China
Africa
Explore chapters and articles related to this topic
Primary retroperitoneal tumours
Published in Anju Sahdev, Sarah J. Vinnicombe, Husband & Reznek's Imaging in Oncology, 2020
Isaac R Francis, Richard H Cohan, William R Burns
Solitary fibrous tumours (SFTs) are rare spindle cell neoplasms. These were previously classified as hemangiopericytomas and/or giant cell angiofibromas. These tumours are CD34 positive and overexpress the STAT6 protein. The majority of these tumours are benign, but up to 20% may demonstrate aggressive malignant behaviour. While SFTs most commonly arise from the pleura, the most common extrapleural site is the retroperitoneum (2,6,7). Fewer than 5% of patients may present with hypoglycaemia—Doege–Potter syndrome (41).
Malignant Solitary Fibrous Tumor of the Pleura Associated with a Paraneoplastic Hypoglycemia
Published in Wickii T. Vigneswaran, Thoracic Surgery, 2019
Evgeny V. Arshava, Adnan Al Ayoubi, Kalpaj R. Parekh
Due to intractable hypoglycemia, the patient required intravenous therapy with 10% dextrose with resolution of the mental status changes. An endocrine workup was performed with the following serum levels found: insulin 0.1 units/mL (reference range [RR] 1.9–23), proinsulin 1.6 pMole/L (RR 0–10), C-peptide 0.21 ng/mL (RR 0.78–5.19), IGF-1 159 ng/mL (RR 36–215), and IGF-2 670 ng/mL (ref range 333–967). The patient’s hypoglycemia was felt to be secondary to a noninsulin-mediated paraneoplastic Doege–Potter syndrome.
Carbohydrate metabolism
Published in Martin Andrew Crook, Clinical Biochemistry & Metabolic Medicine, 2013
Some tumours release proinsulin, which can also be assayed. Imaging will also be necessary, such as magnetic resonance imaging or computerized tomography scanning, to localize the tumour and to help exclude MEN syndrome (see Chapter 24). If both the plasma insulin and C-peptide concentrations are suppressed, the hypoglycaemia (hypoinsulinaemic hypoglycaemia) can then be divided into non-ketotic and ketotic forms. Hypoglycaemia with low plasma ketone concentrations, that is, β-hydroxybutyrate less than 600 μmol/L, is suggestive of increased insulin or IGF-1 activity such as may occur in non-islet cell tumour hypoglycaemia (NICTH). In NICTH, there is often increased IGF-2 secretion. Some spindle cell tumours, for example of the thorax, may release a variant ‘big-IGF-2’ resulting in hypoglycaemia (Doege–Potter syndrome).
Doege-Potter syndrome in a patient with a giant abdominal solitary fibrous tumor: a case report and review of the literature
Published in Acta Clinica Belgica, 2023
Joris Rötgens, Bruno Lapauw, Guy T’Sjoen
Doege-Potter syndrome is a rare paraneoplastic phenomenon in which hypoinsulinemic hypoglycemia occurs secondary to the excessive secretion of a high-molecular weight prohormone form of insulin-like growth factor II (pro-IGF-II), also called ‘big’ IGF-II, from a solitary fibrous tumor (SFT). SFTs are rare soft tissue lesions of mesenchymal origin, which can occur in a wide variety of anatomical locations. Although less frequent, many other tumors have also been reported to cause non-islet cell tumor hypoglycemia (NICTH) due to the secretion of insulin-like growth factors (for example gastrointestinal tumors, Wilms’ tumor, lymphoma, adrenocortical carcinoma, pheochromocytoma). Tumors producing IGF-II are also called IGF-II-oma. Other potential mechanisms of NICTH are the production of autoantibodies against insulin or the insulin receptor (both extremely rare) and extensive tumor burden with destruction of the liver or adrenal glands [3].