Solitary fibrous tumors (SFT) are mesenchymal cell tumors that may arise from any site throughout the body. A small percentage of patients with SFT develop non-islet cell tumor-induced hypoglycemia (NICTH), eponymously termed Doege-Potter Syndrome (DPS). DPS is characterized by severe, refractory hypoinsulinemic hypoglycemia. Diagnosis of SFT is dependent on histologic findings and immunohistochemistry (IHC). NAB2-STAT6 gene fusions are pathognomonic for SFT but may be difficult to identify in routine cytogenetic studies. STAT6 IHC is a highly sensitive and specific surrogate for the NAB2-STAT6 gene fusion. Total resection of the tumor remains the gold-standard definitive treatment of SFT of the pleura. Palliative tumor debulking is recommended if total resection is not feasible. We here report a case of DPS in a 73-year-old female, managed with palliative care.
Keywords: endocrinology; hypoglycemia; recurrent hypoglycemia; solitary fibrous pleural tumour; solitary fibrous tumor.
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