Summary
The primary malignant fibrous histiocytoma of bone (Stout) is a rare and autonomous sarcoma of the bone which has to be separated from the osseous sarcoma and the osseous fibrosarcoma. The malignant histiocytoma is morphologically characterized by the storiform pattern of interlacing spindle cell bundles and functionally by the phagocytosis of lipids, glycogen, hemosiderin and hematoidin. Contrary to the osteosarcomas, the main age of osseous histiocytomas are the fifth and sixth decade. Location of predilection are the metaphyses of the long tubular bones. The X-rays show moth-eaten spongiolysis, endosteal erosion and perforation of the cortex. Early metastases in the lungs are frequent. The primary treatment is a surgical one.
Case record of a typical malignant osseous histiocytoma from the proximal metaphysis of the left femur with metastases in the lung of a female child, aged 14 years.
Zusammenfassung
Fallbericht. Das ossäre maligne fibröse Histiozytom ist eine seltene, selbständige ossäre Sarkomform. Sic ist morphologisch gekennzeichnet durch Spindelzellbündel, die sich vielfach überkreuzen (storiform pattern) und funktionell durch die Phagozytose von Fett, Glykogen, Hämosiderin. Das Häupterkrankungsalter fällt in die 5. und 6. Dekade. Häufigste Lokalisation sind die Metaphysen der langen Röhrenknochen, im besonderen die distale Femurmetaphyse. Das Röntgenbild zeigt eine mottenfraßdhäliche Spongiolyse, eine endostale Arrosion und Perforation der Kortikalis. Die Metastasierung erfolgt früihzeitig. Häufigster Metastasenort sind die Lungen. Die Behandlung der Primärgeschwulst ist eine chirurgische. Die Zahl der 5-Jahres-Heilungen liegt um 50%.
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Uehlinger, E., Haferkamp, O. Das maligne fibröse Histiozytom des Knochens. Arch. Orth. Traum. Surg. 92, 89–97 (1978). https://doi.org/10.1007/BF00381646
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DOI: https://doi.org/10.1007/BF00381646