Glucocorticoid Therapy Ameliorated Hypoglycemia in Insulin-like Growth Factor-II-producing Solitary Fibrous Tumor

Tsuro, Kiminori; Kojima, Hideyuki; Okamoto, Shingo; Yoshiji, Hitoshi; Fujimoto, Masao; Uemura, Mamoru; Yoshikawa, Masahide; Nakamura, Takashi; Kou, Saihou; Nakajima, Yoshiyuki; Fukui, Hiroshi

doi:10.2169/internalmedicine.45.1611

Cited by 48 publications

(29 citation statements)

References 20 publications

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“…[10] Western blot analysis of serum IGF-II revealed the presence of IGF-II species with increased molecular weights, and decreased levels of normal IGF-II. [11,12] …”

Section: Clinical Features Of Doege–potter Syndromementioning

confidence: 99%

Doege–Potter syndrome

Han¹,

Zhang²,

Xing-bin³

et al. 2017

Medicine

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Rationale:We reviewed 76 published cases of Doege–Potter syndrome, and non-islet cell tumor hypoglycemia (NICTH) secondary to a solitary fibrous tumor (SFT) between 1989 and 2016, to study disease pathogenesis, diagnosis, and treatment of this rare paraneoplastic disease. Further, we report 1 new case of a patient presenting with Doege–Potter syndrome.Patients concerns:The tumors originated from the pleural cavity, lung, pelvis, liver, retroperitoneum, kidney, mediastinal, the sella, uterus, bladder, intestine, mandibular, and the thigh. The most common location was the pleural cavity (left 12 cases and right 28 cases). Moreover, 28/71 (39.4%) were benign and 43/71 (60.6%) were malignant. SFTs with NICTH were more likely to be malignant and present at a higher rate than previously published (5%–10.4%). The malignancy rate of extrathoracic SFTs was higher than that of thoracic SFTs, 20 (66.7%) as compared with 23 (56.1%). Age of onset varied from 24 to 85 years (mean 59 years), with 47 males and 28 females, and gender unavailable for 1 case. When comparing clinical characteristics of patients with benign as compared malignant tumors, no significant differences in the age of onset, gender, or size of tumor were seen. Among 15/19 cases, the insulin-like growth factor II (IGF-II)/IGF-I ration was >10.0. Complete tumor resection remained the only definitive treatment.Outcomes and lessens:Glucocorticoids dose-dependently reduce the frequency and severity of hypoglycemic episodes. Low doses of prednisone were ineffective at relieving hypoglycemia. The effect of neoadjuvant treatment, consisting of chemoradiation, and consecutive selective embolization of vessels feeding the tumor were not identified.

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“…[10] Western blot analysis of serum IGF-II revealed the presence of IGF-II species with increased molecular weights, and decreased levels of normal IGF-II. [11,12] …”

Section: Clinical Features Of Doege–potter Syndromementioning

confidence: 99%

Doege–Potter syndrome

Han¹,

Zhang²,

Xing-bin³

et al. 2017

Medicine

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“…However, it mainly occurs in patients with solid tumours of mesenchymal and epithelial origin, but rarely also in patients with tumours of haematopoietic and neuroendocrine origin (Table 2; Zapf 1993, Frystyk et al 1998, Fukuda et al 2006, Tsuro et al 2006. In general, the mesenchymal tumours have in common that they are well differentiated and slowly growing, although many usually weigh between 2 and 4 kg at diagnosis.…”

Section: Incidence and Tumour Typesmentioning

confidence: 99%

Non-islet cell tumour-induced hypoglycaemia: a review of the literature including two new cases

Groot¹,

Rikhof²,

Doorn³

et al. 2007

Endocrine Related Cancer

201

326

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This review focuses on the tumour types and symptoms associated with non-islet cell tumourinduced hypoglycaemia (NICTH) as well as the pathogenesis, diagnosis and treatment of this rare paraneoplastic phenomenon. In addition, we report two illustrative cases of patients suffering from NICTH caused by a solid fibrous tumour and a haemangiopericytoma respectively. In the first case, NICTH resolved following complete resection of the tumour, but in the second case the patient needed long-term treatment aimed at controlling hypoglycaemia because of nonresectable metastases. Many tumour types have been associated with NICTH. The crucial event in the development of NICTH seems to be overexpression of the IGF-II gene by the tumour. NICTH is characterised by recurrent fasting hypoglycaemia and is associated with the secretion of incompletely processed precursors of IGF-II ('big'-IGF-II) by the tumour. This induces dramatic secondary changes in the circulating levels of insulin, GH, IGF-I and IGF-binding proteins, resulting in an insulin-like hypoglycaemic activity of 'big'-IGF-II.

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“…Glucocorticosteroids seem to be the most effective symptomatic treatment. They stimulate gluconeogenesis and inhibit the big IGF2 tumor production (8). This effect is dose dependent and reversible if doses are below a critical level (9).…”

Section: Discussionmentioning

confidence: 99%

Paraneoplastic hypoglycemia in a patient with a malignant solitary fibrous tumor

Mohammedi¹,

Khalil²,

Olivier³

et al. 2014

Endocrinology, Diabetes &Amp; Metabolism Case Reports

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SummaryHypoglycemia is a common medical emergency. It is the most frequent complication induced by anti-diabetic treatment. However, it can be observed in other conditions unrelated to diabetes such as insulinoma, autoimmune disorders, and neoplasia. Herein, we report the case of a rare cause of severe and recurrent hypoglycemia in a 77-year-old woman with a malignant solitary fibrous tumor (MSFT). A 77-year-old woman was admitted to the emergency department for loss of consciousness induced by severe hypoglycemia. Her standard laboratory findings were unremarkable. HbA1c, albumin, renal, liver, thyroid, and adrenal function tests were normal. Cerebral CT scan was also normal. At the time of confirmed hypoglycemia, the serum level of insulin and C-peptide was low. On the basis of the past medical history and the absence of other comment etiologies, a paraneoplastic cause was suspected. Thus, the diagnosis of a non-islet cell tumor-induced hypoglycemia (NICTH) was established by the presence of incompletely processed precursors of IGF2 (big IGF2) in plasma electrophoresis. However, the IGF1 level was low. Therapy with corticosteroids improved hypoglycemia and clinical symptoms. NICTH is a rare cause of hypoglycemia. It should be considered in patients with mesenchymal or malignant epithelial tumors suffering from recurrent episodes of hypoglycemia. The diagnosis will be established in the case of low serum insulin concentrations and elevated levels of big IGF2. Treatment with corticosteroids, GH, or both can improve hypoglycemic symptoms and restore plasma glucose to normal levels.Learning points NICTH is a very rare condition that should be considered in patients known to have mesenchymal or malignant epithelial tumors and suffering from recurrent episodes of hypoglycemia.The diagnosis of an NICTH is established on the basis of the hypoinsulinemic hypoglycemia, the MSFT history, and the presence of paraneoplastic secretion of IGF1 or an immature form of IGF2.Treatment with corticosteroids, GH, or both can improve hypoglycemic symptoms and restore plasma glucose to normal levels in NICTH.

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Glucocorticoid Therapy Ameliorated Hypoglycemia in Insulin-like Growth Factor-II-producing Solitary Fibrous Tumor

Abstract: Abstract

Cited by 48 publications

References 20 publications

Doege–Potter syndrome

Doege–Potter syndrome

Non-islet cell tumour-induced hypoglycaemia: a review of the literature including two new cases

Paraneoplastic hypoglycemia in a patient with a malignant solitary fibrous tumor

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