrascapular, mammary, and infraaxillary fields and rhonchi were present.

Chest X-ray posteroanterior [Figure 1] revealed emphysematous lung fields with flattening of the diaphragm and a homogeneous, irregular margined opacity in the left upper and middle zones suggestive of the mass lesion. Due to the derangement in kidney function test a noncontrast, high resolution computed tomography of the thorax [Figure 2] was done and showed irregular margined, pleura based mass in the anterior segment of the right upper lobe, lingular lobe, and superior segment of the left lower lobe with osteolytic destruction of involved rib and invasion of the chest wall and extensive emphysema.
Figure 1: Chest X-ray showing left lung mass

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Figure 2: CT thorax showing pleura based mass with rib destruction

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A computed tomography guided biopsy of the lung mass was done, and histopathology [Figure 3] was reported as a biphasic appearance composed of carcinomatous areas with areas composed of malignant spindle cells. The carcinomatous area was composed of sheets of polygonal cells with eosinophilic cytoplasm, large vesicular pleomorphic-appearing nuclei, and prominent nucleoli. The malignant spindle cell component consisted of spindle cells arranged in sheets and fascicles with vesicular nuclei and prominent nucleoli. Foci of necrosis, frequent mitotic figures, and inflammatory cell infiltrate were also present. The epithelial component showed Thyroid transcription factor 1 (TTF-1) immunopositivity.and the sarcomatous areas were positive for cytokeratin and vimentin. Immunohistochemistry was negative for epidermal growth factor receptor. A diagnosis of SC was made, and the patient was referred to the Department of Medical Oncology.
Figure 3: Histopathology of the biopsy showed the biphasic appearance

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   Discussion Top


SC, is a relatively uncommon form of cancer whose malignant cells have histological, cytological, or molecular properties of both epithelial tumors (carcinoma) and mesenchymal tumors (sarcoma).[2] SC can occur in different organs such as the thyroid gland, bone, skin, breast, pancreas, liver, urinary tract, and lung.[3] However, SC in the lung is very rare, accounting for 0.1%–0.4% of all lung malignancies.[4]

PSC comprises a distinct clinicopathologic entity on the basis of morphologic, behavioral, and genotypic/phenotypic attributes.[5] The 2015 World Health Organization (WHO) classification of tumors of the lung, pleura, thymus, and heart divides PSC into five histological variants – pleomorphic carcinoma (>50%), spindle cell carcinoma, giant cell carcinoma, carcinosarcoma, and pulmonary blastoma.[6]

PSC is a poorly differentiated, aggressive tumor that affects smokers and males predominantly.[7] The average age of individuals at the diagnosis is 65 years, except for the pulmonary blastoma subtype, for which the average age at the diagnosis is 35 years. Multiple risk factors have been associated with SC of the lung, such as smoking cigarettes, cigars, or pipes, and exposure to asbestos in building construction and electrical insulation.[8]

SCs are unique among lung carcinomas in that, although they are considered carcinomas, they contain cytological and tissue architectural features that are usually characteristic of sarcoma. The 1981 WHO histological typing of lung tumors categorized SCs that corresponded to spindle cell, as a variant of squamous cell carcinoma.[9] The 1999 WHO histological typing of lung and pleural tumors, classified carcinomas with spindle and/or giant cells under the heading of “carcinomas with pleomorphic, sarcomatoid, or sarcomatoid elements.”[10] The 2004 WHO classification of lung tumors defined PSCs as “poorly differentiated nonsmall cell carcinoma that have a histological appearance suggesting mesenchymal differentiation.”[11] The 2015 WHO classification of lung tumors had no changes to diagnostic criteria/terminology for these tumors since the 2004 classification, but recommends molecular testing with histological findings.[6]

The location of SC may be central or peripheral. There is no specific clinical presentation, and patients may present with cough, dyspnea, hemoptysis, chest pain, or weight loss.[12] It is characterized by rapid growth, invasion, disease recurrence, and metastases.[5] SC had a median survival of 10 months.[13]

Most of these PSC can be diagnosed by light microscopy alone, although in certain situations, immunohistochemistry can be helpful.[14] The carcinoma components are usually squamous but may be glandular or neuroendocrine. The heterologous sarcomatoid component may comprise rhabdomyosarcoma, osteosarcoma, and chondrosarcoma.

Immunohistochemical analysis must show cytokeratin positivity. Vimentin, carcinoembryonic antigen, and smooth-muscle markers are also positive. Epithelial marker positivity is not essential if histopathological features of cancer are present. Till date, no prospective studies of SC have been done, due to the low incidence, complicated diagnostics needed, and tumor aggressiveness. Retrospective studies that compared PSC to conventional NSCLC reported worse median survival, overall survival, and more disease progression.[15]

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Baldovini C, Rossi G, Ciarrocchi A. Approaches to tumor classification in pulmonary sarcomatoid carcinoma. Lung Cancer (Auckl) 2019;10:131-49.  Back to cited text no. 1
    
2.
Ram R, Saadat P, Peng D, Vadmal M. Case report and literature review: Primary cutaneous carcinosarcoma. Ann Clin Lab Sci 2005;35:189-94.  Back to cited text no. 2
    
3.
Arshad HS, Dudekula RA, Niazi M, Malik S, Khaja M. A rare case of sarcomatoid carcinoma of the lung with spine metastasis, including a literature review. Am J Case Rep 2017;18:760-5.  Back to cited text no. 3
    
4.
Karim NA, Schuster J, Eldessouki I, Gaber O, Namad T, Wang J, et al. Pulmonary sarcomatoid carcinoma: University of Cincinnati Experience. Oncotarget 2017;9:4102-8.  Back to cited text no. 4
    
5.
Pelosi G, Sonzogni A, De Pas T, Galetta D, Veronesi G, Spaggiari L, et al. Review article: Pulmonary sarcomatoid carcinomas: A practical overview. Int J Surg Pathol 2010;18:103-20.  Back to cited text no. 5
    
6.
Travis WD, Brambilla E, Nicholson AG, Yatabe Y, Austin JH, Beasley MB, et al. The 2015 World Health Organization Classification of lung tumors: Impact of genetic, clinical and radiologic advances since the 2004 classification. J Thorac Oncol 2015;10:1243-60.  Back to cited text no. 6
    
7.
Smadhi H, Boudaya MS, Abdannadher M, BenAbdelghaffar H, Kamoun H, Ayadi A, et al. Pulmonary sarcomatoid carcinoma: A surgical diagnosis and prognostic factors. Tunis Med 2019;97:128-32.  Back to cited text no. 7
    
8.
Ishida T, Tateishi M, Kaneko S, Yano T, Mitsudomi T, Sugimachi K, et al. Carcinosarcoma and spindle cell carcinoma of the lung. Clinicopathologic and immunohistochemical studies. J Thorac Cardiovasc Surg 1990;100:844-52.  Back to cited text no. 8
    
9.
The World Health Organization histological typing of lung tumours. Second edition. Am J Clin Pathol 1982;77:123-36.  Back to cited text no. 9
    
10.
Travis WD, Colby TV, Corrin, B, Shimosato Y, Brambilla E. 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. New York: Springer-Verlag; 1999.  Back to cited text no. 10
    
11.
Beasley MB, Brambilla E, Travis WD. The 2004 World Health Organization classification of lung tumors. Semin Roentgenol 2005;40:90-7.  Back to cited text no. 11
    
12.
Yoshino N, Kubokura H, Yamauchi S, Ohaki Y, Koizumi K, Shimizu K. A true pulmonary carcinosarcoma that required diagnostic differentiation from a pleomorphic adenoma: A case report. Ann Thorac Cardiovasc Surg 2009;15:42-5.  Back to cited text no. 12
    
13.
Ro JY, Chen JL, Lee JS, Sahin AA, Ordóñez NG, Ayala AG. Sarcomatoid carcinoma of the lung. Immunohistochemical and ultrastructural studies of 14 cases. Cancer 1992;69:376-86.  Back to cited text no. 13
    
14.
Travis WD. Sarcomatoid neoplasms of the lung and pleura. Arch Pathol Lab Med 2010;134:1645-58.  Back to cited text no. 14
    
15.
Martin LW, Correa AM, Ordonez NG, Roth JA, Swisher SG, Vaporciyan AA, et al. Sarcomatoid carcinoma of the lung: A predictor of poor prognosis. Ann Thorac Surg 2007;84:973-80.  Back to cited text no. 15
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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