A Rare Diagnosis After the Fall of a 96-Year-Old Woman: Doege-Potter Syndrome

Author(s): Angela Ida Pincelli, Mario Perotti, Francesca Massariello, Antonella Gatti, Damiano Calella, Vincenzo Cimino, Justin Haas, Giuseppe Bellelli, Paolo Mazzola*, Giorgio Annoni.

Journal Name: Current Aging Science

Volume 11 , Issue 3 , 2018

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Graphical Abstract:


Introduction: Doege-Potter Syndrome (DPS) is a rare but life-threatening paraneoplastic syndrome, characterized by Non-Islet Cell Tumor-Induced Hypoglycemia (NICTH) secondary to a Solitary Fibrous Tumor (SFT), which secretes an incompletely processed form of Insulin-like Growth Factor 2 (IGF-2).

Results: A 96-year-old woman was admitted with head trauma due to an accidental fall. During her hospital stay she experienced frequent hypoglycemic episodes. Multiple injections of 33% dextrose and continuous infusion with 10% dextrose were required to maintain normal blood glucose levels. Biochemical analyses revealed hypoinsulinemic hypoglycemia, low C-peptide levels, suppressed insulin-like growth factor-1, normal insulin-like growth factor-2, and an elevated IGF-2:IGF-1 ratio, all consistent with IGF-2 secretion by a non-islet cell tumor.

A contrast-enhanced chest and abdominal CT scans showed a single large pleural mass in the left lower hemithorax measuring 15x14 cm without secondary lesions. Histological analysis of biopsied specimens suggested a solitary fibrous pleural tumor; accordingly, a diagnosis of Doege-Potter syndrome was considered.

Due to extensive tumor burden and the advanced age of the patient, supportive and non-invasive management was chosen. Dexamethasone therapy was started, and while receiving this therapy she was able to discontinue glucose infusion and successfully maintain euglycemia.

Discussion: In the elderly, a sudden and unexplained fall can be the expression of severe hypoglycemia, usually as a complication of insulin therapy or of oral hypoglycemic agents administered to patients with diabetes. However, in patients without diabetes, other causes should be investigated, and the hypothesis of neoplastic diseases should be considered.

Conclusion: In this case report we describe an uncommon cause of paraneoplastic hypoglycemia occurring in the oldest patient with a non-islet cell tumor reported thus far.

Keywords: Doege-potter syndrome, solitary fibrous tumor, hypoglycemia, IGF, fall, lower hemithorax measuring.

Schutt RC, Gordon TA, Bhabhra R, et al. Doege-Potter syndrome presenting with hypoinsulinemic hypoglycemia in a patient with a malignant extrapleural solitary fibrous tumor: A case report. J Med Case Reports 2013; 7: 11.
Nadler WH, Wolfer JA. Hepatogenic hypoglycemia associated with primary liver cell carcinoma. Arch Intern Med (Chic) 1929; 44(5): 700-10.
Doege KW. Fibro-Sarcoma of the Mediastinum. Ann Surg 1930; 92(5): 955-60.
Potter RP. Intrathoracic tumors. Radiology 1930; 14(1): 60-1.
Meng W, Zhu HH, Li H, et al. Solitary fibrous tumors of the pleura with Doege-Potter syndrome: A case report and three-decade review of the literature. BMC Res Notes 2014; 7: 515.
Alharbi MSA. 94-year-old man with recurrent hypoglycemia caused by Non-Islet Cell Tumor Hypoglycemia (NICTH). Int J Health Sci 2014; 8(4): 430-3.
Katz S, Ford AB, Moskowitz RW, et al. Studies of illness in the aged. The index of Adl: A standardized measure of biological and psychosocial function. JAMA 1963; 185: 914-9.
Lawton MP, Brody EM. Assessment of older people: Self-maintaining and instrumental activities of daily living. Gerontologist 1969; 9(3): 179-86.
Bellelli G, Morandi A, Davis DH, et al. Validation of the 4AT, a new instrument for rapid delirium screening: A study in 234 hospitalised older people. Age Ageing 2014; 43(4): 496-502.
Bellelli G, Morandi A, Davis DH, et al. Corrigendum to ‘Validation of the 4AT, a new instrument for rapid delirium screening: A study in 234 hospitalised older people’. Age Ageing 2015; 44(1): 175.
Folstein MF, Folstein SE, McHugh PR. “Mini-mental state”: A practical method for grading the cognitive state of patients for the clinician. J Psychiatr Res 1975; 12(3): 189-98.
Kaiser MJ, Bauer JM, Ramsch C, et al. Frequency of malnutrition in older adults: A multinational perspective using the mini nutritional assessment. J Am Geriatr Soc 2010; 58(9): 1734-8.
Bodnar TW, Acevedo MJ, Pietropaolo M. Management of non-islet-cell tumor hypoglycemia: A clinical review. J Clin Endocrinol Metab 2014; 99(3): 713-22.
Strambu IR, Leonte DG, Bolca CN. Large pleural tumor revealed by severe hypoglycemia: Doege-Potter syndrome. Rom J Morphol Embryol 2017; 58(1): 287-91.
Kantarova D, Sagova I, Stancik M, et al. Hypoglycemia associated with non-islet cell tumors. Neoplasma 2015; 62(6): 841-5.
Daughaday WH, Emanuele MA, Brooks MH, et al. Synthesis and secretion of insulin-like growth factor II by a leiomyosarcoma with associated hypoglycemia. N Engl J Med 1988; 319(22): 1434-40.
Dynkevich Y, Rother KI, Whitford I, et al. Tumors, IGF-2, and hypoglycemia: Insights from the clinic, the laboratory, and the historical archive. Endocr Rev 2013; 34(6): 798-826.
Miraki-Moud F, Grossman AB, Besser M, et al. A rapid method for analyzing serum pro-insulin-like growth factor-II in patients with non-islet cell tumor hypoglycemia. J Clin Endocrinol Metab 2005; 90(7): 3819-23.
Teale JD, Wark G. The effectiveness of different treatment options for non-islet cell tumour hypoglycaemia. Clin Endocrinol 2004; 60(4): 457-60.
Ndzengue A, Deribe Z, Rafal RB, et al. Non-islet cell tumor hypoglycemia associated with uterine leiomyomata. Endocr Pract 2011; 17(4): e109-12.
Maruyama H, Tatsumi M, Kitayama H, et al. A case of gastric cancer with non-islet cell tumor hypoglycemia detected by insulin-like growth factor II. Pathol Int 2010; 60(8): 595-7.
Rose MG, Tallini G, Pollak J, et al. Malignant hypoglycemia associated with a large mesenchymal tumor: Case report and review of the literature. Cancer J Sci Am 1999; 5(1): 48-51.
Morbois-Trabut L, Maillot F, De Widerspach-Thor A, et al. “Big IGF-II”-induced hypoglycemia secondary to gastric adenocarcinoma. Diabetes Metab 2004; 30(3): 276-9.
Silveira LF, Bouloux PM, MacColl GS, et al. Growth hormone therapy for non-islet cell tumor hypoglycemia. Am J Med 2002; 113(3): 255-7.
Teale JD, Blum WF, Marks V. Alleviation of non-islet cell tumour hypoglycaemia by growth hormone therapy is associated with changes in IGF binding protein-3. Ann Clin Biochem 1992; 29(Pt 3): 314-23.
Drake WM, Miraki F, Siddiqi A, et al. Dose-related effects of growth hormone on IGF-I and IGF-binding protein-3 levels in non-islet cell tumour hypoglycaemia. Eur J Endocrinol 1998; 139(5): 532-6.
Bourcigaux N, Arnault-Ouary G, Christol R, et al. Treatment of hypoglycemia using combined glucocorticoid and recombinant human growth hormone in a patient with a metastatic non-islet cell tumor hypoglycemia. Clin Ther 2005; 27(2): 246-51.

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Article Details

Year: 2018
Page: [195 - 200]
Pages: 6
DOI: 10.2174/1874609812666181205142247

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