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Extraabdominelle Fibromatose

Fallbericht und Literaturübersicht

Extra-abdominal desmoid tumors

Case report and literature review

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Zusammenfassung

Muskuloaponeurotische Fibromatosen oder Desmoidtumoren sind seltene Erkrankungen, ihr Anteil an allen Neoplasien beträgt 0,03–0,1%. Es wird der Fall einer 57-jährigen Frau mit einem langsam wachsenden Tumor hinter dem M. sternocleidomastoideus vorgestellt, der in toto entfernt wurde. Die histologische Untersuchung bestätigte den klinischen Verdacht auf ein Desmoid. Desmoide sind aggressive, lokal infiltrierende, nicht metastasierende Tumoren mit einer hohen lokalen Rezidivrate. Genetische, endokrine und physikalische Faktoren werden als Ursache für Desmoide angesehen. Die Diagnosestellung erfolgt morphologisch, insbesondere reaktive Fibromatosen sowie das Fibrosarkom müssen differenzialdiagnostisch abgegrenzt werden. Durch die Kombination verschiedener bildgebender Verfahren gelingt die Größenbestimmung sowie die Abgrenzung von Gefäßen, Nerven und knöchernen Strukturen. Therapie der Wahl ist die chirurgische Tumorresektion. Adjuvante Therapiemaßnahmen wie Bestrahlung, Chemo- und Hormontherapie sind bei Inoperabilität oder ausgedehnten Rezidiven angezeigt.

Abstract

Musculoaponeurotic fibromatosis or desmoid tumors are rare. We report the case of a 57 year old woman with a slowly growing tumor behind the sternocleidomastoid muscle, which was completely removed. Histological examination confirmed the clinical suspicion of a desmoid tumor. Desmoid tumors are aggressive, locally infiltrating, non-metastasizing tumors with a high local recurrence. Genetic, endocrine and physical factors have been implicated as causative agents. The diagnosis is made histologically, reactive fibromatosis and fibrosarcoma must be eliminated in differential diagnosis. By the combination of different radiographic techniques, it is possible to describe the tumors and differentiate between vessels, nerves and bones. The therapy of the choice is the surgical resection. Adjuvant therapy, such as x-ray treatment, chemo- and hormone therapy, are indicated when the tumor is inoperable or too extensive for surgery.

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Authors and Affiliations

  1. Klinik für Hals-, Nasen-, Ohrenheilkunde, Unfallkrankenhaus Berlin

    J. Ridders, A. Ernst, I. Todt &  R. O. Seidl

  2. Klinik für Hals-, Nasen-, Ohrenheilkunde, Kopf- und Halschirurgie, Unfallkrankenhaus Berlin, Warener Straße 7, 12683, Berlin

    R. O. Seidl

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  1. J. Ridders
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  2. A. Ernst
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Correspondence to R. O. Seidl.

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Ridders, J., Ernst, A., Todt, I. et al. Extraabdominelle Fibromatose. HNO 53, 639–644 (2005). https://doi.org/10.1007/s00106-004-1134-9

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