Malignant Peripheral Nerve Sheath Tumor (MPNST) of the Uterine Cervix – A Systematic Literature Review and New Case Report with Possible New Therapeutic Approaches

Abstract

Malignant schwannomas of the visceral organs are extremely rare. To date, 19 cases of cervical schwannoma have been reported in the literature worldwide. A single case prompted a review of the literature. The differential diagnosis between melanoma and schwannoma is made using the marker HMB-45, which is positive for melanoma and negative for schwannoma. This differentiation allowed us to identify two of the 20 cases that were probably not schwannomas.

The prognosis of malignant cervical schwannoma is poor. Approximately half of the cases develop local recurrence or distant metastases within a short time, despite resection in healthy tissue.

In one case, gene sequencing revealed a potential treatment approach with the mTOR inhibitor everolimus and trastuzumab. Unfortunately, the potential efficacy of these treatment options could not be tested because the patient was being treated at another hospital at the time of relapse, and they did not use a drug that matched the NGS profile.

Zusammenfassung

Maligne Schwannome der viszeralen Organe sind extrem selten. Bis heute gibt es weltweit nur 19 Fallberichte von zervikalen Schwannomen in der Literatur. Das Auftreten eines solchen Schwannoms in unserem Krankenhaus war der Anlass für eine Sichtung der aktuellen Literatur. Die Differenzialdiagnose unterschiedet zwischen einem Melanom und einem Schwannom mithilfe des Markers HMB-45, der positiv für Melanome und negativ für Schwannome ist. Diese Differenzierung erlaubte uns, aus 20 Fällen 2 zu identifizieren, die wahrscheinlich keine Schwannome waren.

Die Prognose für ein malignes zervikales Schwannom ist schlecht. In der Hälfte aller Fälle entwickelt sich innerhalb kurzer Zeit ein Lokalrezidiv oder eine Fernmetastase, selbst wenn die Resektion im gesunden Gewebe durchgeführt wurde.

In einem Fall hat die Genomsequenzierung einen möglichen Behandlungsansatz mit dem mTOR-inhibitor Everolimus und Trastuzumab aufgezeigt. Leider konnte die potenzielle Wirksamkeit dieser Behandlungsoption nicht geprüft werden, weil die Patientin zum Zeitpunkt des Rückfalls in einem anderen Krankenhaus behandelt wurde und das Krankenhaus kein Arzneimittel, das dem NGS-Profil entsprach, einsetzte.

Keywords

malignant schwannoma - cervix uteri - therapy - prognosis - schwannoma

Schlüsselwörter

malignes Schwannom - Cervix uteri - Therapie - Prognose - Schwannom

Publication History

Received: 14 July 2024

Accepted after revision: 18 November 2024

Article published online:
10 December 2024

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