Ossifizierender fibromyxoider Tumor der Nasenhaupt- und Nasennebenhöhlen

Zusammenfassung

Hintergrund: Ossifizierende fibromyxoide Tumoren (OMFT) sind in der Regel gutartige, zu Rezidiven neigende Weichteiltumoren des Erwachsenen, deren Histogenese ungeklärt ist. Bei Kindern wurden bisher erst drei Fälle beschrieben. Patient: Wir berichten über den Kasus eines 12-jährigen Jungen erstmals mit Lokalisation in der Nasenhaupthöhle und im Nasennebenhöhlensystem. Schlussfolgerungen: OMFT sind seltene Neoplasien, ihre Diagnose erfolgt über die histologische Sicherung, die Therapie besteht in der totalen Exstirpation.

Ossifying Fibromyxoid Tumor of Soft Parts (OMFT) of the Nasal Cavity and the Paranasal Sinus

Background: OMFT are nearly always benign, usually presented in soft tissue, of uncertain origin and tend to relapse. They are regarded as neoplasms of the adult, only three cases of children have been reported so far. Patient: A case of a 12-year-old boy is presented, for the first time, with an OMFT in the nasal cavity and the paranasal sinus. The diameter measured 9 centimetres. Histologically the tumor consisted of a myxoid matrix and showed areas of metaplastic bone. Most of the cells showed a spindleform and sometimes polymorphic hyperchromatic nuclei. The tumor expressed S-100 protein and vimentin. The cellularity was moderately high, the mitotic rate was very low. Conclusions: OMFT are rare neoplasms, the diagnosis should be verified histopathologically. Complete excision should be performed. A clinical follow-up is necessary.

Schlüsselwörter:

Ossifizierender fibromyxoider Tumor - Kind - Nasenhaupt, -nebenhöhlen - S-100-Protein - Vimentin - Kasuistik

Key words:

Ossifying fibromyxoid tumor - Children - Nasal cavity - Paranasal sinus - S-100 protein - Vimentin - Case report

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