Das metachrone multifokale Osteosarkom – zwei Fallstudien

Zusammenfassung

Das multifokale Osteosarkom ist eine charakteristische und gleichermaßen ungewöhnliche Form des Osteosarkoms, bei dem zusätzlich zu dem dominanten Primärtumor multiple weitere Läsionen auftreten. Treten diese Läsionen gleichzeitig auf und sind bereits bei der Diagnosestellung vorhanden, spricht man von einem synchronen multifokalen Osteosarkom. Das metachrone multifokale Osteosarkom ist definiert als das Auftreten multipler Läsionen (ohne Lungenmetastasen) zu unterschiedlichen Zeitpunkten nach der initialen Therapie des ursprünglichen Osteosarkoms, wobei eine jede sich klinisch als Primärtumor darstellt. Das multifokale Osteosarkom ist eine seltene Erkrankung mit schlechter Prognose. Es ist weiterhin nicht hinreichend geklärt, ob es sich um das Auftreten multipler primärer Tumoren oder doch um eine metastatische Erkrankung handelt. Bislang erschien lediglich eine geringe Anzahl von Fallstudien über das metachrone Auftreten des multifokalen Osteosarkoms. Wir berichten über zwei Patienten mit metachron aufgetretenen multifokalen Osteosarkomen, die auf eine multiple Primärtumorerkrankung hindeuten mit anschließender Diskussion der hierzu vorliegenden Literatur.

Summary

The multifocal osteosarcoma is a distinct and unusual form of osteosarcoma whereby, in addition to a single dominant tumour, multiple new bone lesions occur. A synchronous multifocal osteosarcoma is described as the simultaneous presence of two or more lesions at diagnosis. Metachronous multifocal osteosarcoma has been described as the occurrence of the tumour at two or more sites in a patient (without pulmonary metastases) at different intervals after treatment of the dominant lesion, each of them presenting as a primary tumour. Metachronous multifocal osteosarcoma is a rare disease with poor prognosis. There is much debate in the literature on whether it represents multiple primary lesions or metastatic disease. There are only few reports of cases of multifocal osteosarcoma. We present here two cases of metachronous multifocal osteosarcoma and review of the literature.

Schlüsselwörter

Knochentumor - metachrones multifokales Osteosarkom - synchrones multifokales Osteosarkom

Keywords

Bone tumour - metachronous multifocal osteosarcoma - synchronous multifocal osteo- sarcoma

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