Das PECom der Leber – eine neue Entität der Lebertumoren?

Zusammenfassung

Die perivaskulär epitheloiden Zelltumoren (PECom) der Leber sind seltene Tumoren, die immunhistochemisch durch eine duale Expression von melanozytären und glattmuskulären Markern gekennzeichnet sind. Durch die moderne Diagnostik werden sie zunehmend erfasst. In dieser Arbeit berichten wir über den Fall eines PEComs der Leber als Zufallsbefund. Die klinischen und bildmorphologischen Charakteristika des Tumors werden beleuchtet und mit der Literatur verglichen. Das Management von Patienten mit PECom der Leber ist bisher nicht standardisiert, sodass eine Histologie inkl. Immunhistochemie zur Differenzierung unumgänglich ist. Wichtig ist bei nicht sicher klassifizierbaren Tumoren der Leber auch an diese seltene Entität zu denken.

Abstract

Perivascular epitheloid cell tumor (PEComa) is a rare tumor, characterized by dual Expression of smooth muscle and melanocytic markers. Due to the development of diagnostic procedures, we now diagnose PEComa more often. We report about a case of PEComa of the liver as an accidental finding. We analyze the clinical and morphological characteristics of this tumor and compare it with the data of the literature. Management of patients with PEComa is not yet standardized; therefore biopsy with immunhistochemical staining is necessary for the diagnosis. In case of liver tumors which cannot be classified by their morphology on imaging modalities, it is important to think about this rare entity.

Schlüsselwörter

Leber - PECom - KMUS - CT - MRT - Immunhistochemie

Key words

liver - PEComa - CMES - CT - MRI - immunohistochemistry

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