SESSION TYPE: Pleural Case Report Posters
PRESENTED ON: Tuesday, October 23, 2012 at 01:30 PM - 02:30 PM
INTRODUCTION: Kaposi Sarcoma(KS) and Gorham syndrome are both established but unusual causes of pleural effusion. While there is no known association, the pathogenesis of both is based on abnormal vascular proliferation. We describe a patient with both KS and Gorham syndrome who presented with recurrent pleural effusion related to pleural KS.
CASE PRESENTATION: A 49 year old male presented with recurrent, symptomatic right pleural effusion. He had undergone 2 prior thoracenteses, each time yielding a hemorrhagic, lymphocytic exudate. His medical history was significant for treated cutaneous KS and Gorham syndrome. His chest CT revealed a large right pleural effusion with lung collapse. There were no parenchymal lesions, and no pleural or thoracic bony abnormalities by CT scan. Pleural fluid analysis was similar to previous studies. Gram stain, culture and cytology were negative. The patient’s HIV and Quantiferon gold were negative. VATS revealed a fibrous pleural rind without focal pleural lesions. Biopsy of his parietal pleura was consistent with KS.
DISCUSSION: KS is a vascular tumor associated with human herpesvirus 8. It is a rare cause of pleural effusion, usually reported in the HIV population. Gorham Syndrome is a very rare disorder characterized by vascular proliferation within bone causing destruction and often extending into surrounding tissues. It is also associated with pleural effusions, although typically in association with thoracic bony lesions . KS and HHV have been associated with other lymphoproliferative disorders but not specifically with Gorham Syndrome.
CONCLUSIONS: We describe a case of recurrent pleural effusion related to KS in a non immunocompromised patient who also had a history of Gorham Syndrome. To our knowledge, there has been no reported association between KS and Gorham Syndrome. Given the proposed underlying patholophysiology of both processes, however, an association seems possible.
1) Choma ND, Biscotti CV, Bauer TW, Mehta AC, Licata AA. Gorham's syndrome: a case report and review of theliterature. Am 7 Med 1987;83:1151 -5.
2) D Feigl, L Seidel and A Marmor Gorham's disease of the clavicle with bilateral pleural effusions. Chest 1981;79;242-244
3) Shultz, TF. Kaposi’s sarcoma-associated herpesvirus (human herpesvirus 8): epidemiology and pathogenesis. Journal of Antimicrobial Chemotherapy (2000) 45, Topic T3, 15-27
DISCLOSURE: The following authors have nothing to disclose: Benjamin Michels
No Product/Research Disclosure Information, San Antonio, TX