Ultrastructure of hemangiopericytoma associated with paraneoplastic hypoglycemia

Abstract

A recurred and metastasized hemangiopericytoma of menigeal origin caused a terminal hypoglycemia syndrome in a 40 year old man. The disease had been observed over a period of 10 years. The total weight of the tumour metastases was 1800 g. Electron microscopical examination of the tumour cells revealed, in particular, a markedly developed ergastoplasm, prominent Golgi complexes surrounded by many microvesicles, round to ovoid electron dense bodies and fine fibrillar structures. Furthermore, large deposits of basement membrane-resembling material were found in the pericapillary and intercellular spaces. On the basis of the structural characteristics which indicate distinct synthesizing capacity of the cells, an excessive glucose consumption by the tumour is suggested to be an important factor in the pathogenesis of tumour hypoglycemia. The question whether the ultrastructure of the tumour also exhibits secretory processes, which may be related to the release of a presumed inhibitor of hepatic gluconeogenesis and/or glycogenolysis, remains open. Typical granules as in polypeptide hormon secreting cells were not observed. The possibility that the demonstrated electron dense membrane limited bodies represent atypical secretory granules is discussed.

Malignant solitary fibrous tumor of the meninges

“…It is the present consensus that small foci of HPClike tissue can be accepted in SFT, but the histological overlap is not yet clear. Moreover, while not observed in the present case, not only intrathoracic but also extrathoracic HPC [3,9] as well as SFT [12] may be sources of hypoglycemia due to the production of an insulin-like growth factor. Intracranial HPCs are reported to have a relatively high rate of recurrence (60-83%) and metastasis (23-28%) [18,30] and it is difficult to predict malignant potential from cell proliferation indices such as Ki67 or PCNA [32].…”

Ultrastructure of hypertrophic hemangioma

“…The ultrastruetural examination of one case of hypertrophic hemangioma revealed that the lesion consisted of three cell types; the endothelial cells, the pericytes and the fusiform intervascular cells. Pericytes are considered multipotential cells like Schwann cells and primitive fibrous histiocytie cells, and can be the precursors of a number of cells such as smooth muscle cells, endothelial cells, rhabdomyoblasts, fibroblasts and phagoeytie cells (Murad et al 1968, Kuhn & Rosai 1969, Bommer et al 1976, Eimoto 1977, Waldo et al 1977, Angervall et al 1978. The fusiform intervascular cells in the present case which contained many lysosomes could be phagoeytie fibroblasts.…”

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

“…In five of these publications it is reported that the authors had looked for secretory granules in the neoplastic parenchymal cells (1, 2, 5 , 7, 38), i.e., an important criterion for neuroendocrine differentiation. However, only in 2 of the 17 case reports were any granule-like structures present (1,7). In the neoplastic cells of our HAP, no signs of neuroendocrine differentiation were observed, i.e., no IR with antibodies to chromogranin A, synaptophysin, NSE, or NFF?…”

“…In 7 of the 17 reviewed cases, an electronmicroscopic investigation supported the histopathological HAP diagnosis (1,2,5,7,38,39,54). In five of these publications it is reported that the authors had looked for secretory granules in the neoplastic parenchymal cells (1, 2, 5 , 7, 38), i.e., an important criterion for neuroendocrine differentiation.…”

“…In the review, it was observed that the IGF-2-induced NICTH symptoms obviously did not differ from those caused by regular hyperinsulinism; this also includes the effects on the CNS (2,5,7,11,13,21,28,37,38,39,46,47,54). Prompt relief was obtained after food intake.…”