Doege-Potter Syndrome Case Study

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Doege-Potter Syndrome, cause of non-islet cell tumor hypoglycemia, the first case report from Nepal. Abstract Doege–Potter syndrome (DPS), a paraneoplastic syndrome, presents as a hypoinsulinaemic hypoglycemia from the ectopic secretion of insulin-like growth factor II (IGF-II) from a solitary fibrous tumor which may be intrapleural or extrapleural in origin. We report a case of severe hypoglycemia in a 68-year old lady initially admitted for resection of left sided solitary fibrous tumor of pleura (SFTP). Investigation revealed true hypoglycemia and DPS was diagnosed. The tumor was completely resected and the patient made a full recovery with no further hypoglycemic episodes. This is probably the first case of SFTP with DPS reported from…show more content…
Of the cases reported globally, 50% are benign and 50% have malignant transformation.6 Definitive diagnosis of a SFT relies on histological identification of a spindle cell tumor with a distinctive fibroblastic morphology, arranged as areas of alternating cellularity and hypocellular collagenous stroma, as well as consistent CD34 expression.7 In trucut biopsy of the specimen taken from this case, diffuse proliferation of spindle shaped cells with blood vessels in between was seen. These spindle cells were mildly pleomorphic and had elongated cytoplasm with hyperchromatic nucleus. Mitotic figure was not observed and there was no necrosis. Blood testing at time of hypoglycemia in these patients reveals a high IGF-II: IGF-I ratio (indicating high IGF-II) and low insulin concentration.7 Estimation of IGF-I and II could not be done in this case due to non-availability of the test. Serum C-Peptide was very less during the period of hypoglycemia suggesting true hypoglycemia. Surgery is the first effective treatment for benign and malignant

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