|Year : 2015 | Volume
| Issue : 4 | Page : 940-942
Mesothelioma with superior vena cava obstruction in young female following short latency of asbestos exposure
Anupam Patra, Susmita Kundu, Amitava Pal, Sayantan Saha
Department of Pulmonary Medicine, R. G. Kar Medical College, Kolkata, West Bengal, India
|Date of Web Publication||15-Feb-2016|
Department of Pulmonary Medicine, R. G. Kar Medical College, Kolkata, West Bengal
Source of Support: None, Conflict of Interest: None
An 18 years female was admitted with right-sided chest pain, dry cough, and low-grade fever and weight loss for last 1 month. On examination, patient had features of superior vena cava (SVC) syndrome with right-sided pleural effusion. Chest X-ray showed mediastinal widening with nonhomogenous opacity mainly in the periphery of right upper and mid zone with right-sided pleural effusion. Ultrasonography thorax confirmed mild pleural effusion. Pleural fluid analysis showed lymphocytic, exudative, low adenosine deaminase with negative for Pap smear. Contrast-enhanced computed tomography (CT) thorax revealed large extensive nodular soft tissue lesion along right mediastinum as well as costal pleura, with enlarged pretracheal lymphadenopathy and SVC obstruction. CT guided Tru-cut biopsy report came as malignant epithelial tumor with polygonal shape, abundant eosinophilic cytoplasm and nuclei with prominent nucleoli suggestive of mesothelioma of epithelioid type. The tumor cell expressed calretinin, WT-1, and immunonegative for thyroid transcription factor-1.
Keywords: Female, malignant mesothelioma, superior vena cava obstruction, young
|How to cite this article:|
Patra A, Kundu S, Pal A, Saha S. Mesothelioma with superior vena cava obstruction in young female following short latency of asbestos exposure. J Can Res Ther 2015;11:940-2
|How to cite this URL:|
Patra A, Kundu S, Pal A, Saha S. Mesothelioma with superior vena cava obstruction in young female following short latency of asbestos exposure. J Can Res Ther [serial online] 2015 [cited 2020 Jul 8];11:940-2. Available from: http://www.cancerjournal.net/text.asp?2015/11/4/940/160924
| > Introduction|| |
Malignant mesothelioma is uncommon cancer. It is the most common malignant tumor of the pleura. Approximately, 70% of cases of pleural mesothelioma are associated with asbestos exposure. Incidence is higher in men than women, and mean age of presentation is 60 years as there is long latency period 30-40 years. , Patients of malignant mesothelioma usually presented with chest pain, dyspnea and cough.  Superior vena cava (SVC) obstruction is very unusual presentation.  We are going to present a case of mesothelioma with SVC obstruction in an 18 years young female with short period of exposure to asbestos roofing with only 2 years.
| > Case report|| |
An 18 years female was admitted with shortness of breath of Modified Medical Research Council grade II for 7 days, right-sided dull aching chest pain and dry cough with scanty expectoration for 4 weeks. The patient also noticed swelling of neck for 7 days. Patient had a history of low-grade intermittent fever for last 4 weeks and loss weight of about 3 kg in 1-month. Patient had no history of hemoptysis, hoarseness of voice or dysphagia.
On general examination, patient was conscious, co-operative, body mass index 17.8 kg/m 2 ; pallor, cyanosis, clubbing, edema, jaundice were absent only nonpulsatile engorged jugular venous pressure was found. On respiratory system examination, there was venous prominence over front of chest, with flow above downward and decreased movement of right side of chest. On percussion, there was impaired note on right side, and stony dull percussion note on right infrascapular area along with impaired mediastinal percussion. On auscultation, there was diminished breath sound on right side.
Chest X-ray showed mediastinal widening with pleural based homogenous opacity. On routine blood examination, Hb - 12 g%, white blood cell - 7500, red blood cell-normochromic normocytic, platelet - 2 lakhs/μL, blood sugar, liver function test were within normal limits. Pleural fluid analysis came as lymphocytic, exudative, low adenosine deaminase with negative for Pap smear. Contrast-enhanced computed tomography (CT) thorax revealed large extensive nodular soft tissue along right mediastinum as well as costal pleura, with enlarged paratracheal lymphadenopathy with SVC obstruction [Figure 1]. CT guided fine needle aspiration cytology revealed clusters of epithelial cells having round nuclei, ample eosinophilic cytoplasm and mild to moderate pleomorphism with vacuolation in Cytoplasm. Exact categorization was not possible from this report. CT guided Tru-cut biopsy report came as malignant epithelial tumor with polygonal shape, abundant eosinophilic cytoplasm and nuclei with prominent nucleoli suggestive of mesothelioma of epithelioid type [Figure 2]. Fiberoptic bronchoscopy revealed no abnormality. Immunohistochemistry was done for confirmation and to differentiate from metastatic adenocarcinoma and mesothelioma. The tumor cell expressed calretinin [Figure 3], WT-1 [Figure 4], and immunonegative for thyroid transcription factor-1 [Figure 5]. The final diagnosis was right-sided malignant pleural mesothelioma epithelioid type with SVC obstruction (tumor nodes metastases Stage IV).
|Figure 1: Contrast-enhanced computed tomography thorax: Large extensive nodular soft tissue along right mediastinum as well as costal pleura, with enlarged paratracheal lymphadenopathy with superior vena cava obstruction|
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|Figure 2: Tru-cut biopsy: Malignant epithelial tumor with polygonal shape, abundant eosinophilic cytoplasm and nuclei with prominent nucleoli suggestive of mesothelioma of epithelioid type|
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|Figure 5: The tumor cell expressed immunonegative for thyroid transcription factor-1|
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Patient was treated with chemotherapy cisplatin and premetraxed. After three cycles of receiving chemotherapy, patient clinically improved and now she is under follow-up.
On enquiry, patient gave history of living in a house with asbestos roofing for last 2 years and no other history of any secondary asbestos exposure.
| > Discussion|| |
Mesothelioma is the most common primary malignant tumor of the pleura. It is a rare cancer usually associated with asbestos exposure. Approximately, 70% of cases of pleural mesothelioma are associated with documented asbestos exposure. Other causes of mesothelioma have been reported. Recently, it is found that simian virus (SV40) contamination during the polio vaccination may be contributing to the development of mesotheliomas in the United States and other countries. 
Malignant pleural mesothelioma most commonly presents in the fifth to seventh decades of life. The mean age at presentation is 60 years because of the long latency (usually 30-40 years) from the time of first exposure to asbestos to the development of clinically evident disease.  But this female patient presented at the age of 18 years only. Few case reports in this age group are reported; lowest reported is 24 years
To develop mesothelioma long latency period usually 30-40 years are required.  But in our case, patient had a history of living in a house with asbestos roofing for last 2 years and no secondary exposure.
It is more common in men, almost 3-4 times than women. This is because of most asbestos exposures occurring in traditionally male-dominated work settings. It is seen asbestos-induced mesothelioma in women may be due to secondary asbestos exposure from their spouses' contaminated clothing. 
The most frequent presenting symptoms of pleural mesothelioma are nonpleuritic chest pain (60-70% of patients), dyspnea (25%), and cough (20%).  Mesothelioma in advanced stage can present as SVC syndrome. It is also rare presentation. In our case, the patient with 1-month history of chest pain presented with SVC syndrome. Very less number of cases of mesothelioma presented with SVC syndrome was reported. 
Median survival of patients with mesothelioma is between 9 and 12 months and varies depending on stage, histological subtype. Patients with Stage I disease had a median survival of 30 months and those with Stage IV had a median survival of 8 months. The prognostic scoring systems derived by the Cancer and Leukemia Group B (CALGB) and the European Organization for Research and Treatment of Cancer (EORTC) are the most useful clinical prognostic scoring schemes available.  According to CALGB prognostic index, serum lactate dehydrogenase (LDH) >500 IU/L, poor performance status, chest pain, platelet count over 400,000/μL, nonepithelial histology, and age older than 75 years jointly predicted poor survival and according to EORTC, female sex is the additional good prognostic indicator. Our patient was female with good performance status and LDH level <500 IU/L. In comparison to mixed and sarcomatoid type, epithelioid type of mesothelioma in our patient predicts better prognosis. , Only chest pain was the poor prognostic indicator in this female patient.
Most patients with mesothelioma are treated with chemotherapy. The most commonly used regimen is pemetrexed with a platinum drug such as cisplatinum. The use of this combination is better than the cisplatin-alone in respect of response rates (41.3% vs. 16.7%), survival (median survival 12.1 months vs. 9.3 months).  The other regimen used commonly is gemcitabine with a platinum agent and 50% patients on this doublet regimen had been found symptomatic improvement, 33% had a partial response, and 60% had stable disease; no survival benefit was demonstrated compared with historical controls. This patient was received three cycles of chemotherapy with cisplatin and premetrexed; clinical response was good. She is now under follow-up.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]