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A Rare Presentation of Malignant Mesothelioma in a Young Adult
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A4050 - A Rare Presentation of Malignant Mesothelioma in a Young Adult
Author Block: K. Ilyas1, M. Raza2, J. Subramanian2; 1Internal Medicine, Mercy health Saint Mary's, Grand Rapids, MI, United States, 2Internal Medicine, Spectrum health, Grand Rapids, MI, United States.
Introduction:
Mesothelioma is a malignant neoplasm that arises from the mesothelial surfaces of serous cavities. The majority of cases are attributed to occupational or environmental asbestos exposure and there is a very long latency period before the exposure and development of mesothelioma. Up to 20% of mesothelioma cases occur in the patient population without asbestos exposure. Risk factors in this patient population includes radiation exposure, exposure to non-asbestos mineral fibers, simian virus and genetic predisposition. Most patients present with gradual onset of non-specific symptoms including chest pain, dyspnea, cough or hoarseness. Chest imaging typically shows unilateral pleural thickening and pleural effusion. The prognosis is poor with medial survival time of 9 to 17 months after the diagnosis. We describe a rare presentation of malignant mesothelioma in young adult.
Case Presentation:
22 years old Man without any significant past medical history presented initially with ascites, bilateral pleural effusion, Bilateral Pulmonary embolism, thrombi in left subclavian and gastrointestinal blood vessels, mediastinal and neck lymphadenopathy. Serial CT scans showed developing ground glass opacities in the Right lower lob and a loculated pulmonary effusion. thoracocentesis showed chylothorax with triglyceride count greater than 2000 mg/dl, pleural fluid negative for infection or malignancy. Duodenal and jejunal biopsy intraepithelial lymphocytes without villous blunting. Inguinal lymph node excision biopsy and peritoneal biopsy showed reactive cells. Extensive ID work up Ruled out systemic brucella, Q fever, CMV, EBV, HIV, HHV8, histoplasmosis. PET scan showing hypermetabolic nodules in supraclavicular, inguinal area and diffusely increased uptake in peritoneal cavity. Based on these findings he was diagnosed with Multicentric Castleman’s disease of plasma cell variant though he was negative for HIV and HHV-8, he was given a course of high dose steroids with no improvements in his symptoms. FNA biopsy of Enlarging Right lower paratracheal and subcranial lymph node showed malignant epithelioid cells positive for AE, calretinin and negative for TTF and alk-1, which finally established the diagnosis of malignant mesothelioma.
Discussion:
Although it is considered a disease of the elderly, it can present in young population. 2% of the reported cases in USA are in age less than 40. In this young patient population, genetic predisposition and exposure to carcinogenic mineral fibers from an early age are possible etiological factors. The prognosis is very poor but young age is associated with better survival. Our case demonstrates that mesothelioma should be kept in the differential list without asbestos exposure and old age.
Author Block: K. Ilyas1, M. Raza2, J. Subramanian2; 1Internal Medicine, Mercy health Saint Mary's, Grand Rapids, MI, United States, 2Internal Medicine, Spectrum health, Grand Rapids, MI, United States.
Introduction:
Mesothelioma is a malignant neoplasm that arises from the mesothelial surfaces of serous cavities. The majority of cases are attributed to occupational or environmental asbestos exposure and there is a very long latency period before the exposure and development of mesothelioma. Up to 20% of mesothelioma cases occur in the patient population without asbestos exposure. Risk factors in this patient population includes radiation exposure, exposure to non-asbestos mineral fibers, simian virus and genetic predisposition. Most patients present with gradual onset of non-specific symptoms including chest pain, dyspnea, cough or hoarseness. Chest imaging typically shows unilateral pleural thickening and pleural effusion. The prognosis is poor with medial survival time of 9 to 17 months after the diagnosis. We describe a rare presentation of malignant mesothelioma in young adult.
Case Presentation:
22 years old Man without any significant past medical history presented initially with ascites, bilateral pleural effusion, Bilateral Pulmonary embolism, thrombi in left subclavian and gastrointestinal blood vessels, mediastinal and neck lymphadenopathy. Serial CT scans showed developing ground glass opacities in the Right lower lob and a loculated pulmonary effusion. thoracocentesis showed chylothorax with triglyceride count greater than 2000 mg/dl, pleural fluid negative for infection or malignancy. Duodenal and jejunal biopsy intraepithelial lymphocytes without villous blunting. Inguinal lymph node excision biopsy and peritoneal biopsy showed reactive cells. Extensive ID work up Ruled out systemic brucella, Q fever, CMV, EBV, HIV, HHV8, histoplasmosis. PET scan showing hypermetabolic nodules in supraclavicular, inguinal area and diffusely increased uptake in peritoneal cavity. Based on these findings he was diagnosed with Multicentric Castleman’s disease of plasma cell variant though he was negative for HIV and HHV-8, he was given a course of high dose steroids with no improvements in his symptoms. FNA biopsy of Enlarging Right lower paratracheal and subcranial lymph node showed malignant epithelioid cells positive for AE, calretinin and negative for TTF and alk-1, which finally established the diagnosis of malignant mesothelioma.
Discussion:
Although it is considered a disease of the elderly, it can present in young population. 2% of the reported cases in USA are in age less than 40. In this young patient population, genetic predisposition and exposure to carcinogenic mineral fibers from an early age are possible etiological factors. The prognosis is very poor but young age is associated with better survival. Our case demonstrates that mesothelioma should be kept in the differential list without asbestos exposure and old age.
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