Tumor-like Lesions, unter besonderer Berücksichtigung der spinalen aneurysmatischen Knochenzyste

Zusammenfassung

Die aneurysmatische Knochenzyste (AKZ) ist ein lokal aggressiv wachsender gutartiger primärer osteoklastischer Riesenzell-reicher Tumor, der in 10-30% die Wirbelsäule betrifft. Lange Zeit wurde angenommen, dass es sich um eine Tumor-ähnliche Läsion handelt. Durch den Nachweis einer chromosomalen Translokation des USP (Ubiquitin-spezifische Protease) 6 Gens auf Chromosom 17p13 entspricht die primäre AKZ jedoch einer echten Neoplasie [4] [5] [6] [7] [8]. Die Prognose ist sehr gut mit Heilungsraten von > 90% nach intraläsionaler Resektion und Gesamtüberlebensraten von ≥98% [11] [12]. Die Einteilung erfolgt nach ihrem Wachstumsverhalten in die Enneking Stadien S1 bis S3. Die chirurgische Therapie galt bisher als Goldstandard für die Therapie der AKZ: die intraläsionale Kürettage ± adjuvante Therapie bei Enneking S1 und S2 Tumoren und die extraläsionale en bloc Resektion bei S3 Tumoren. In neueren Studien ist ein Trend erkennbar S2 Tumore zunehmend primär mittels serieller selektiver arterieller Embolisationen zu behandeln mit erfolgsversprechenden Ergebnissen [11]. Bei S3 Tumoren gibt es Expertenmeinungen, die die en bloc Resektionen aufgrund der primären Gutartigkeit des Tumors, der guten Ergebnisse nach intraläsionaler Resektion und der hohen perioperativen Morbidität als Übertherapie betrachten [11]. Eine Besonderheit bei der Therapie sind die vielfältigen nicht chirurgischen primären und adjuvanten Therapieansätze, die i.d.R. auf kleinen Fallserien beruhen. Bei lokal fortgeschrittenen, inoperablen oder Rezidivtumoren gibt es neuerdings Therapieansätze mit dem off-Label Einsatz von Denosumab primär, adjuvant oder neoadjuvant mit vielversprechenden ersten Ergebnissen.

Abstract

The aneurysmatic bone cysts (ABC) is a locally aggressive benign osteoclastic giant cell-rich primary bone tumors. Between 10–30% are localized in the spine. For a long time it was presumed to be a tumor-like lesion. The presence of the chromosomal translocation of the USP (ubiquitine-specific protease) 6 gene on chromosome 17p13 however seems to prove that ABCs are of genuine neoplastic nature [4] [5] [6] [7] [8]. The overall cure rates (>90%) as well as overall survival rates (≥98%) are excellent [11] [12]. According to their growth pattern ABCs are staged into Enneking stages S1 to S3. Up to now surgery was the goldstandard of treatment: intralesional curettage ± adjuvant therapy for Enneking S1 and S2 tumors and extralesional en bloc resection for S3 tumors. In more recent studies there is a trent toward non-surgical treatment for S2 tumors with primary selective arterial embolization mit promising results [11]. With regard to S3 tumors expert opinions exist stating that en bloc resection might be considered an overtreatment since the tumor is of benign nature, satisfactory results after intralesional resection and the associated high perioperative morbidity [11]. Noteworthy it the high variety of non-surgical primary and adjuvant treatments, which usually are based on small case series. Recently, the off-label treatment with Denosumab In locally progressive, inoperable or recurrent tumors has been advocated as primary, adjuvant or neoadjuvant treatment with promising results.

Schlüsselwörter

Tumor-like Lesions - Aneurysmatische Knochenzyste (AKZ) - intraläsionale Kürettage - intraläsionale Resektion - extraläsionale en bloc Resektion - Selektive arterielle Embolisation (SAE) - off-Label Denosumab

Keywords

Tumor-like lesion - aneurysmatic bone cyst (ABC) - intralesional curettage - intralesional resection - extralesional en bloc resection - selective arterial embolization (SAE) - off-label Denosumab

Publication History

Article published online:
03 November 2021

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