[Synovial sarcoma of the head and neck].
BACKGROUND
Synovial sarcoma (SS) is a mesenchymal malignancy, which predominantly occurs at the lower extremities of young adults. Only 3 % of the SS show a primary manifestation in the head and neck region.


METHODS
A retrospective chart review (years 2002 - 2006) of our Department of Otorhinolaryngology, head and neck surgery, School of Medicine, Bonn, Germany, was performed searching for patients with primary head and neck manifestations of SS.


METHODS
Four patients (2 female, 2 male) with a median age of 21 (12 - 28) years were assessed at our facility.


RESULTS
The location of the SS was the oropharynx respectively, infiltrating adjacent structures. 3 patients underwent radical surgery followed by chemotherapy, 2 in combination with irradiation. 1 patient underwent primary chemotherapy and irradiation. The histological diagnosis was a biphasic SS as well as a monophasic SS in 2 patients respectively. Fluorescence-in-situ-hybridization was performed in order to detect the distinctive chromosomal translocation t(X;18). 2 patients were in complete remission at the recent follow-up, 1 patient died 14 months after diagnosis due to a lethal carotidal haemorrhage. 1 patient was lost to follow-up.


CONCLUSIONS
The aggressive character of the SS as well as its high recurrence rate afford a radical therapeutic scheme enclosing surgical, chemotherapeutical and radiological treatment as well as a thorough follow-up. The genetic translocation t(X;18) leads the way to the right diagnosis. The prognosis of head and neck SS must be considered as poor. SS should be taken into consideration in head and neck tumors of unclear nature.
Kuehnemund
M
M
Klinik und Poliklinik für Hals-Nasen-Ohrenheilkunde/Chirurgie, Universitätsklinikum Bonn. Matthias.Kuehnemund@ukb.uni-bonn.de
Friedrichs
N
N
Bootz
F
F
D000293:Adolescent; D000328:Adult; D014408:Biomarkers, Tumor / Q000032:analysis; D002648:Child; D003131:Combined Modality Therapy; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008198:Lymph Nodes / Q000473:pathology; D008207:Lymphatic Metastasis / Q000175:diagnosis / Q000473:pathology; D008279:Magnetic Resonance Imaging; D008297:Male; D018396:Mucin-1 / Q000032:analysis; D009367:Neoplasm Staging; D010039:Otorhinolaryngologic Neoplasms / Q000175:diagnosis* / Q000473:pathology / Q000628:therapy; D013584:Sarcoma, Synovial / Q000175:diagnosis* / Q000473:pathology / Q000628:therapy; D014746:Vimentin / Q000032:analysis
D014408:Biomarkers, Tumor; D018396:Mucin-1; D014746:Vimentin