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Case Reports
. 2015 Oct;97(7):e105-7.
doi: 10.1308/rcsann.2015.0023.

Doege-Potter Syndrome

N Ahluwalia  1 , R Attia  1 , A Green  1 , P Cane  1 , T Routledge  1
Affiliations
Case Reports

Doege-Potter Syndrome

N Ahluwalia et al. Ann R Coll Surg Engl. 2015 Oct.

Abstract

Doege-Potter syndrome is a rare paraneoplastic syndrome presenting as a hypoinsulinaemic hypoglycaemia from the ectopic secretion of a prohormone of insulin-like growth factor II (IGF-II) from a solitary fibrous tumour. Surgical resection is curative in the majority of cases. If, however, the diagnosis is not suspected and treatment is delayed, it can lead to hypoxic cerebral injury or death. The underlying tumour can be a benign or malignant pleural tumour but may be present in extrapleural sites. For a diagnosis of Doege-Potter syndrome, symptoms attributable to hypoglycaemia and low blood glucose levels should be present along with the secretion of prohormone IGF-II. We report a case of severe hypoglycaemia in a 76-year-old inpatient admitted for resection of a recurrent left-sided pleural tumour. Investigation revealed true hypoglycaemia and Doege-Potter syndrome was diagnosed. The tumour was completely resected and the patient made a full recovery with no further hypoglycaemic episodes.

Keywords: Fibroma; Hypoglycaemia; Insulin-like growth factor II; Thoracic surgery.

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Figures

Figure 1
Figure 1
Serial computed tomography of the patient’s chest showing the left pleural fibroma
Figure 2
Figure 2
Preoperative magnetic resonance imaging of the thorax delineating the boundaries of the pleural tumour: Sagittal (A), coronal (B) and axial (C) views show the tumour invading the anterior aspect of the lung down to the hilum and pericardium (but not into the pericardial space).
Figure 3
Figure 3
Histological appearance of solitary fibrous tumour (SFT) with haematoxylin and eosin staining of tumour sections: the typical patternless pattern of SFTs (4x magnification) (A) and plump spindle cells arranged in short fascicles separated by thick collagen bundles (10x magnification) (B)
Figure 4
Figure 4
Immunohistochemical staining for CD34: positive staining seen in the main tumour (20x magnification) (A); a small second tumour showing diffuse strong positivity (4x magnification) (B)
See this image and copyright information in PMC

References

    1. Wagner E. Das tuberkelähnliche Lymphadenom. (Der cytogene oder reticulirte Tuberkel). Arch Heilk 1870; : 497.
    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; : 515. - PMC - PubMed
    1. England DM, Hochholzer L, McCarthy MJ. Localized benign and malignant fibrous tumors of the pleura. A clinicopathologic review of 223 cases. Am J Surg Pathol 1989; : 640–658. - PubMed
    1. Wignall OJ, Moskovic EC, Thway K, Thomas JM. Solitary fibrous tumors of the soft tissues: review of the imaging and clinical features with histopathologic correlation. Am J Roentgenol 2010; : W55–W62. - PubMed
    1. Hajdu M, Singer S, Maki RG, et al. . IGF2 over-expression in solitary fibrous tumours is independent of anatomical location and is related to loss of imprinting. J Pathol 2010; : 300–307. - PMC - PubMed

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