Insulin-like growth factor II-producing intra-abdominal hemangiopericytoma associated with hypoglycemia

Matsuda, Shinsuke; Usui, Masanobu; Sakurai, Hiroyuki; Suzuki, Hideaki; Ogura, Yuichiro; Shiraishi, Taizo

doi:10.1007/s005350170009

Cited by 13 publications

(7 citation statements)

References 24 publications

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“…Hollmann et al 14 described hemangiopericytomas as often being large and slow-growing, pursuing a course over many years with repeated recurrences and metastases, with hypoglycemia as a late event, in the natural history of the disease. 15 In the present patient, hypoglycemia did not occur until the multiple visceral and muscular metastases grew to be about 5 cm in size. The etiology of NICTH is well documented as attributable to tumor production of insulin-like growth factor II (IGF-II).…”

Section: Discussionmentioning

confidence: 41%

Multiple metastases from a meningeal hemangiopericytoma associated with severe hypoglycemia

et al. 2005

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Hemangiopericytomas are rare vascular tumors that may be associated with profound hypoglycemia. We present correlative imaging findings for a 57-year-old woman who had multiple abdominal and muscular metastases with severe hypoglycemia 11 years after resection of meningeal hemangiopericytoma. The metastases were well-defined masses containing solid and cystic components, and they were extremely hypervascular in the solid component. Those characteristics on imaging associated with hypoglycemia and the past history of meningeal hemangiopericytoma may suggest the diagnosis and be helpful in differentiating the mass from other vascular tumors. Color and power Doppler ultrasound may play a valuable role in characterizing and diagnosing metastasis from hemangiopericytoma. A delay of several years between diagnosis of the primary hemangiopericytoma and the manifestation of metastasis appears to be a special feature of hemangiopericytoma.

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Section: Discussionmentioning

confidence: 41%

Multiple metastases from a meningeal hemangiopericytoma associated with severe hypoglycemia

et al. 2005

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“…Big IGF-2, while not detectable on most commercial assays, can be bioactive [ 11 ] and often underlies the pathogenesis of hypoglycemia [ 5 , 12 ]. Indeed, many IGF-2omas, including hemangiopericytomas, present with normal or even low mature IGF-2 levels [ 13 , 14 ]. Nonetheless, in instances like the present case, measurably high mature IGF-2 levels [ 8 , 15 ] may aid in making the diagnosis and monitoring response to therapy.…”

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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“…Although some tumor regressions have occurred in response to radiotherapy, hemangiopericytomas generally are believed to be radioresistant [ 9 ]. Another interesting features are various paraneoplastic syndromes accompanying this tumor, such es hypoglycemia [ 8 , 12 ], neurofibromatosis or von Recklinghausen disease [ 13 ]. Several mechanisms, such as the secretion of insulin-like substances, insulin-like growth factor II by the tumor, and the hyperutilization of glucose have been proposed as possible causes of tumor-related hypoglycemia.…”

Section: Discussionmentioning

confidence: 99%

Hemangiopericytoma in the Small Intestine: A Case Report

Coha

Krizanovic

Perušinović

et al. 2008

Case Rep Gastroenterol

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Hemangiopericytoma is a rare tumor, mostly composed of pericytes of ramified appearance that are normally found all along the venules and capillaries. An 18-year-old female was admitted on March 17, 1997 to the Department of Infectious Diseases for fever, chills and failure to thrive. Physical examination revealed a palpable tumorous mass in the iliac region on the right. The patient was transferred to the Department of Surgery. Vaginal examination and echotomography of the abdomen and pelvis minor were performed and revealed the presence of an irregular, inhomogeneous tumorous formation of 8 cm in diameter in the pelvis minor, differentially diagnostic right ovary, i.e. acute adnexitis or perityphlitic tumor. After that, small intestine resection, tumor excision in toto, and appendectomy were performed on April 16, 1997. Pathohistological diagnosis was hemangiopericytoma and chronic appendicitis. The biological behavior of this tumor is difficult to predict on the basis of clinical and morphological parameters. In our patient, local tumor excision was not followed by adjuvant therapy. After ten years, the patient is well, healthy and a mother of two healthy children.

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Insulin-like growth factor II-producing intra-abdominal hemangiopericytoma associated with hypoglycemia

Cited by 13 publications

References 24 publications

Multiple metastases from a meningeal hemangiopericytoma associated with severe hypoglycemia

Multiple metastases from a meningeal hemangiopericytoma associated with severe hypoglycemia

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

Hemangiopericytoma in the Small Intestine: A Case Report

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