PMC2853979_01
Pre- and postoperative radiological imaging of the right leg and in situ pictures: (a and b) preoperative conventional X-rays of the right knee. The tumor shows an eccentric osteolytic lesion located at the epimetaphyseal proximal tibia, not respecting the epiphyseal plate. The medial cortical bone is completely destroyed and the formation of partially calcified periosteal lining suggests a Lodwick-type 1C lesion. (c) MRI scan demonstrating a T1 isointense, T2 hypointense tumor formation. A similar zonal architecture with a large central core of very low signal intensity and a peripheral rim of intermediate to high signal intensity on T1- and T2-weighted spin-echo pulse sequences was observed by Christensen et al.. (d) MRI scan showing high uptake of Gadolinium predominantly at the tumor's periphery. A thin layer of sclerotic bone separates the tumor from circumjacent marrow edema. (e and f) Intraoperative images during tumor resection and implantation of the endoprosthesis. Resection of the tumor was performed in no-touch technique. The scar of the previous open biopsy (white arrows) as well as the former access path remained on the resected bone. Reconstruction was accomplished by implanting a partially custom-made tumor endoprosthesis in combination with linked knee replacement. (g and h) Postoperative radiographs of the right leg show the endoprosthesis in proper position.
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fig1
After two courses of Cisplatin/Doxorubicin and five courses of high-dose Methotrexate our patient underwent tumor resection and endoprosthetic reconstruction (Figure 1).
H&E staining of sclerosing epithelioid fibrosarcoma after treatment with neoadjuvant chemotherapy (magnification x400). The tumor is composed of few scattered polynuclear giant cells (black arrow) and groups of cells, which are rich in cytoplasm and show pale, vesicular, and irregular nuclei without any substantial mitotic activity (white arrow). The tumor cells are embedded in a collagen-rich extracellular matrix and display no signs of regression.
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fig2
Moreover, the tumor showed no signs of regression in the resection specimen (grade of regression VI according to Salzer-Kuntschik) (Figure 2).
Schematic illustration of the treatment regimen including cumulative dosages of the chemotherapeutic drugs. Each chemotherapy cycle of the main therapy is depicted as a yellow box, and the week when the cycle started is shown below. The time point of surgery and implantation of endoprosthesis is indicated with a red box (OP). The regimens of the maintenance therapy and follow-up are not displayed in detail.
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fig3
Trofosfamide (overview in Figure 3).