gms | German Medical Science

64. Jahrestagung der Deutschen Gesellschaft für Neurochirurgie (DGNC)

Deutsche Gesellschaft für Neurochirurgie (DGNC) e. V.

26. - 29. Mai 2013, Düsseldorf

DIA/DIG: Rare low-grade neoplasms with very different behaviour patterns

Meeting Abstract

  • Heidi Baechli - Neurochirurgische Klinik, Universitätsklinikum Heidelberg; Universitätsspital Basel, Schweiz
  • Christopher Beynon - Neurochirurgische Klinik, Universitätsklinikum Heidelberg
  • Andreas von Deimling - Abteilung für Neuropathologie, Universitätsklinikum Heidelberg
  • F. Sahm - Abteilung für Neuropathologie, Universitätsklinikum Heidelberg
  • Stefan M. Pfister - Abteilung Pädiatrische Neuroonkologie, DKFZ, Heidelberg
  • Olaf Witt - Abteilung für Pädiatrische Onkologie, Universitätsklinikum Heidelberg
  • Andreas Unterberg - Neurochirurgische Klinik, Universitätsklinikum Heidelberg

Deutsche Gesellschaft für Neurochirurgie. 64. Jahrestagung der Deutschen Gesellschaft für Neurochirurgie (DGNC). Düsseldorf, 26.-29.05.2013. Düsseldorf: German Medical Science GMS Publishing House; 2013. DocMI.15.10

doi: 10.3205/13dgnc415, urn:nbn:de:0183-13dgnc4153

Veröffentlicht: 21. Mai 2013

© 2013 Baechli et al.
Dieser Artikel ist ein Open Access-Artikel und steht unter den Creative Commons Lizenzbedingungen ( Er darf vervielfältigt, verbreitet und öffentlich zugänglich gemacht werden, vorausgesetzt dass Autor und Quelle genannt werden.



Objective: Desmoplastic infantile astrocytomas/gangliogliomas (DAI/DIG) are extremely rare glioneural tumours and are considered benign tumours (World Health Organization [WHO] grade one). In the majority of cases, they occur in infants younger than two years of age. Superficial location and complete surgical excision are associated with an excellent clinical outcome. In contrast, in cases of incomplete resection, these tumours seem to have a heterogenous biological behaviour rangig from complete or partial regression up to rapid tumour progression and occurrence of metastases

Method: We have retrospectively reviewed the medical records of four patients with DAI/DAG treated at our institution. Characteristics in regard to clinical symptoms, diagnostic imaging, surgical treatment, and follow-up treatment were analysed.

Results: A total of 4 patients (age range: 3 months–2 years; male: 2 female: 2) with DAI/DAG have been treated at our institution. Tumours were located in the superficial cortex in three cases, while one tumour was located on the skull base. In two cases of superficial tumour localization, complete surgical resection was carried out. Patients received no further specific treatment and follow-up examinations of up to 14 years after surgery revealed no tumour recurrence. In one case of partial resection of a superficially located tumour, complete spontaneous regression was observed after five years although no tumour-specific treatment was carried out. Partial resection of the skull-based DIA/DIG was followed by chemotherapy after genetic examination had revealed a BRAFV600E mutation and residual tumour had shown rapid progression shortly after surgical treatment.

Conclusions: Our reported cases support the evidence of the literature that complete surgical resection is the treatment of choice in this rare entity. In cases of partial surgical resection, malignant courses have been described and therefore, regular follow-up is mandatory in these patients. As these tumours may show high mitotic activity and cancer-associated gene mutations, further research into biologic pathways is warranted. Tumour-specific chemotherapy may promote a favourable clinical course in cases of partial resection.