Doege–Potter Syndrome

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.

Non-Islet Cell Tumor Hypoglycemia Caused by Recurrent Pelvic Solitary Fibrous Tumor

“…The definitive diagnosis of SFT is based on histologic identification of spindle cell tumor with a distinctive fibroblastic morphology, comprising a patternless pattern of spindle cells, arranged as areas of alternating cellularity and hypocellular collagenous stroma, as well as CD34 (most cases), CD99, and/or BCL-2 expression [ 13 - 14 ]. The criteria for malignancy in SFT are increased cellularity, nuclear atypia, infiltrative growth, and mitotic count of more than four per 10 high-power fields [ 13 ]. Ki-67, a marker of cell proliferation, can be used to stratify lesions, and a rate higher than 12% is suggested as a cut-off level for malignancy [ 15 - 16 ].…”

“…In the case reported, Ki-67 <10% was verified, which indicates less aggressive behavior. The postoperative risk of recurrence or metastasis is seen to increase with a tumor size of >10 cm or if a malignant component is detected [ 13 ], therefore, considering the size of our patient's lesion, there is a high probability of relapse.…”

Doege-potter syndrome: a report of a histologically benign but clinically malignant case

“…To make a histological diagnosis, an optical microscope and immunohistochemical staining are used. Histological sections show diverse shades of grey and a spiral, solid area mixed with soft phlegmatic components and spindle cells with patternless patterns, collagenous stroma with cellularity and hypocellularity tissue [ 10 , 11 ] According to the standards suggested by England et al [ 11 ], malignancy is determined in patients with invasive growth with an unclear boundary, high cell density, 4 or more mitoses per 10 high magnification fields of view, pleomorphism, and the presence of bleeding and necrosis. Cell necrosis and dense tissues are additional signs of a malignant tumor.…”

“…For patients without symptoms, a plain radiograph is sufficient for follow-up. Reports on the effects of chemotherapy and irradiation therapy are very rare and limited [ 9 , 10 , 12 ]. As a result, operative management is the only solution for radical treatment.…”

Hypoglycemia as a Symptom of Neoplastic Disease, with a focus on Insulin-like Growth Factors Producing Tumors

“…Nonislet cell tumor hypoglycemia, also called Doege-Potter syndrome , is a rare disease with an estimated incidence of one case per million 27, 28. NICTH, as excellently reviewed by de Groot et al , is thought to be rather a fasting hypoglycemia characterized by diminished hepatic glucose production resulting from the inhibition of glycogenolysis and gluconeogenesis; diminished lipolysis in adipose tissue resulting in low levels of serum-free fatty acids and increased peripheral glucose consumption 2.…”