Malignant Abdominal Mesothelioma Presenting with Features of Superior Vena Cava Syndrome. A Case Report
Athanasia Proklou1, Evaggelos Maridakis1, Evaggelia E Vassalou2, Eumorfia Kondili*1
1ICU, University Hospital of Heraklion, Medical School, University of Crete, Heraklion, Crete, Greece
2Department of Medical Imaging, University Hospital of Heraklion, Medical School, University of Crete, Heraklion, Crete, Greece
Superior vena cava (SVC) syndrome is life threatening clinical condition caused by obstruction of the SVC either by extrinsic compression or by internal thrombus. More than 80% of cases of SVCS are caused by malignant mediastinal tumors. Herein we report a rare case of superior vena cava syndrome as first manifestation of abdominal malignant mesothelioma.
A 73-years-old man presented at the emergency department complaining of acute loss of vision within few hours, sore throat, shortness of breath, and feeling of swelling with signs of cyanosis of the upper limb, head and neck. A chest computed tomography following intravenous contrast administration revealed the presence of a well defined central thrombus in superior vena cava extending from the level of the right pulmonary artery to the proximal right internal jugular vein. A contrast enhanced CT of the abdomen revealed an enhancement of the diaphragmatic surface of the liver by soft tissue, and thickening of the mesentery, with abundant fluid between bowel loops. Histological examination of a specimen obtained by biopsy established the diagnosis of malignant mesothelioma.
We report a case of malignant peritoneal mesothelioma with leading symptoms and signs of Superior vena cava syndrome. Clinicians should be aware that malignant peritoneal mesotheliomas can present without any typical symptoms from the abdomen.
Keywords: Superior Vena Cava Syndrome; Thrombus; Peritoneal Malignant Mesothelioma; Laparoscopy
Mesotheliomas are highly aggressive tumors arising from serous surfaces as pleura (65%-70%), peritoneum (30%), tunica vaginalis testis, and pericardium (1%-2%)  Peritoneal mesothelioma is a rare malignant tumor with a rapid fatal course and median survival 6-12 months. Asbestos exposure is the principal risk factor for the disease. It can occur in any age group, although the 50 to 69 year age group is the that most affected. It is more common in men, possibly because of the higher male occupational exposure to asbestos.  Only 50% of patients with a peritoneal origin of malignant mesothelioma (MPeM) have a history of asbestos exposure, in contrast to 80% in mesotheliomas with pleural origin. . Clinical presentation of malignant peritoneal mesothelioma includes pain and or swelling , in the abdomen, ascites, nausea ,vomiting, weight loss, fever , bowel obstruction and anemia. Herein we report a rare case of malignant abdominal mesothelioma presenting with features of Superior vena cava syndrome.
A 73-years-old Caucasian male, lifelong non-smoker, presented at the emergency department complaining of acute loss of vision within few hours, sore throat, shortness of breath, and feeling of swelling with signs of cyanosis of the upper limb, head and neck. His physical examination revealed sinus tachycardia, with 90rpm, arterial hypertension and tachypnea. Laryngoscopy revealed gelatinous uvular edema, swelling of the right arytenoids and aryepiglottic folds without movement deficit.
A chest computed tomography following intravenous contrast administration showed the presence of a well defined central thrombus in superior vena cava extending from the level of the right pulmonary artery to the proximal right internal jugular vein with no signs of pulmonary embolism (Figure 1).Cardiac U/S demonstrated the presence of a mobile thrombus in the entrance of the right atrium and dilatation of the right heart.
The patient was admitted to the intensive care unit (ICU) and treated with anticoagulants for prevention of clot propagation and corticosteroids for the treatment of the laryngeal edema. A contrast enhanced CT of the abdomen was performed with evidence of encasement of the diaphragmatic surface of the liver by mildly enhancing soft tissue, and thickening of the mesentery. Moreover there was abundant fluid between the bowel loops and in the left paracolic gutter and a multicystic intrapelvic mass with enhancing internal septations (Figures 2, 3). However, accounting on the inconclusive cytologic findings from the diagnostic paracentesis of the exudative ascitic fluid, we decided to proceed with diagnostic laparoscopy. Histological examination of peritoneum specimen revealed scattered epithelioid malignant mesothelioma of abdomen origin.
Figure1: Chest computed tomography following intravenous contrast administration, in transverse planes (a, b,) and coronal reconstruction (c). There is a centrally located superior vena cava thrombus (arrowheads in a) extending to the proximal right internal jugular vein (arrow in c). There is also complete occlusion of the azygous vein (arrows in b). Note the extensive right chest wall and lateral thoracic collateral venous network (arrows in a).
During the course of hospitalization the patient’s medical condition was deteriorated and was complicated with multiple organ failure. A multidisciplinary approach of the ICU with the oncology and surgical department was held. Due to the advanced state of the disease, any treatment approach was considered to be futile.
Figure 2. Abdominal contrast enhanced computed tomography images in transverse plane. There is enhancement of the diaphragmatic surface of the liver by mildly enhancing soft tissue (arrowheads)
In our case we believe that the SVC syndrome was due to in situ thrombosis due to malignant hypercoagulopathy state or thromboembolic disease. Hypercoagulopathy is common complication in patients with malignant disease. The incidence of clinical episodes of thromboembolism in patients with cancer varies from 1% to 11%. In cases of malignant mesothelioma, is described that clotting abnormalities occur in 10-20%. 
Cite this article: Eumorfia.Malignant Abdominal Mesothelioma Presenting with Features of Superior Vena Cava Syndrome. A Case Report. J J Pulmonol. 2015, 1(3): 014.