Angioretikulome des Zentralnervensystems

Zusammenfassung

Angioretikulome des ZNS lassen sich in 3 histologische Typen einteilen: xanthomatöser, spindelzelliger und pleomorph-undifferenzierter Typ. Prognostisch unterscheidet sich der pleomorph-undifferenzierte Typ, der die maligne Variante darstellt, grundsätzlich von den gutartigen xanthomatösen und spindelzelligen Formen.

Entgegen der allgemein vertretenen Ansicht werden Angioretikulome des ZNS auch bei Kindern unter 10 Jahren beobachtet.

Summary

63 vascular tumours of the central nervous system were examined histologically. 11 cases were excluded from the catamnestic investigation because they represented cerebral manifestations of a systemic angiodysplasia. Of 52 angioreticulomas, 41 were localized in the cerebellum. No preference of sex was observed. 6 cases were children of less than 10 years. The angioreticulomas of the cerebellum showed a peak incidence in the third and forth decade.

3 morphologically different types of angioreticulomas can be distinguished. The xanthomatous and the spindle-celled type are benign tumours. In these cases the patients' fate is determined by location and extent of the tumour. 43 cases belonged to these two types. 32 of these cases are still living (30 after more than 5 years, 20 after more than 10 years). Only three have died (two because of malignant kidney tumours two and three years after the operation, and one due to cerebral changes not directly related to the tumour).

The pleomorphocellular type represents a malignant tumour. Of 9 cases of this type, only one is still living (11/2 years after the surgical removal of the tumour).