Giant Solitary Fibrous Tumor of the Pleura (SFTPs) associated with paraneoplastic Doege Potter Syndrome

Abstract

SFTPs are uncommon spindle cell mesenchymal tumors. These tumors arise in pleural as well as in extra pleural sites like liver, orbit, lung, nasal passages, meninges, respiratory tract, thyroid, soft tissues, major salivary glands, kidney, periosteum and spinal cord. SFTPs of thorax are interesting in that they can present with mass effect and paraneoplastic syndrome. Doege Potter Syndrome that is Giant SFTP presenting as hypoglycemic coma is an exceptional presentation of SFTPs. SFTPs are often mistaken for epithelial mesotheliomas. Positivity for vimentin, a marker of mesenchymal cells, negativity for cytoplasmic keratin which is positive in mesotheliomas and expression of Clusters of Differentiation (CD) 34 antigens can confirm the presence of a SFTP. Ultra structural studies further support the fibroblastic nature of these tumors. We hereby report an exceptional case of a 50-year-old lady with giant SFTP presenting with hypoglycemic coma. A diagnostic as well as therapeutic thoracotomy with complete surgical resection of the tumor was performed with complete relief of symptoms.