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Orales adenoid-squamöses Karzinom

Tumormarker und Prognose

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Zusammenfassung

Fall

Der Fall einer 58-jährigen Patientin mit einem exophytischen adenoid-squamösen Karzinom (ASK) auf dem rechten Unterkiefer-Alveolarfortsatz wird mit der histologischen und immunhistochemischen Differenzialdiagnose sowie dem zellbiologischen Hintergrund einer ungünstigen Prognose vorgestellt. Das ASK wurde unter kurativer Zielsetzung durch radikale Tumorresektion mit Unterkieferteilresektion, radikal konservierender Neck Dissection und postoperativer Radiatio behandelt und histologisch als pT4 pN0 cM0, G3, R0 klassifiziert. Unter der Bestrahlung entwickelten sich regionale Lymphknoten- und Fernmetastasen. Der metastasenbedingte Tod trat 7 Monate nach Diagnosestellung ein.

Befunde

Die Tumorzellen des adenoid-squamösen Karzinoms exprimieren das epitheliale Intermediärfilament Zytokeratin, das epitheliale Membranantigen (EMA) und das epitheliale Basalmembranprotein Laminin-5 (Ln-5). Eine drüsige Differenzierung kann durch das Fehlen epithelialer Muzine (Alcianblau, Muzikarmin) ausgeschlossen werden. Die Abgrenzung des strukturähnlichen Angiosarkoms gelingt durch die endothelialen Differenzierungsmuster CD31, CD34 und Faktor-VIII-assoziiertes Antigen. Die Proliferation ist in beiden Entitäten hoch, der Ki-67-Index beträgt 20%. Das β-Catenin (Zell-Zell-Adhäsionsprotein) verliert seine membranbetonte Markierung und kann das histologische Muster der Akantholyse erklären. Ln-5 (Leitschiene der Invasion) ist in den ASK-Zellen massiv überexprimiert und kann zur zellbiologischen Erklärung der raschen Progression herangezogen werden.

Schlussfolgerungen

Die pseudopapilläre Wuchsform, zelluläre Atypie, gefäßartige Hohlraumbildungen sowie die Expression von Zytokeratin, EMA und von Ln-5 sind gemeinsame Merkmale des oralen ASK und des Angiosarkoms. Diagnoseentscheidend ist das Fehlen endothelialer Differenzierungsmarker wie CD31, CD34 und Faktor-VIII-assoziiertes Antigen im ASK. Die Modulation des β-Catenin-Musters (Transkriptionsfaktor für Ln-5) und die massive Überexpression des Invasionsfaktors Ln-5 werden als zellbiologische Ursachen der raschen Progredienz des ASK vorgeschlagen.

Abstract

Case report

A 58-year-old female patient presented with an exophytic adenoid squamous cell carcinoma on the right alveolar process of the lower jaw. Histological and immunohistochemical differential diagnosis and cellular background of the unfavorable prognosis are described. The patient was treated with curative intent by radical tumor resection including partial mandibulectomy, extensive conservative/radical neck dissection, and postoperative radiation. The adenoid squamous cell carcinoma was classified as pT4, pN0, cM0, R0. During radiation, regional lymph node metastases and distant metastases developed. The patient died of distant metastases 7 months after the initial diagnosis.

Histopathologic findings

Tumor cells of adenoid squamous cell carcinoma express epithelial intermediate filament cytokeratin, epithelial membrane antigen (EMA), and epithelial basal membrane protein laminin-5 (Ln-5). Glandular differentiation can be excluded by the absence of epithelial mucins (Alcian blue, mucicarmine). Differentiation from angiosarcoma can be performed using endothelial differential markers CD31, CD34, and factor VIII-associated antigen (FVIII-ass. AG). Both entities are characterized by high proliferation and Ki-67 index of 20%. β-catenin (cell-cell adhesive protein) loses its primary membrane-bound localization and can explain the histologic pattern of acantholysis. Ln-5 (guide rail of invasion) is massively expressed in adenoid squamous cell carcinoma cells and may be responsible for rapid progression.

Conclusions

Pseudopapillary proliferation, cellular atypia, vascular-like cavities, expression of cytokeratin, EMA, and Ln-5 are common features of oral adenoid squamous cell carcinoma and angiosarcoma. Diagnosis is determined by the absence of endothelial differential markers CD31, CD34, and FVIII-ass. AG. Modulation of the β-catenin pattern (transcription factor of Ln-5) and massive expression of invasion factor Ln-5 are suggested as cell biological reasons for rapid progression of adenoid squamous cell carcinoma.

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Authors and Affiliations

  1. Klinik für Mund-, Kiefer- und Gesichtschirurgie Plastische Operationen, Helios-Klinikum Erfurt

    O. Driemel & H. Pistner

  2. Institut für Pathologie, Helios-Klinikum Erfurt

    K. Braxein & H. Kosmehl

  3. Klinik für Mund-, Kiefer- und Gesichtschirurgie Plastische Operationen, Helios-Klinikum, Nordhäuser Straße 74, , 99089, Erfurt

    O. Driemel

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  1. O. Driemel
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  2. K. Braxein
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  3. H. Pistner
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  4. H. Kosmehl
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Correspondence to O. Driemel.

Additional information

Vortrag gehalten bei der 54. Jahrestagung der Arbeitsgemeinschaft für Kieferchirurgie gemeinsam mit dem Arbeitskreis für Oralpathologie und Oralmedizin innerhalb der DGZMK, 29.–31. Mai 2003 in Bad Homburg.

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Driemel, O., Braxein, K., Pistner, H. et al. Orales adenoid-squamöses Karzinom. Mund Kiefer GesichtsChir 8, 118–122 (2004). https://doi.org/10.1007/s10006-004-0532-1

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  • DOI: https://doi.org/10.1007/s10006-004-0532-1

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