Zusammenfassung
Gestationsbedingte Trophoblasterkrankungen (GTD) sind selten und stellen aufgrund ihrer klinikopathologischen Besonderheiten eine große Herausforderung für Pathologen und Kliniker dar. Die Blasenmole und die Partialmole als Formen der villösen GTD sind aufgrund ihres differenten Risikos für eine persistierende Erkrankung und ihres zytogenetischen Hintergrundes streng zu trennen. Die häufigste Form der nicht villösen GTD ist das Chorionkarzinom. Die notwendige Chemotherapie erfolgt in strenger Korrelation zum WHO-Prognosescore. Als weitere Entitäten sind in den vergangenen Jahren der Plazentabettknoten und die hyperplastische Implantationsstelle abgegrenzt worden, die keiner weiteren Therapie bedürfen und im Allgemeinen Zufallsbefunde darstellen. Zu den Tumoren, die vom intermediären Trophoblasten ausgehen, zählen der Plazentabetttumor und der jüngst beschriebene epitheloide Trophoblasttumor. Beide zeigen in der Majorität einen benignen Verlauf, sollten jedoch, ähnlich wie die Blasenmole und das Chorionkarzinom, mit sequenziellen HCG-Bestimmungen überwacht werden. Die Prognose der GTD hängt in entscheidendem Maße vom rechtzeitigen Beginn der adäquaten Therapie ab, was einer engen Kooperation zwischen Pathologen und Gynäkologen bedarf. Angestrebt werden sollte die Zentralisation der betroffenen Patientinnen in spezialisierten Einrichtungen.
Abstract
Gestational trophoblastic disease (GTD) comprises a spectrum of uncommon and unique clinical and pathologic entities. The process of GTD is now better understood and new entities such as placental-site trophoblastic tumor (PSST) and epitheloid trophoblastic tumor have been morphologically defined. Complete hydatiform mole, a form of the villous type of GTD, has to be distinguished from partial mole because of the different cytogenetic composition and risk for persistent disease. Choriocarcinoma is the most frequent form of nonvillous GTD. Both PSST and epitheloid trophoblastic tumor derive from the intermediate trophoblastic cells and usually have a benign course. Optimal treatment requires a precise morphologic classification and close cooperation between the pathologist and the clinician. All patients treated for GTD require long-term monitoring of serum hCG. Patients with GTD should be registered, and possibly treated, at specialized centers.
Schlüsselwörter
Chorionkarzinom - hyperplastische Implantationsstelle - Plazentabetttumor - Trophoblast - maligne - Trophoblasterkrankung - Schwangerschaft - gestationsbedingt
Key words
Trophoblast - gestational - trophoblastic disease - placental site trophoblastic tumor - placental site nodule - epitheloid trophoblastic tumor - choriocarcinoma - malignant - treatment - placenta
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OA PD Dr. med. L.-C. Horn
Institut für Pathologie
Universität Leipzig
Liebigstraße 26
04103 Leipzig
Email: hornl@medizin.uni-leipzig.de
