Submit Your Article CMED MEACR meeting
Home Print this page Email this page Users Online: 628
Home About us Editorial board Search Ahead of print Current issue Archives Submit article Instructions Subscribe Contacts Login 


 
 Table of Contents  
CASE REPORT
Year : 2012  |  Volume : 1  |  Issue : 4  |  Page : 236-238

Synchronous squamous cell carcinoma of lung and adenocarcinoma of esophagus in a patient with progressive systemic sclerosis: A case report and review of literature


Department of Radiotherapy, Sanjay Gandhi Post-graduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India

Date of Web Publication21-Jan-2013

Correspondence Address:
Tamojit Chaudhuri
Department of Radiotherapy, Sanjay Gandhi Post-graduate Institute of Medical Sciences, Lucknow - 226 014
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2278-0513.106276

Rights and Permissions
  Abstract 

We report an extremely rare case of synchronous metastatic squamous cell carcinoma of lung and adenocarcinoma of esophagus in a 32-year -old, non-smoker, Asian male with progressive systemic sclerosis. The short history and rapid progression of both the malignancies and systemic sclerosis in our patient suggested the possibility that systemic sclerosis may represent a systemic manifestation of malignancy.

Keywords: Adenocarcinoma esophagus, squamous cell carcinoma lung, systemic sclerosis


How to cite this article:
Chaudhuri T, Sharma D. Synchronous squamous cell carcinoma of lung and adenocarcinoma of esophagus in a patient with progressive systemic sclerosis: A case report and review of literature. Clin Cancer Investig J 2012;1:236-8

How to cite this URL:
Chaudhuri T, Sharma D. Synchronous squamous cell carcinoma of lung and adenocarcinoma of esophagus in a patient with progressive systemic sclerosis: A case report and review of literature. Clin Cancer Investig J [serial online] 2012 [cited 2020 Mar 28];1:236-8. Available from: http://www.ccij-online.org/text.asp?2012/1/4/236/106276


  Introduction Top


Systemic sclerosis (SSc) is a connective tissue disorder, characterized by fibrosis of the skin and visceral organs due to collagen deposition. According to the results of international studies and several reports, it is frequently associated with neoplastic diseases, particularly lung and breast malignancy, but whether there is a causal relationship, or not, that is still controversial. Here, we report a very rare case of synchronous metastatic squamous cell carcinoma of lung and adenocarcinoma of esophagus in a 32-year-old, non-smoker, Asian male with progressive SSc. To the best of our knowledge, this is the first ever reported case of SSc associated with synchronous lung and esophageal malignancy.


  Case Report Top


A 32-year-old, non-smoking, Asian male, presented with complaints of gradually progressive dysphagia to solids for last 2 months, and pain in his left shoulder for last 1 month. He had no family history of cancer. The diagnosis of systemic sclerosis (SSc) had been established 6 months earlier by skin biopsy. He was positive for anti-centromere and anti-topoisomerase-I antibody. At the time of presentation with dysphagia, he also had arthralgia, dryness and thickening of skin and Raynaud's phenomenon, which were aggravated for last 1 month. He had undergone an upper gastrointestinal endoscopic (UGIE) examination, which revealed an ulceroproliferative mass in the lower third thoracic esophageal lumen, reaching up to gastro-esophageal junction. A biopsy obtained during endoscopy was interpreted histopathologically as well-differentiated adenocarcinoma [Figure 1], immunohistochemically which was negative for p63 and cytokeratin 5/6. Clinically, metastasis to the left humerus head was suspected, and he was advised for whole body positron emission tomographic (PET) scan, which showed a FDG avid (maximum standardized uptake value (SUV)- 7.3) ill-defined enhancing soft tissue lesion in the right hilar region along with FDG uptake (SUV maximum- 6.1) and thickening involving distal esophagus and gastro-esophageal junction. There were also three other FDG avid lesions, involving left humerus head, left adrenal gland and right superior pubic ramus. Bronchoscopy guided biopsy was taken from the right hilar mass, histopathologically which revealed poorly-differentiated squamous cell carcinoma [Figure 2], which was immunoreactive for p63, cytokeratin 5/6 [Figure 3] and cytokeratin 19. A computed tomography (CT) guided biopsy was taken from the left humerus head, histopathologically which showed metastatic squamous cell carcinoma. The patient therefore, was diagnosed as a case of synchronous metastatic squamous cell carcinoma of lung and adenocarcinoma of esophagus associated with progressive SSc. He was planned for palliative chemotherapy with Gemcitabine (1000 mg/m 2 iv on day 1), Cisplatin (75 mg/m 2 iv on day 1) and Paclitaxel (175 mg/m 2 iv on day 1), every 3 weekly. There was no subjective improvement after receiving 2 cycles of chemotherapy. The patient died after the third chemotherapy cycle due to gross disease progression.
Figure 1: Histopathologic study of the esophageal biopsy showing well-differentiated adenocarcinoma with focal tubular proliferations (H and E, ×200)

Click here to view
Figure 2: Histopathologic study of the bronchoscopic lung biopsy showing poorly- differentiated squamous cell carcinoma (H and E, ×200)

Click here to view
Figure 3: The focal strong cytoplasmic staining in >50% tumour cells of poorly-differentiated squamous cell carcinoma by cytokeratin 5/6. (×400)

Click here to view



  Discussion Top


The association of malignancy with collagen vascular diseases is well documented in medical literature. Especially, relation between dermatomyositis and malignancy is well-recognized. [1] Systemic sclerosis (SSc) or scleroderma is a connective tissue disorder, characterized by fibrosis of the skin and visceral organs. According to the results of international studies, scleroderma is frequently accompanied by neoplastic diseases, among which the most often occurring is the neoplastic pathology of the lung and breast. Association of SSc with some other malignancies, such as cancer of the esophagus, stomach, colon, uterus, prostate, malignant lymphoproliferative disorders and functioning carcinoid tumors have also been reported. [2],[3],[4],[5],[6],[7] Malignancies, mainly in lung and breast, co-exist with idiopathic SSc or with SSc-like disorders, but not with localized forms of scleroderma (morphoea). Till date, the mechanisms connecting SSc and malignancies are not well understood. The occurrence of different types of cancer with SSc suggest different underlying mechanisms, including altered immune response, organ fibrosis, as a part of paraneoplastic syndrome, common genetic and environmental links, disease-dependent factors, tumor-derived biologic substances and therapies. [5],[6] The process of sclerosis itself may favour cancer in certain sites, and a reaction between T cells and neoantigens formed during irradiation may be a possible explanation of the frequent development of localized scleroderma after breast irradiation. [5],[8]

Gastrointestinal disease occurs in up to 90% of patients with SSc, with the esophagus being the most commonly affected organ. Heartburn, dysphagia, and gastroesophageal reflux is well documented in scleroderma. Esophageal disease in SSc is a common and difficult-to-treat problem. [9] In a retrospective analysis of Barrett's, metaplasia and adenocarcinoma of the esophagus in scleroderma, Katzka, et al. have reported that, Barrett's metaplasia of the esophagus occurs in one-third of patients with scleroderma and patients with scleroderma and Barrett's metaplasia have an increased risk of complications such as, stricture or adenocarcinoma. [10] Although, periodic endoscopic or radiologic surveillance, or both, of SSc patients with gastroesophageal reflux symptoms has been recommended by several authors for early detection of Barrett's mucosa and esophageal adenocarcinoma, but in their review of 680 closely followed SSc patients for esophageal carcinoma of all types, Segel MC, et al. did not find a significant increase in the frequency of esophageal carcinoma in SSc. Ultimately, they concluded that, regular surveillance for Barrett's esophagus and esophageal adenocarcinoma would not appear to be cost-effective because of the rarity of carcinoma, increased patient discomfort and expense, and the questionable benefit for long-term survival. [11]

Pulmonary involvement is the second most common visceral complication of SSc, after esophageal involvement. It has surpassed renal involvement as the most common cause of death. Interstitial lung disease and pulmonary vascular disease are the most commonly encountered types of lung involvement. [12] In a retrospective analysis of lung cancer in SSc, Colaci, et al. have reported higher frequency of lung cancer among SSc patients compared to general population, particularly within patients' subset with serum anti-Scl70 (also known as anti-topoisomerase- I) antibodies and lung involvement. [13]

The short history and rapid progression of both the malignancies and SSc in our patient suggested the possibility that SSc may represent a systemic manifestation of malignancy.

 
  References Top

1.Barnes BE, Mawr B. Dermatomyositis and malignancy. Ann Intern Med 1976;84:68-76.  Back to cited text no. 1
[PUBMED]    
2.Bielefeld P, Meyer P, Caillot D, Dalac S, Camus P, Tavernier C, et al. Systemic scleroderma and cancers: 21 cases and review of the literature. Rev Med Interne 1996;17:810-3.  Back to cited text no. 2
[PUBMED]    
3.Kang KY, Yim HW, Kim IJ, Yoon JU, Ju JH, Kim HY, et al. Incidence of cancer among patients with systemic sclerosis in Korea: Results from a single centre. Scand J Rheumatol 2009;38:299-303.  Back to cited text no. 3
[PUBMED]    
4.Szekanecz E, Szamosi S, Horváth A, Németh A, Juhász B, Szántó J, et al. Malignancies associated with systemic sclerosis. Autoimmun Rev 2012;11:852-5.  Back to cited text no. 4
    
5.Marasini B, Conciato L, Belloli L, Massarotti M. Systemic sclerosis and cancer. Int J Immunopathol Pharmacol 2009;22:573-8.  Back to cited text no. 5
[PUBMED]    
6.Takeda S. Systemic sclerosis (scleroderma) and malignancy. Nihon Rinsho Meneki Gakkai Kaishi 2004;27:389-96.  Back to cited text no. 6
[PUBMED]    
7.Zerafonetis CJ, Lorber SH, Hanson SM. Association of functioning carcinoid syndrome and scleroderma. I. Case report. Am J Med Sci 1958;236:1-14.  Back to cited text no. 7
    
8.Szabolcsi O, Nagy-Toldi A, Zeher M, Végh J. Systemic sclerosis in a patient suffering from breast cancer. Magy Onkol 2012;56:50-4.  Back to cited text no. 8
    
9.Ebert EC. Esophageal disease in progressive systemic sclerosis. Curr Treat Options Gastroenterol 2008;11:64-9.  Back to cited text no. 9
[PUBMED]    
10.Katzka DA, Reynolds JC, Saul SH, Plotkin A, Lang CA, Ouyang A, et al. Barrett's metaplasia and adenocarcinoma of the esophagus in scleroderma. Am J Med 1987;82:46-52.  Back to cited text no. 10
[PUBMED]    
11.Segel MC, Campbell WL, Medsger TA Jr, Roumm AD. Systemic sclerosis (scleroderma) and esophageal adenocarcinoma: Is increased patient screening necessary? Gastroenterology 1985;89:485-8.  Back to cited text no. 11
[PUBMED]    
12.Le Pavec J, Launay D, Mathai SC, Hassoun PM, Humbert M. Scleroderma lung disease. Clin Rev Allergy Immunol 2011;40:104-16.  Back to cited text no. 12
[PUBMED]    
13.Colaci M, Giuggioli D, Sebastiani M, Manfredi A, Vacchi C, Spagnolo P, et al. Lung cancer in scleroderma: Results from an Italian rheumatologic center and review of the literature. Autoimmun Rev 2012 Jun 25. In Press.  Back to cited text no. 13
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

Top
 
 
  Search
 
Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
Access Statistics
Email Alert *
Add to My List *
* Registration required (free)

 
  In this article
Abstract
Introduction
Case Report
Discussion
References
Article Figures

 Article Access Statistics
    Viewed1566    
    Printed53    
    Emailed0    
    PDF Downloaded104    
    Comments [Add]    

Recommend this journal