Erfolgreiche Therapie rezidivierender Hypoglykämien durch operative Entfernung eines malignen Hämangioperizytoms

We report a patient with insulin-like growth factor (IGF)-II-producing hemangiopericytoma with hypoglycemia in whom repeated intra-abdominal recurrences developed over a period of about 10 years and tumor resection was performed four times. A 67-year-old woman was admitted to our hospital in 1995 because of hypoglycemic attacks. In 1985, partial resection of the small bowel had been performed for a 17-cm abdominal tumor of the transverse mesocolon, and the pathological diagnosis was hemangiopericytoma. In 1991, left hemicolectomy had been performed for a mesosigmoidal tumor associated with hypoglycemia. In 1994, hysterectomy, bilateral adnexectomy, and resection of an intrapelvic tumor were performed. The fourth operation was performed in 1996, about 10 years after the first operation. The spleen was removed, together with more than 1500 tumors having a total weight of 1,660 g. The hypoglycemia was ameliorated after each operation. Before this operation, her serum IGF-I level was low, but her IGF-II level was within the normal range; however, the Western immunoblot method showed that most of the IGF-II was high-molecular-weight IGF-II. The tissue IGF-I level was also low, and the IGF-II level was high, suggesting an IGF-II-producing tumor. We suspect that the mechanism of the hypoglycemia in this patient was related to the high-molecular-weight IGF-II produced by the tumor. The patient died in 1997 because of tumor recurrence.

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High‐molecular weight IGF‐2 expression in a haemangiopericytoma associated with hypoglycaemia

Höög

Nordqvist

Hulting

et al. 1997

APMIS

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Spontaneous hypoglycaemia is usually caused by an insulin‐producing islet‐cell tumour of the pancreas. Rarely, it can be caused by non‐islet cell tumours. Most of the tumours are of mesenchymal type, large, and slowly growing. One representative is haemangiopericytoma (HAP). The present report describes a case of a large recurrent retroperitoneal HAP associated with severe hypoglycaemia. Blood serum insulin and proinsulin concentrations were low. By means of acid‐gel chromatography and dot‐blot techniques, an increased amount of a high‐molecular‐weight IGF‐2 peptide was found. By using antigen retrieval procedures, IGF‐2‐immunoreactive tumour cells were found in specimens of the recent tumour recurrence ‐ but not in the original. When the in situ hybridization technique was used it could be shown that IGF‐2 mRNA labelling had already occurred in the original tumour specimen, 11 years before the onset of hypoglycaemic symptoms. These observations confirm the hypothesized hypoglycaemic effects of high‐molecular‐weight (HMW) IGF‐2, but also point to the presence of a prolonged compensation of this effect. A literature review, based on 17 similar cases of haemangiopericytoma with hypoglycaemia, is presented. Our observation and findings in the literature review support the idea that non‐islet‐cell tumour hypoglycaemia is caused by an overproduction of a HMW IGF‐2 peptide. The insulin‐like effect is mediated via non‐specific binding to the insulin receptors. To anticipate patients at risk of developing this kind of hypoglycaemia, the histopathological investigation should include not only immunohistochemical analyses of the presence of IGF‐2 peptide, but also in situ hybridization of the IGF‐2 mRNA expression.

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Erfolgreiche Therapie rezidivierender Hypoglykämien durch operative Entfernung eines malignen Hämangioperizytoms

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Hemangiopericytoma

Hemangiopericytoma

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

High‐molecular weight IGF‐2 expression in a haemangiopericytoma associated with hypoglycaemia

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