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Lipom und atypischer lipomatöser Tumor innerhalb einer Neoplasie

Hinweis für einen kontinuierlichen Übergang

Lipoma and atypical lipomatous tumor within the same neoplasia

Evidence for a continuous transition

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Zusammenfassung

Der Fall eines lipomatösen Tumors mit prädominierender Lipomkomponente und Übergang zu einem atypischen lipomatösen Tumor (ALT) vom lipomähnlichen Typ wird vorgestellt. Bei einer 70-jährigen Patientin wurde am rechten Oberschenkel ein tief lokalisierter Weichgewebstumor mit einer maximalen Größe bis 14 cm reseziert. Korrespondierend zum makroskopischen Erscheinungsbild bot der Tumor überwiegend das histologische Bild eines Lipoms ohne Atypie. Im restlichen Anteil (etwa 20%) fanden sich histologische Befunde eines ALT vom lipomähnlichen Typ. Immunhistochemisch war keine MDM 2-/CDK4-Expression innerhalb der Lipomkomponente, jedoch in der ALT-Komponente zu belegen. Mit Hilfe der Fluoreszenz-in-situ-Hybridisierung war keine MDM 2-Gen-Amplifikation und nur vereinzelt eine CDK4-Amplifikation innerhalb des Lipomanteils, jedoch eine deutliche Amplifikation des MDM 2-/CDK4-Gens in der ALT-Komponente nachweisbar, wodurch die morphologisch basierte Diagnose eines lipomatösen Tumors mit Arealen eines Lipoms und eines atypischen lipomatösen Tumors innerhalb einer Neoplasie zusätzlich molekularbiologisch belegt wurde. Der hier dargestellte Fall spricht für den graduellen Übergang eines Lipoms zu einem ALT und untermauert das Konzept einer kontinuierlichen schrittweisen Entwicklung benigner zu malignen lipomatösen Weichgewebstumoren im Sinne eines biologischen Kontinuums.

Abstract

The case of a lipomatous tumor with a predominant lipoma component and transition to an atypical lipomatous tumor is presented. A deep-seated soft tissue tumor of the right thigh with a maximum size of 14 cm was resected in a 70-year-old female patient. Corresponding to a comparable macroscopic aspect, the lesion revealed the histological features of an ordinary lipoma without atypia in about 80% of the specimen. In the remaining portion (approximately 20%) histopathology showed an atypical lipomatous tumor (ALT, lipoma-like subtype). Immunohistochemistry for MDM 2 and CDK4 revealed no immunoreactivity in the lipoma component, but within the ALT component. Interphase dual-color fluorescence in situ hybridization showed no amplification of the MDM 2 gene and rarely CDK4 gene amplification within the lipoma component, but high level amplification of MDM 2/CDK4 gene in the ALT area, further supporting the morphologically based diagnosis of a lipomatous tumor including areas of a true lipoma and ALT. This case underlines the concept of a continuous stepwise development of lipomatous soft tissue tumors from benign to malignant counterparts as a biological continuum.

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Abbreviations

ALT:

Atypischer lipomatöser Tumor

CDK4:

„Cyclin-dependent kinases“

FISH:

Fluoreszenz-in-situ-Hybridisierung

MDM 2:

„Murine double minute chromosome-2“

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Authors and Affiliations

  1. Institut für Pathologie am Clemenshospital, Medical Center, Münster, Düesbergweg 128, 48153, Münster, Deutschland

    C. Kuhnen

  2. Dermatopathologie Bodensee, Friedrichshafen, Friedrichshafen, Deutschland

    T. Mentzel

  3. Klinik für Hand-, Plastische und Rekonstruktive Chirurgie – Schwerbrandverletztenzentrum, BG-Unfallklinik Ludwigshafen, Ludwigshafen, Deutschland

    M. Lehnhardt

  4. Klinik für Plastische und Ästhetische Chirurgie, Handchirurgie , Helios Kliniken Wuppertal, Wuppertal, Deutschland

    H.H. Homann

  5. Department of Pathology, University Hospitals, Katholieke Universiteit Leuven, Leuven, Belgien

    R. Sciot

  6. Center for Human Genetics, Katholieke Universiteit Leuven, Leuven, Belgien

    M. Debiec-Rychter

Authors
  1. C. Kuhnen
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  2. T. Mentzel
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  3. M. Lehnhardt
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  4. H.H. Homann
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  5. R. Sciot
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  6. M. Debiec-Rychter
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Correspondence to C. Kuhnen.

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Kuhnen, C., Mentzel, T., Lehnhardt, M. et al. Lipom und atypischer lipomatöser Tumor innerhalb einer Neoplasie. Pathologe 31, 129–134 (2010). https://doi.org/10.1007/s00292-009-1257-6

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