title: Surgically cured hypoglycemia secondary to pleural solitary fibrous tumour: case report and update review on the Doege-Potter syndrome.
abstract: The association of paraneoplastic hypoglycemia [Doege-Potter syndrome] and finger clubbing [Pierre-Marie-Bamberg syndrome] with pleural solitary fibrous tumour is rare. We present a previously unpublished but typical example of this rare occurrence together with a detailed updated literature review of previously published cases of pleural SFT discussing the histopathology of SFT; pathophysiology of the hypoglycemia and finger clubbing; treatment and outcome of pleural SFT. The patient, a 57-year-old African male was admitted at our hospital with recurrent episodes of hypoglycemia. He was found to have digital clubbing and decreased breath sounds in the right lower chest but no other significant clinical findings. His insulin level measured during an episode of hypoglycemia was undetectable. Chest radiograph and CT-scan revealed a lobulated mass in the right chest which was diagnosed to be SFT on histology. Surgical excision of the mass resulted in cure of the hypoglycemic episodes and rapid regression of the clubbing. Less than 65 cases of pleural SFT manifesting with hypoglycemia with or without finger-clubbing have been published in the English literature. The mean diameter of these tumours manifesting with hypoglycemia is 20 cm, 54% being benign while 42% were malignant. They predominantly present in the 6th-8th decade, average age of 64 years and a slight male preponderance at 58%. Complete surgical resection remains the most important predictor of clinical outcome in terms of recurrence and metastases, while providing instant cure for the hypoglycemia and rapid resolution of the finger clubbing.
authors.affiliation: Department of Anatomical Pathology, National Health Laboratory Service [NHLS], Johannesburg, South Africa. ahmedkalebi@yahoo.com
authors.forename: Ahmed Y
authors.initials: AY
authors.lastname: Kalebi
authors.forename: Martin J
authors.initials: MJ
authors.lastname: Hale
authors.forename: Michelle L
authors.initials: ML
authors.lastname: Wong
authors.forename: Tessa
authors.initials: T
authors.lastname: Hoffman
authors.forename: Jill
authors.initials: J
authors.lastname: Murray
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001786:Blood Glucose / Q000032:analysis; D002096:C-Peptide / Q000032:analysis; D005260:Female; D006801:Humans; D007003:Hypoglycemia / Q000209:etiology / Q000601:surgery*; D007328:Insulin / Q000032:analysis; D008297:Male; D008875:Middle Aged; D009364:Neoplasm Recurrence, Local; D010005:Osteoarthropathy, Secondary Hypertrophic / Q000175:diagnosis / Q000601:surgery*; D010257:Paraneoplastic Syndromes / Q000209:etiology / Q000601:surgery*; D054363:Solitary Fibrous Tumor, Pleural / Q000150:complications / Q000175:diagnosis / Q000601:surgery*; D016896:Treatment Outcome
chemical_list: D001786:Blood Glucose; D002096:C-Peptide; D007328:Insulin
references.citation: J Assoc Physicians India. 2006 May;54:413-5
references.pmid: 16909744
references.citation: Pathol Int. 2007 Dec;57(12):791-3
references.pmid: 17988280
references.citation: Ann Thorac Surg. 1999 May;67(5):1456-9
references.pmid: 10355431
references.citation: Exp Clin Endocrinol Diabetes. 2000;108(8):515-8
references.pmid: 11149628
references.citation: J Clin Endocrinol Metab. 2007 May;92(5):1600-5
references.pmid: 17299065
references.citation: Eur J Cardiothorac Surg. 1999 Jan;15(1):84-6
references.pmid: 10077378
references.citation: Clin Orthop Relat Res. 2003 Jun;(411):236-44
references.pmid: 12782880
references.citation: Cancer. 1981 Jun 1;47(11):2678-89
references.pmid: 7260861
references.citation: Intern Med. 2006;45(8):525-9
references.pmid: 16702745
references.citation: J Thorac Cardiovasc Surg. 2006 Sep;132(3):713-4
references.pmid: 16935145
references.citation: Med Oncol. 2003;20(4):403-8
references.pmid: 14716039
references.citation: Obstet Gynecol. 1995 May;85(5 Pt 2):810-3
references.pmid: 7724120
references.citation: APMIS. 1997 Jun;105(6):469-82
references.pmid: 9236865
references.citation: Respiration. 2008;75(1):109-12
references.pmid: 17259691
references.citation: Arch Pathol Lab Med. 2004 Apr;128(4):460-2
references.pmid: 15043457
references.citation: J Clin Endocrinol Metab. 1992 Jul;75(1):110-5
references.pmid: 1618998
references.citation: N Engl J Med. 1999 Sep 2;341(10):757-8
references.pmid: 10471466
references.citation: Ann Thorac Surg. 2005 Jan;79(1):303-7
references.pmid: 15620963
references.citation: Cancer. 2002 Feb 15;94(4):1057-68
references.pmid: 11920476
references.citation: J Biol Chem. 1998 Jul 17;273(29):18443-51
references.pmid: 9660813
references.citation: J Endocrinol. 2000 May;165(2):253-60
references.pmid: 10810289
references.citation: J BUON. 2008 Jan-Mar;13(1):117-21
references.pmid: 18404798
references.citation: Interact Cardiovasc Thorac Surg. 2003 Mar;2(1):61-4
references.pmid: 17669989
references.citation: Intern Med. 2001 Apr;40(4):341-4
references.pmid: 11334396
references.citation: Int J Surg. 2008 Aug;6(4):298-301
references.pmid: 18579460
references.citation: Ann Surg. 1930 Nov;92(5):955-60
references.pmid: 17866430
references.citation: J Thorac Oncol. 2006 Jul;1(6):588-90
references.pmid: 17409923
references.citation: J Am Coll Surg. 2006 Sep;203(3):398
references.pmid: 16931312
references.citation: J Hepatobiliary Pancreat Surg. 2007;14(6):595-9
references.pmid: 18040628
references.citation: Diabetologia. 1998 May;41(5):589-94
references.pmid: 9628278
references.citation: Endocr Pathol. 1999 Winter;10(4):291-297
references.pmid: 12114765
references.citation: J Thorac Cardiovasc Surg. 2000 Jan;119(1):185-7
references.pmid: 10612786
references.citation: Am Surg. 2007 May;73(5):478-80
references.pmid: 17521003
references.citation: Proc Natl Acad Sci U S A. 2005 Aug 2;102(31):11047-52
references.pmid: 16040806
references.citation: Chest. 1986 Feb;89(2):289-90
references.pmid: 3943390
references.citation: Eur J Cardiothorac Surg. 2002 Jun;21(6):1087-93
references.pmid: 12048090
references.citation: Thorax. 1991 Dec;46(12):932-3
references.pmid: 1792644
references.citation: Thorac Cardiovasc Surg. 2006 Feb;54(1):65-7
references.pmid: 16485194
references.citation: Eur J Cardiothorac Surg. 1998 Nov;14(5):453-9
references.pmid: 9860200
references.citation: Mt Sinai J Med. 2004 Oct;71(5):344-6
references.pmid: 15543436
references.citation: Cancer Control. 2006 Oct;13(4):264-9
references.pmid: 17075563
references.citation: Am J Surg Pathol. 2008 Nov;32(11):1627-42
references.pmid: 18753943
references.citation: N Engl J Med. 1982 May 13;306(19):1178-9
references.pmid: 7040961
references.citation: Int J Gynecol Pathol. 2005 Jan;24(1):79-84
references.pmid: 15626921
references.citation: Arch Intern Med. 1983 Aug;143(8):1608-10
references.pmid: 6870444
references.citation: Ann Thorac Surg. 2000 Dec;70(6):1808-12
references.pmid: 11156076
references.citation: J Cardiovasc Surg (Torino). 2000 Oct;41(5):781-4
references.pmid: 11149649
references.citation: Spine (Phila Pa 1976). 2008 May 20;33(12):E397-9
references.pmid: 18496336
references.citation: Ann Thorac Surg. 1998 May;65(5):1461-4
references.pmid: 9594894
references.citation: Ann Thorac Surg. 2007 Jul;84(1):292-4
references.pmid: 17588442
references.citation: Magn Reson Imaging. 1997;15(3):377-9
references.pmid: 9201687
references.citation: Chest. 1994 Sep;106(3):965-7
references.pmid: 8082393
references.citation: Eur J Cardiothorac Surg. 2001 Feb;19(2):185-9
references.pmid: 11167110
references.citation: Aust N Z J Med. 1982 Feb;12(1):70-2
references.pmid: 7044359
references.citation: J Clin Endocrinol Metab. 2007 May;92(5):1616
references.pmid: 17483375
references.citation: Int J Dermatol. 2004 Dec;43(12):944-7
references.pmid: 15569027
references.citation: J Ky Med Assoc. 1992 Nov;90(11):557-60
references.pmid: 1474302
references.citation: J Natl Med Assoc. 2004 May;96(5):659-62, 664
references.pmid: 15160981
references.citation: Am J Surg Pathol. 1989 Aug;13(8):640-58
references.pmid: 2665534
references.citation: Eur J Cardiothorac Surg. 2007 May;31(5):955-7
references.pmid: 17317199
references.citation: Int J Clin Oncol. 2006 Dec;11(6):478-81
references.pmid: 17180519
references.citation: Lung Cancer. 1999 Jan;23(1):53-60
references.pmid: 10100146
references.citation: Eur J Cardiothorac Surg. 2007 Sep;32(3):403-8
references.pmid: 17646108
references.citation: Endocr J. 2008 Oct;55(5):905-11
references.pmid: 18552458
references.citation: J Surg Oncol. 2006 Jul 1;94(1):40-4
references.pmid: 16788942
references.citation: South Med J. 1990 Jun;83(6):690-4
references.pmid: 2162571
references.citation: Hum Pathol. 1995 Apr;26(4):440-9
references.pmid: 7705824
references.citation: Anticancer Res. 2000 Nov-Dec;20(6C):4701-6
references.pmid: 11205204
references.citation: Clin Lung Cancer. 2005 Nov;7(3):197-201
references.pmid: 16354315
references.citation: N Engl J Med. 1988 Dec 1;319(22):1434-40
references.pmid: 3185662
references.citation: J Am Acad Dermatol. 2007 Nov;57(5 Suppl):S106-10
references.pmid: 17938018