CASE REPORT: DOEGE-POTTER SYNDROME.

Introduction:- Solitary fibrous tumour of the pleura (SFTP) is a rare pleural tumour. Sometimes, these tumours can produce a large IGF-2 molecule which causes hypoglycemic episodes which is referred to as Doege Potter syndrome (DPS). Doege Potter syndrome can present with life-threatening hypoglycemia, hence its early recognition is important as many are amenable to surgical resection. We report a case of benign SFTP presenting with Doege Potter syndrome, that presented to our hospital in 2014. Case Presentation:- A 45 year old women with history of intra-thoracic tumour presented with recurrent hypoglycemia. At the time of hypoglycemia (blood sugar 32 mg /dl), S. Insulin < 0.5 uU/ml and C-peptide =0.699ng/ml. IGF-1 was <25 ng/ml and IGF-2 was 328 ng/ml. IGF2:IGF1 ratio was >13. CECT Chest showed a large well defined smooth marginated, hypodense, soft tissue mass lesion of size 20x12x13 cms with mild heterogeneous enhancement seen in mid and lower zones of right lung. CT guided biopsy of tumor was suggestive of spindle cell tumor of pleura. Immuno-histochemistry showed a bland spindle cell neoplasm in collagenised stroma. Tumor expessed CD 34, Mic 2, bcl 2 and was immune-reactive for cytokeratin, EMA, Calretinin, Calponin, CD 56. PET scan showed a mild metabolically active large right lung lesion with no tracer activity in liver, adrenal or other sites. Thus, a diagnosis of a benign SFTP with Doege Potter syndrome was made. Hypoglycemic management of our patient included regular IV dextrose infusion, scheduled, intermittent feeding overnight and IV steroids. The patient, then underwent surgical resection of the tumor. During the postoperative period, she had no hypoglycemic episodes, IGF-1 was 25 ng/ml and IGF-2 was 196 ng/ml, and the IGF-2:IGF-1 ratio came down (<10). Patient discharged in stable condition without any hypoglycemia or any need of steroids. Conclusion:- Doege Potter syndrome is a paraneoplastic manifestation of large non-islet tumour due to production of IGF-2. It should be considered in patients who have intra-thoracic tumour and recurrent hypo-insulinemic hypoglycemia. Most case series on pleural fibrous tumours report hypoglycemia in about 2-4%. The ratio IGF-2 to IGF-1 is used as a surrogate marker to diagnose Doege Potter syndrome. Surgical resection of the tumour is the treatment modality of choice, which cures the hypoglycemia. - See more at: .