|Year : 2016 | Volume
| Issue : 4 | Page : 537-540
Pulmonary sarcomatoid carcinoma: An uncommon entity in a 40 years female
Sayantan Saha, Somnath Bhattacharya, Atin Dey, Saurav Kar
Department of Pulmonary Medicine, R. G. Kar Medical College, Kolkata, West Bengal, India
|Date of Web Publication||12-Jul-2016|
136 Parmar Road, Gita Bhawan, Flat No. 202, P.O. Bhadrakali, Hooghly - 712 232, West Bengal
Source of Support: None, Conflict of Interest: None
A middle-aged female presented with nonpleuritic chest pain with progressive dyspnea and unmeasured weight loss and anorexia for last 4 months. The patient had pallor, clubbing, but no peripheral lymphadenopathy. Clinically, vesicular breath sound was diminished over anterior, posterior, and lateral aspect of the right hemithorax with a dull note on percussion. Chest radiography showed homogenous opacity involving the right hemithorax with blunting of right costophrenic angle without contralateral shifting of the lower mediastinum. Contrast-enhanced computed tomography (CT) thorax revealed a large mixed density mass lesion occupying the entire right hemithorax with peripheral contrast enhancement. The lesion narrowed the right pulmonary artery and insinuated the adjacent mediastinum, but no evidence of pleural nodularity. CT guided fine-needle aspiration cytology from the mass was inconclusive. Tru-cut biopsy from the mass showed spindle-shaped cells, with nuclear pleomorphism and mitotic figures, arranged in interlacing fascicles. The histopathology was suggestive of spindle cell neoplasm. Immunohistochemistry (IHC) showed pancytokeratin (AE1/3), cytokeratin 7, vimentin, and epithelial membrane antigen positivity, whereas immunonegativity to thyroid transcription factor-1, WT-1, and calretinin. The radiological picture with IHC pattern favored the diagnosis of pulmonary sarcomatoid carcinoma.
Keywords: Biphasic tumor, immunohistochemistry, pulmonary sarcomatoid carcinoma, spindle cell
|How to cite this article:|
Saha S, Bhattacharya S, Dey A, Kar S. Pulmonary sarcomatoid carcinoma: An uncommon entity in a 40 years female. Med J DY Patil Univ 2016;9:537-40
| Introduction|| |
Sarcomatoid carcinoma is an extremely rare biphasic tumor characterized by a combination of malignant epithelial and mesenchymal cells. Although reported in various sites including bladder, colon, uterus, yet lung is not a common organ to be involved. Diagnosis needs an array of investigations and the prognosis is very poor despite early treatment. Here, a case of pulmonary sarcomatoid carcinoma (PSC) with a radiological resemblance to unilateral massive pleural effusion is being reported.
| Case Report|| |
A 40-year-old nonsmoker housewife residing at a rural part of West Bengal presented in our tertiary care hospital with complaints of chest pain along with gradually progressive dyspnea for last 4 months. Her illness started with the dull aching nonpleuritic chest pain of moderate intensity 4 months back which persisted despite taking an analgesic. She had progressively increasing dyspnea and was modified medical research council (mMRC) Grade 3 on admission. There was single episode of scanty hemoptysis around 15 days back which subsided spontaneously. However, there was no history of persistent fever and cough but had unmeasured weight loss and anorexia during this period.
On general examination, she was thin build with poor nutritional status. The patient had pallor, grade 1 clubbing, but no cyanosis or peripheral lymphadenopathy. She had tachypnea (respiratory rate 32/min) with oxygen saturation 98% at rest. Examination of the respiratory system showed centrally placed trachea, diminished vesicular breath sound over anterior, posterior, and lateral aspect of the right hemithorax with a dull note on percussion in the corresponding areas. Examination of other systems was normal. Blood examination showed anemia (hemoglobin 8.6 g%), high erythrocyte sedimentation rate (82 mm/h), and normal white blood cell count 7900/mm 3. Blood biochemistry was normal and sputum for acid-fast bacilli was negative. Chest radiography [Figure 1] showed homogenous opacity involving the entire right hemithorax with blunting of right costophrenic angle without contralateral shifting of lower mediastinum and trachea remaining central. CECT thorax [Figure 2] and [Figure 3] revealed a large (measuring 106 mm × 98 mm × 144 mm) mixed density mass lesion occupying the entire right hemithorax, having both solid and cystic component with peripheral contrast enhancement. The lesion narrowed the right pulmonary artery and insinuated the adjacent mediastinum but had a line of separation from the mediastinal structures. There was minimal right-sided pleural effusion with no evidence of any pleural nodularity. CT-guided fine-needle aspiration cytology from the mass showed acute on chronic inflammation with Ziehl–Nielsen stain being negative. Fiberoptic bronchoscopy showed narrowing of right intermediate bronchus due to extrinsic compression with scattered areas of unhealthy congested mucosa. There was no evidence of any intraluminal growth. We did tru-cut biopsy from the mass which [Figure 4] showed spindle-shaped cells arranged in interlacing fascicles with occasional areas of necrosis. Those spindle cells showed nuclear pleomorphism and mitotic figures. The histopathology was suggestive of spindle cell neoplasm. For differentiation, we performed immunohistochemistry study which showed pancytokeratin (AE1/3), cytokeratin 7, vimentin, and epithelial membrane antigen (EMA) positivity, whereas immunonegativity to thyroid transcription factor-1, WT-1, and calretinin.
|Figure 1: Chest X-ray showing right sided homogenous opacity involving most of the right hemithorax and mimicking pleural effusion|
Click here to view
|Figure 2: Contrast-enhanced computed tomography thorax showing a large mixed density lesion having both solid and cystic component involving right lung, with no evidence of pleural thickening|
Click here to view
|Figure 3: Contrast-enhanced computed tomography thorax (coronal section) a mixed density lesion involving right hemithorax|
Click here to view
|Figure 4: Histopathology of computed tomography guided tru-cut biopsy slide showing spindle shaped cells arranged in interlacing fascicles. These spindle cells showed nuclear pleomorphism, mitotic figures and areas of necrosis|
Click here to view
We considered possibilities of synovial sarcoma, sarcomatoid carcinoma, and sarcomatoid mesothelioma. Synovial sarcoma though EMA positive, is usually a disease of young adults and children. The presence of large intrapulmonary mass, absence of pleural thickening and asbestos exposure, and immunonegativity to calretinin and WT-1 favored the diagnosis of sarcomatoid carcinoma.
The patient underwent surgical resection of mass followed by initiation chemotherapy with cisplatin and docetaxel. The patient, however, did not survive beyond 3 months from the period of diagnosis.
| Discussion|| |
PSC is a histological subtype of poorly differentiated nonsmall cell carcinoma, characterized by a combination of malignant epithelial cells (spindle cell or giant cell) and sarcomatoid component (neoplastic but differentiated connective tissue element like neoplastic bone, cartilage, striated muscle cells, and mesenchymal cells). The World Health Organization 2004 classification has defined PSC as “poorly differentiated nonsmall cell carcinoma that have a histological appearance suggesting mesenchymal differentiation.” PSC has five different variants namely pleomorphic carcinoma, spindle cell carcinoma, giant cell carcinoma, carcinosarcoma and pulmonary blastoma. PSC represent 0.3-1.3% of all lung tumors  and occur mostly in men in their sixth and seventh decades except for pulmonary blastoma which occur equally in both genders frequently in fourth decade.
Compared to other histological subtypes, PSC generally runs an aggressive clinical course with local advancement, and preoperative diagnosis is very difficult because of its heterogeneity. In the major published series of PSC, no specific clinicoradiological features have been found compared to other typical nonsmall cell lung carcinoma (NSCLC). Typical symptoms are same as other lung cancer including dyspnea, chest pain, cough, hemoptysis, and weight loss. Radiologically, it present as a solitary voluminous peripheral heterogenous mass with chest wall invasion (60% cases) and vascular invasion (20% cases). Moreover, small biopsy samples may not disclose both epithelial and sarcomatoid components of the tumor which is often misdiagnosed as NSCLC in >60% cases. Hence, an excision biopsy en mass is imperative in most of the cases. Macroscopically tumors are voluminous with consistency being soft and fleshy, firm to hard and cut surfaces vary from gray-white to tan-yellow with areas of hemorrhage, necrosis, and occasionally cavitation. Microscopically broad areas of geographic and hemorrhagic necrosis are typically identified. Spindle cells and/or giant cell component should comprise at least 10% of the tumor to be appropriately classified.
PSC generally carries a worse prognosis than the conventional NSCLC even at surgically curable Stage 1. Prognosis depend on stage, size and location of the tumor, metastasis and associated genetic mutation (K-Ras or p53 mutation). Treatment modalities are same as NSCLC. However, additionally, epidermal growth factor receptor targeted therapies (erlotinib) are under trial.
Our case was uncommon in the perspective of the factors that PSC is a rare variety of lung carcinoma that usually do not occur in middle-aged female and its diagnosis warrant a strong clinical suspicion along with different modalities of investigations. There was a case of PSC reported recently in a 68-year-old male with rapid progression, but occurrence in the middle-aged female is a noteworthy factor for our case.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Terzi A, Gorla A, Piubello Q, Tomezzoli A, Furlan G. Biphasic sarcomatoid carcinoma of the lung: Report of 5 cases and review of the literature. Eur J Surg Oncol 1997;23:457.
Girard N, Cadranel J, Brambilla E, Cordier JF. Lymphoma, lymphoproliferative diseases and other primary malignant tumors. Murray and Nadal's Textbook of Respiratory Medicine. 5th
ed., Ch. 48. Philadelphia: Elsevier; 2010. p. 1152-3.
Litzky LA. The pathology of non-small cell lung carcinoma. Fishman's Pulmonary Diseases and Disorders. 4th
ed., Ch. 104. Philadelphia: McGraw Hill; 2008. p. 1840-1.
Beasley MB, Brambilla E, Travis WD. The 2004 World Health Organization classification of lung tumors. Semin Roentgenol 2005;40:90-7.
Chang YL, Lee YC, Shih JY, Wu CT. Pulmonary pleomorphic (spindle) cell carcinoma: Peculiar clinicopathologic manifestations different from ordinary non-small cell carcinoma. Lung Cancer 2001;34:91-7.
Franks TJ, Galvin JR. Sarcomatoid carcinoma of the lung: Histologic criteria and common lesions in the differential diagnosis. Arch Pathol Lab Med 2010;134:49-54.
Rossi G, Cavazza A, Sturm N, Migaldi M, Facciolongo N, Longo L, et al.
Pulmonary carcinomas with pleomorphic, sarcomatoid, or sarcomatous elements: A clinicopathologic and immunohistochemical study of 75 cases. Am J Surg Pathol 2003;27:311-24.
Kim TH, Kim SJ, Ryu YH, Lee HJ, Goo JM, Im JG, et al.
Pleomorphic carcinoma of lung: Comparison of CT features and pathologic findings. Radiology 2004;232: 554-9.
Girard N, Barbareschi M, Cordier JF, Murer B. What is a rare tumor, and how to deal with it clinically? Eur Respir Monogr 2007;39:85-133.
Travis WD, Colby TV, Corrin B, Shimosato Y, Brambilla E, et al
. WHO Histological Classification of Tumours. Histological Typing of Lung and Pleural Tumours. 3rd
ed. Berlin: Springer-Verlag; 1999.
Kakkar A, Jain D, Khanna P, Deo SS, Sarkar C. Sarcomatoid carcinoma of lung – A case report and review of epidermal growth factor receptor mutation status. Lung India 2015;32:533-5.
[Figure 1], [Figure 2], [Figure 3], [Figure 4]