Zusammenfassung
Ein 59-jähriger Patient gab Schmerzen an der rechten Halsseite sowie eine neu aufgetretene Armheberschwäche rechts an.
Es zeigte sich ein Tumor der rechts-paravertebralen Halsweichteile, histologisch ein maligner peripherer Nervenscheidentumor (MPNST). 13 Jahre zuvor war ein Plattenepithelkarzinom der Tonsille rechts operativ entfernt sowie ein cystischer plattenepithelialer Weichgewebstumor im Bereich der ipsilateralen Halsweichteile radiotherapiert worden.
Es entwickelte sich ein hohes Querschnittssyndrom mit Phrenikusparese. Eine palliative Chemotherapie erzielte keine Besserung. Drei Monate später starb der Patient.
MPNST sind selten und können als Spätfolge einer Radiotherapie auftreten . In ehemaligen Bestrahlungsfeldern sollte daher eine strahleninduzierte Zweitneoplasie in die Differenzialdiagnose einbezogen werden.
Abstract
History and diagnosis
A 59-year old engineer was admitted to the hospital because of pain in his right collar region and the onset of incomplete paresis of the right arm. Magnetic resonance tomography displayed an advanced tumour arising from the right paravertebral soft tissue. Histological examination revealed a malignant peripheral nerve sheath tumor (MPNST). Thirteen years before admission, the patient had a right-sided tumor-tonsillectomy of a squamous cell carcinoma and local radiation of a cystic squamous cell carcinoma in the ipsilateral cervical soft tissue.
Clinical course and therapy
In the following course, progressive neurological symptoms occurred including beginning paraplegia, right phrenic paralysis and a severe concomitant pain syndrome. Due to the location and advanced tumor state, surgical treatment was not performed and palliative chemotherapy remained ineffective. Three months later the patient died due to rapidly progressive neurological failure.
Conclusion
MPNST represents a rare entity which has been related to postoperative radiation. Unusual neurological symptoms in anatomical regions of former radiation should therefore include neurogenic secondary malignancies in the differential diagnosis for early surgical intervention.
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Siveke, J.T., Sen Gupta, R., Rieckhoff, K.U. et al. Progrediente Lähmungen infolge eines strahleninduzierten zervikalen malignen peripheren Nervenscheidentumors. HNO 51, 825–828 (2003). https://doi.org/10.1007/s00106-003-0806-1
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DOI: https://doi.org/10.1007/s00106-003-0806-1