Zusammenfassung
Bei einem 28-jährigen Patienten ergaben Biopsie und Kürettage einer osteolytischen Läsion der distalen Tibia die Diagnose eines Riesenzelltumors des Knochens (RZT). Drei Monate später erfolgte die Resektion eines Rezidivs. 6 Monate nach Erstmanifestation traten multiple Lungenmetastasen auf. Eine Chemotherapie nach dem COSS-Protokoll, später mit Carboplatin und VP16, wurde wegen Tumorprogredienz durch Adriamycin ersetzt. Nach Erreichen der Maximaldosis wurde auf Gemcitabin gewechselt, mit insgesamt guter Palliation, weniger Anstrengungsdyspnoe und Gehfähigkeit mit Gehilfen.
Malignitätskriterien waren in den initialen pathologischen Untersuchungen nicht erhoben worden. In Anbetracht des rapid malignen Verlaufs ergab die nochmalige Durchsicht des Gewebes histologische Hinweise auf eine zum RZT zusätzliche fibrohistiozytäre/fibrosarkomatöse Komponente.
Die Malignitätsdiagnose beim RZT ist bei fehlender offensichtlich sarkomatöser Komponente von erheblicher Problematik. Zytologische Atypien einer monomorphen spindelzelligen Komponente und zungenförmige Ausläufer in das Weichgewebe sind wichtige Hinweise und müssen bei der Therapieplanung berücksichtigt werden. Zur Verlangsamung der Krankheitsprogression kann eine Monotherapie mit Adriamycin oder Gemcitabin erwogen werden.
Abstract
The case of a 28-year-old male patient with a locally aggressive lesion of the distal tibia is presented. Following the diagnosis of giant cell tumor of bone (GCT) on biopsy and curettage, a rapid malignant course was observed with recurrence 2.5 months later. Multiple metastases appeared 6 months after initial presentation. Following initial chemotherapy according to the COSS protocol and later with carboplatin and VP-16, therapy was changed to Adriamycin and later gemcitabine due to progressive disease. Good palliation was achieved, and the patient feIt well with less shortness of breath on exertion and was ambulatory with walking aids.
The malignant nature of the tumor was not detected in the initial pathologic examinations. Review of the pathologic material provided histologic clues permitting the diagnosis of a primary malignant GCT with a fibrohistiocytic/fibrosarcomatous component.
Malignancy in a giant cell tumor is a much debated diagnostic dilemma when a frank sarcomatous component is lacking. Cytologic atypias and flame-like tufts of infiltration of soft tissue are important clues. Surgical treatment should be commensurate. Monotherapy with Adriamycin or gemcitabine can be considered in order to inhibit the disease progression.
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Lachat, M.R., Weber, M., Cserhati, M.D. et al. Riesenzelltumor des Knochens mit rapid malignem Verlauf. Orthopäde 33, 344–348 (2004). https://doi.org/10.1007/s00132-003-0554-7
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DOI: https://doi.org/10.1007/s00132-003-0554-7