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S2k-Leitlinie Diagnostik und Therapie uteriner Sarkome – Anforderungen an die Pathologie

Interdisciplinary S2k guidelines on the diagnosis and treatment of uterine sarcomas—recommendations for surgical pathology

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Zusammenfassung

Uterine Sarkome sind eine heterogene Gruppe seltener Malignome des Myometriums, des endometrialen Stromas bzw. des uterinen Bindegewebes mit einer Inzidenz von 1,5 bei Frauen kaukasischer und 3,0 bei afroamerikanischer Ethnie. Für Leiomyosarkome gibt es laut WHO kein Grading, auf Wunsch der Kliniker kann die Angabe des FNCLCC-Gradings in Analogie zu Weichgewebesarkomen erfolgen. Adenosarkome müssen vom Adenofibrom (dessen Existenz fragwürdig ist) abgegrenzt werden. Eine myometrane Infiltrationstiefe von >50 %, eine sarkomatöse Überwucherung und eine high-grade-heterologe Komponente bedingen eine höhere Rezidivrate mit ungünstigerer Prognose. Die Abgrenzung der low-grade- von den high-grade-endometrialen Stromasarkomen gelingt zumeist mit einer immunhistochemischen Paneldiagnostik und kann ergänzt werden durch molekularpathologische Untersuchungen. Aufgrund möglicher Therapierelevanz soll bei diesen Tumoren immer eine immunhistochemische Steroidhormonrezeptorbestimmung erfolgen. Undifferenzierte Sarkome stellen eine Ausschlussdiagnose dar und weisen eine ungünstige Prognose auf. Maligne Müller-Mischtumoren zählen nicht zu den uterinen Sarkomen. Aufgrund der intratumoralen Heterogenität sollen uterine Sarkome ≤2 cm vollständig und größere Tumoren mit einem Paraffinblock pro Zentimeter Größe eingebettet werden.

Abstract

Uterine sarcomas represent a heterogeneous group of rare malignancies, derived from the myometrium, the endometrial stroma, and very rarely from the nonspecialized uterine soft tissue. The actual incidence is about 1.5 for Caucasian and 3.0 for Afro-American women. There is no grading system for leimoysarcoma defined by the WHO classification; however, if clinicians request, the FNCLCC grading can be specified in analogy to soft tissue sarcomas. Adenosarcomas must be distinguished from adenofibromas (the existence of which is questionable)—with the vast majority of these tumors being uterine adenosarcomas. Within adenosarcomas, deep myometrial invasion (>50%), sarcomatous overgrowth, and a high-grade heterologous component are associated with a higher recurrence rate and poor survival. The immunohistochemical panel represents a very helpful tool for distinguishing low-grade from high grade endometrial stromal sarcomas (ESS) and may be supplemented by molecular analyses. Steroid hormone receptor analysis should be performed for all ESS due to the possible therapeutic relevance. Undifferentiated uterine sarcomas represent a diagnosis of exclusion and have a very poor prognosis. Carcinosarcomas represent a special subtype of endometrial carcinomas and are in fact not uterine sarcomas. Uterine sarcomas may present substantial intratumoral heterogeneity and adequate embedding is mandatory. Lesions ≤2 cm in the largest dimension should be processed completely and larger tumors should be processed with one block per centimeter for the largest tumor dimension.

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  1. Abteilung Mamma‑, Gynäko- & Perinatalpathologie, Institut für Pathologie, Universitätsklinikum Leipzig AöR, Liebigstraße 26, 04103, Leipzig, Deutschland

    Lars-Christian Horn & Anne Kathrin Höhn

  2. Frauenklinik, Hochtaunus-Kliniken gGmbH, Bad Homburg, Deutschland

    Dominik Denschlag

  3. Deutsche Krebsgesellschaft, Berlin, Deutschland

    Markus Follmann

  4. Institut für Pathologie, Viersen, Deutschland

    Dietmar Schmidt

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Lars-Christian Horn, Dominik Denschlag, Markus Follmann und Dietmar Schmidt sind Mitglieder der Kommission zur Erstellung der S3-Leitlinie Endometriumkarzinom.

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Horn, LC., Höhn, A.K., Denschlag, D. et al. S2k-Leitlinie Diagnostik und Therapie uteriner Sarkome – Anforderungen an die Pathologie. Pathologe 41, 621–633 (2020). https://doi.org/10.1007/s00292-020-00826-4

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