Großes Hibernom des proximalen Oberarms und der Axilla – Literaturüberblick und Fallbeschreibung eines sehr seltenen gutartigen Weichteiltumors

 

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Zusammenfassung

Hibernome sind sehr seltene gutartige Weichteiltumoren, die von braunem Fettgewebe ausgehen. Eine Metastasierung oder maligne Entartung ist unbekannt. Epidemiologisch scheinen Männer etwas bevorzugt betroffen. Das Auftreten ist in der 3.–4. Lebensdekade gehäuft. Patienten mit intraossären Hibernomen sind im Schnitt deutlich älter. Bei Kindern sind Hibernome eine Rarität. Klinisch zeichnen sich Hibernome durch ein langsam progredientes Tumorwachstum aus. Die Dauer der klinischen Anamnese umfasst oftmals einige Jahre. Prädilektionsstellen sind der Oberschenkel, der Nacken, die Axilla und die peri- und interskapuläre Region. Die Diagnostik umfasst bei symptomatischen Hibernomen meist, neben der Röntgenbildgebung, die MRT mit Kontrastmittel. Asymptomatische Hibernome sind oft diagnostische Zufallsbefunde. Wichtige Differenzialdiagnosen sind das Lipom, das gut differenzierte Liposarkom, das Rhabdomyom, der Granularzelltumor und das Adenoma sebaceum. Eine Inzisionsbiopsie sollte immer zur histologischen Diagnosesicherung vor der definitiven Therapie erfolgen. Für die kurative Therapie ist nach Literaturmeinung die marginale Resektion empfohlen. Bei vollständiger Resektion sind Rezidive nicht beschrieben. Der folgende Artikel beschreibt die Kasuistik eines großen Hibernoms des proximalen Oberarms mit Beteiligung der Axilla und informiert anhand einer aktuellen Literaturrecherche über Epidemiologie, Klinik, Diagnostik, Therapie und Prognose dieser sehr seltenen, gutartigen Fettgewebsgeschwulst.

Abstract

Hibernomas are very rare benign soft tissue tumors arising from brown fat. Malignant transformation or metastases are unknown. Males seems to be affected more often. Most patients are aged 20 to 40, but patients with intraosseous hibernomas are older. In children, hibernomas are extremely rare. The tumors grow slowly and have usually been present for a few years on presentation. Hibernomas are typically located on the thigh, neck, axilla or in the peri- und interscapular region. Diagnostic work-up in symptomatic hibernomas usually includes conventional X-ray and magnetic resonance tomography (MRI) with contrast medium. Asymptomatic hibernomas are often found accidentally in the diagnostic work-up of other diseases. Important differential diagnoses are lipomas, well differentiated liposarcomas, rhabdomyomas, granular cell tumors and sebeceous adenomas. Incisional biopsy should be performed to allow definitive histological diagnosis before definitive therapy. According to the literature, histologically preserved hibernomas can be removed with curative intention and marginal resection. After complete tumor removal, local recurrence has not been described. The following article describes the case of a large hibernoma of the proximal arm, involving the axilla, and describes the epidemiology, clinical behavior, diagnostic work-up, therapy and prognosis of this very rare benign fatty soft tissue tumor, on the basis of a review of current literature.

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