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Pleomorphe High-grade-Sarkome der Weichgewebe

Ist die Subklassifikation zeitgemäß?

Pleomorphic high-grade soft tissue sarcomas

Is the subclassification up to date?

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Zusammenfassung

Die konzeptionelle Entwicklung in der Klassifikation pleomorpher „High-grade-Sarkome“ zeigt auf, welchen Beitrag die integrative morphologische, immunphänotypische und molekulargenetische Analyse für eine klinisch, prognostisch und therapeutisch ausgerichtete Charakterisierung zu leisten vermag. Inzwischen ist die klinische und prognostische Relevanz einer verfeinerten Subtypisierung pleomorpher „High-grade-Sarkome“ unbestritten. Es gilt, morphologisch ähnliche, nichtsarkomatöse Malignome von pleomorphen Sarkomen abzugrenzen und zu definieren. Innerhalb der Gruppe der pleomorphen „High-grade-Sarkome“ sind im Hinblick auf die Prognose- und Therapiestratifizierung die besonders aggressiven und prognostisch ungünstigen Subtypen von solchen zu unterscheiden, die durch ein weniger aggressives tumorbiologisches Verhalten gekennzeichnet sind. Zudem müssen benigne mesenchymale Tumoren mit fakultativer Pleomorphie erkannt werden. Schließlich gilt es, die erfolgversprechenden molekularen Ansätze diagnostisch, prognostisch und therapeutisch relevanter Gensignaturen an pathomorphologisch und klinisch klar definierten Kollektiven verschiedener Subtypen pleomorpher „High-grade-Sarkome“ zu vertiefen.

Abstract

The conceptual evolution in the classification of pleomorphic high-grade sarcomas is a paradigm of how the integrative morphological, immunohistochemical and molecular genetic analysis has contributed to a clinical, prognostic and therapy-oriented characterization of this complex group of tumors. The clinical and prognostic relevance of a refined subtyping of pleomorphic high-grade sarcomas, which until recently was considered a mere academic exercise, is now undisputed. It is imperative to unequivocally differentiate sarcomas from non-sarcomatous, clearly defined malignancies to start adequate therapy. Furthermore, pleomorphic sarcomas which are particularly aggressive and prone to poor prognosis, have to be separated from sarcomas which, in contrast to the pleomorphic phenotype, are characterized by a less aggressive behavior. Also, morphologically pleomorphic but benign mesenchymal tumors must be recognized. Finally, it is important to promote the promising, array-based identification of diagnostic, prognostic and clinically relevant gene signatures on larger collections of pathomorphologically and clinically precisely defined subtypes of pleomorphic high-grade sarcomas.

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Abbreviations

AFH:

Angiomatoides fibröses Histiozytom

AFX:

Atypisches Fibroxanthom

CGH:

Komparative genomische Hybridisierung

DDLS:

Dedifferenziertes Liposarkom

ESOS:

Extraskelettales Osteosarkom

FISH:

Fluoreszenz-in-situ-Hybridisierung

FS:

Fibrosarkom

GIST:

Gastrointestinaler Stromatumor

LMS:

Leiomyosarkom

LS:

Liposarkom

MFH:

Malignes fibröses Histiozytom

MFS:

Myxofibrosarkom

miRNA:

Mikro-RNA

MPNST:

Maligner peripherer Nervenscheidentumor

NF-1:

Neurofibromatose Typ 1

NFI:

Neurofibrom

OS:

Osteosarkom

PLLS:

Pleomorphes Liposarkom

PLMS:

Pleomorphes Leiomyosarkom

PRMS:

Pleomorphes Rhabdomyosarkom

RMS:

Rhabdomyosarkom

UPS:

Undifferenziertes pleomorphes Sarkom

SYSA:

Synovialsarkom

WGS:

Weichgewebssarkom

WHO:

„World Health Organization“

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  1. Pathologisches Institut, Universitsklinikum Heidelberg, Im Neuenheimer Feld 220, 69120, Heidelberg, Deutschland

    G. Mechtersheimer, M. Renner, R. Penzel & P. Schirmacher

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  1. G. Mechtersheimer
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  2. M. Renner
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Correspondence to G. Mechtersheimer.

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Mechtersheimer, G., Renner, M., Penzel, R. et al. Pleomorphe High-grade-Sarkome der Weichgewebe. Pathologe 32, 47–56 (2011). https://doi.org/10.1007/s00292-010-1400-4

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