Successful Multimodal Treatment of an IGF2-Producing Solitary Fibrous Tumor With Acromegaloid Changes and Hypoglycemia

Journal of the Endocrine Society
Ramón G De Los Santos-AguilarNicole M Iñiguez-Ariza

Abstract

Doege-Potter syndrome with acromegaloid facial changes is extremely rare. Uncooked cornstarch along with glucocorticoids have been used as supportive care in patients with non-islet cell tumor hypoglycemia (NICTH). Preoperative embolization of hepatic solitary fibrous tumors (SFT) with NICTH has yielded unsatisfactory results. Herein we present the case of a 61-year-old man with a 3-month history of severe frequent hypoglycemic episodes and acromegaloid facial changes. During a spontaneous hypoglycemia (26 mg/dL), laboratory values showed a hypoinsulinemic pattern with low levels of GH, IGFPB3, and an IGF2/IGF1 ratio of 8.5:1. Cross-sectional imaging revealed a large (16 × 13 × 11 cm) hepatic tumor, and cytology was consistent with SFT. A preoperative right portal embolization was performed in an effort to induce normal remnant liver hypertrophy to allow for safe tumor resection. After the procedure, uncooked starch treatment followed by prednisone was started, achieving complete remission of hypoglycemic episodes in the preoperative setting. He subsequently underwent partial hepatectomy. The histologic diagnosis was compatible with a potentially malignant SFT. The patient had an excellent outcome with complete remission of hyp...Continue Reading

References

Apr 1, 1995·Clinical Endocrinology·N TrivediW H Daughaday
Jun 30, 2004·Diabetes & Metabolism·L Morbois-TrabutC Couet
Nov 28, 2007·Journal of Hepato-biliary-pancreatic Surgery·Gabriel ChanPeter Metrakos
Nov 30, 2007·Endocrine-related Cancer·Jan Willem B de GrootWinette T A van der Graaf
Apr 18, 2008·Langenbeck's Archives of Surgery·F FamàO Saint Marc
Jun 8, 2013·World Journal of Gastroenterology : WJG·Manuel JakobReto Kaderli
Oct 2, 2013·Endocrine-related Cancer·Callum Livingstone
Jan 16, 2014·The Journal of Clinical Endocrinology and Metabolism·Timothy W BodnarMassimo Pietropaolo
Jan 25, 2014·European Journal of Endocrinology·Pedro Iglesias, Juan J Díez
Apr 4, 2015·Internal Medicine·Yuki MakinoTetsuo Takehara
Oct 23, 2015·Journal of Gastrointestinal Cancer·Marcelo A Beltrán
Mar 21, 2018·Annales d'endocrinologie·Arnaud JanninMarie-Christine Vantyghem

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BETA
surgical resection
biopsy

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