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01-05-2013 | Original article

What’s in a name?

Intracranial peripheral primitive neuroectodermal tumors and CNS primitive neuroectodermal tumors are not the same

Authors: K. Müller, MD, B. Diez, A. Muggeri, T. Pietsch, C. Friedrich, S. Rutkowski, K. von Hoff, A.O. von Bueren, I. Zwiener, F. Bruns

Published in: Strahlentherapie und Onkologie | Issue 5/2013

Abstract

Background

Intracranial peripheral primitive neuroectodermal tumors (P-PNET) are extremely rare. They can be easily misdiagnosed as central nervous system primitive neuroectodermal tumors (CNS-PNET) or meningiomas. Little is known about the optimal treatment and prognosis of these tumors.

Patients and methods

We evaluated the treatment and outcome of 17 patients with intracranial, nonmetastatic, genetically confirmed P-PNET. Three patients were treated at our institutions. Thirteen other cases providing sufficient treatment and follow-up information were extracted from the literature.

Results

The median age at diagnosis was 17 years. All patients underwent initial surgery. Complete resection was achieved in 9 of the 17 cases (53 %). Combined adjuvant treatment consisting of radiotherapy (focal, n = 10; craniospinal, n = 1) and chemotherapy was administered to 11 of the 17 patients (59 %). The median follow-up time was 1.4 years. In 8 of the 17 patients (47 %), the disease progressed; 4 of the 17 patients (24 %) died. The 2-year progression-free and overall survival rates were 64 % and 76 %, respectively.

Conclusion

The differential diagnosis for intracranial, meningeal-based, small, round-cell tumors should include P-PNET. It is highly probable that complete resection has a positive impact on survival—as previously reported for extracranial P–PNET—but this cannot be shown by our data. Intensive adjuvant treatment consisting of radiotherapy and chemotherapy seems to be essential. A statistically grounded recommendation for the appropriate target volume and radiation dose is not yet possible. However, in most case reports of primary intracranial P-PNET published to date, patients were treated with focal irradiation. The optimal chemotherapy regimen has yet to be established, with both the Ewing tumor and CNS-PNET protocols being promising candidates for effective treatment.

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Title: What’s in a name?
Intracranial peripheral primitive neuroectodermal tumors and CNS primitive neuroectodermal tumors are not the same
Authors: K. Müller, MD
B. Diez
A. Muggeri
T. Pietsch
C. Friedrich
S. Rutkowski
K. von Hoff
A.O. von Bueren
I. Zwiener
F. Bruns
Publication date: 01-05-2013
Publisher: Springer-Verlag
Published in: Strahlentherapie und Onkologie / Issue 5/2013
Print ISSN: 0179-7158
Electronic ISSN: 1439-099X
DOI: https://doi.org/10.1007/s00066-013-0315-4

At a glance: The ONWARDS insulin icodec trials

Springer Medicine

What’s in a name?

Intracranial peripheral primitive neuroectodermal tumors and CNS primitive neuroectodermal tumors are not the same

Abstract

Background

Patients and methods

Results

Conclusion

At a glance: The ONWARDS insulin icodec trials

Springer Medicine

Abstract

Background

Patients and methods

Results

Conclusion

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