Hypoglycemia Observed on Continuous Glucose Monitoring Associated With IGF-2-Producing Solitary Fibrous Tumor

Otake, Sonoko; Kikkawa, Takuma; Takizawa, Miho; Oya, Junko; Hanai, Ko; Tanaka, Nobue; Miura, Junnosuke; Izumi, Fujio; Kanzaki, Masato; Sawada, Tatsuo; Hizuka, Naomi; Onuki, Takamasa; Uchigata, Yasuko

doi:10.1210/jc.2014-4534

Cited by 12 publications

(10 citation statements)

References 44 publications

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“…Glucose monitoring with CGMS was critical in the current case to alert the patient of overnight hypoglycemia and to titrate doses of anti-hypoglycemic agents. Increasingly employed in the American type 1 diabetic population, CGMS has recently been used in the management of IGF-2-mediated hypoglycemia [ 24 ]. It is important to note that acetaminophen interferes with the CGMS glucose sensor and is therefore contraindicated for patients on these systems.…”

Section: Discussionmentioning

confidence: 99%

Subcutaneous glucagon infusion and continuous glucose monitoring enable effective management of hypoglycemia in a patient with IGF-2-producing hemangiopericytoma

Buras

Weatherup

Wyckoff

2018

Clin Diabetes Endocrinol

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BackgroundEctopic insulin-like growth factor (IGF)-2 production is a rare complication of an array of epithelial and mesenchymal tumors, and can clinically manifest as life-threatening hypoglycemia.Case presentationA 49-year-old woman with 13-year history of metastatic hemangiopericytoma, previously treated with multiple rounds of chemotherapy and palliative radiation, presented to the emergency department after a hypoglycemic seizure. On arrival, glucose was 18 mg/dL (1.0 mmol/L) and required continuous dextrose infusion for maintenance within normal limits. Insulin was <2.0 μU/mL, C-peptide 0.1 ng/mL, and beta-hydroxybutyrate <0.2 mmol/L. Random cortisol was 21 μg/dL; sulfonylurea screen, and insulin antibodies were negative. IGF-2 level was 1320 ng/mL; IGF-1 was within normal limits and IGF binding protein (BP)-3 suppressed. Dexamethasone, started at 6 mg twice daily, allowed discontinuation of the glucose infusion. Given concern for nocturnal hypoglycemia, and patient interest in steroid-sparing anti-hypoglycemic regimen, she was also started on overnight continuous subcutaneous glucagon infusion via insulin pump. She was discharged with instructions to maintain a diet high in complex carbohydrates during the day, while utilizing glucagon pump at night. She was also started on continuous glucose monitoring system (CGMS) with an alarm to warn of hypoglycemia. Glucagon infusion rate was later titrated based on CGMS readings. Abdominal CT revealed increasing size of a right upper quadrant mass not previously subjected to radiotherapy. After radiation to this area, hypoglycemia improved, allowing further glucagon titration. In parallel, IGF-2 level declined to 380 ng/mL.ConclusionsEctopic IGF-2 production is a rare but often fatal complication of many cancers, and should be considered on the differential diagnosis in patients with malignancy and unexplained hypoglycemia. Once hypoglycemia is diagnosed, patients often have end-stage disease. While treatment of the causative tumor is the only definitive intervention, anti-hypoglycemia therapy is a life-saving, temporizing measure. In this case, the patient attained euglycemia and survived 3 months after presentation before ultimately succumbing to other malignancy-related complications. Given efficacy in management of hypoglycemia while awaiting definitive tumor-directed therapy, we submit nighttime subcutaneous glucagon infusion and CGMS are valuable additions to the physician’s armamentarium in managing this condition.

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Section: Discussionmentioning

confidence: 99%

Subcutaneous glucagon infusion and continuous glucose monitoring enable effective management of hypoglycemia in a patient with IGF-2-producing hemangiopericytoma

Buras

Weatherup

Wyckoff

2018

Clin Diabetes Endocrinol

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“…Moreover, SFTs can cause paraneoplastic syndromes and mainly hypoglycemia, which is thought to arise due to the production of Insulin like Growth Factor-2 (IGF-2) from the tumor. Such a condition may be the presenting symptom for these cases [ 10 ]. When complete resection is feasible these syndromes subside.…”

Section: Discussionmentioning

confidence: 99%

Retroperitoneal Solitary Fibrous Tumor: A “Patternless” Tumor

Myoteri

Dellaportas

Nastos

et al. 2017

Case Reports in Oncological Medicine

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Introduction Solitary fibrous tumor is a rare type of mesenchymal, spindle-cell tumor reported mostly in the pleura. Retroperitoneal occurrence is rare and histopathological diagnosis is challenging. Case Presentation A 55-year-old woman with nonspecific abdominal pain was found to have a retroperitoneal/pelvic mass adjacent to the upper rectum. The patient underwent surgical resection in clear margins of this pelvic tumor, entering the total mesenteric excision surgical plane. Final histopathology revealed a solitary fibrous tumor and the case is presented herein. Discussion Solitary fibrous tumor in the retroperitoneum is rarely found in the literature and to the best of our knowledge less than a hundred cases are described so far. Histopathological diagnosis is mostly based on a “patternless pattern” on microscopic examination, which is a storiform arrangement of spindle cells combined with a “hemangiopericytoma-like appearance” and increased vascularity of the lesion. Surgery is the mainstay of treatment and recurrence rates are generally low.

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“…These mesenchymal or epithelial tumors secrete IGF-2 and its precursors in excess because of a loss of ICR1 imprinting on 11p15, causing hypoglycemia and the suppression of insulin, IGF-1, and GH [21]. With respect to IGF-2, this oncological disorder seems to be complementary to the IGF-2 deficiency caused by genomic mutations of the paternal IGF2 .…”

Section: Discussionmentioning

confidence: 99%

The Diagnostic Value of IGF-2 and the IGF/IGFBP-3 System in Silver-Russell Syndrome

et al. 2017

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Background/Aims: Recently, we have described a family of 4 members presenting with intrauterine and postnatal growth failure, low IGF-2 levels, and signs of Silver-Russell syndrome (SRS) who carried a genomic IGF2 mutation. Here, we assess the value of IGF-2 in relation to SRS. Methods: We collected data from 48 SRS children and 48 short children born small for gestational age (SGA) seen at our center. The SRS children were 4.6 ± 2.0 years of age, and the SGA children were 4.8 ± 1.8 years of age. All patients were prepubertal and growth hormone naive. An 11p15 ICR1 loss of methylation (11p15LOM) was present in 22, maternal uniparental disomy of chromosome 7 (upd(7)mat) in 7, and IGF2 genomic mutation (IGF2mut) in 3 patients. Growth factors were measured by in-house radioimmunoassays. Results: The median IGF-2 standard deviation scores (SDSs) were: IGF2mut –1.75, upd(7)mat –1.69, nonsyndromic SGA –1.52, 11p15LOM –0.61, and clinical (tested negative) –0.55. The median IGF-2:IGF-1 concentration ratio was 2.57 in IGF2mut compared to 5.44 in 11p15LOM (p = 0.036), 7.84 in clinical, and 7.98 in upd(7)mat. Upd(7)mat patients had significantly lower IGF-1 and IGFBP-3 SDSs than patients with 11p15LOM (p ≤ 0.002). Conclusion: Serum IGF-2 in combination with IGF-1 and IGFBP-3 can add to the clinical signs of SRS patients and help to perform targeted genetic testing. Further studies are needed.

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Hypoglycemia Observed on Continuous Glucose Monitoring Associated With IGF-2-Producing Solitary Fibrous Tumor

Cited by 12 publications

References 44 publications

Subcutaneous glucagon infusion and continuous glucose monitoring enable effective management of hypoglycemia in a patient with IGF-2-producing hemangiopericytoma

Subcutaneous glucagon infusion and continuous glucose monitoring enable effective management of hypoglycemia in a patient with IGF-2-producing hemangiopericytoma

Retroperitoneal Solitary Fibrous Tumor: A “Patternless” Tumor

The Diagnostic Value of IGF-2 and the IGF/IGFBP-3 System in Silver-Russell Syndrome

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