We evaluated the results of limb-sparing surgery and reconstruction of bone defects with vascularized fibula grafts in 8 consecutive patients (mean age at operation 13.6 years (range 4.1&#x2013;24.2 years), female/male = 6/2) with bone sarcomas (BS) (osteosarcoma/Ewing's sarcoma/chondrosarcoma= 4/3/1) operated on form 2000 to 2006. The bone defects reconstructed were proximal femoral diaphysis and epiphysis (<svg style="vertical-align:-0.10033pt;width:35.099998px;" id="M1" height="10.8125" version="1.1" viewBox="0 0 35.099998 10.8125" width="35.099998"  xmlns="http://www.w3.org/2000/svg">
	<g transform="matrix(1.25,0,0,-1.25,0,10.8125)">
		<g transform="translate(72,-63.35)">
			<text transform="matrix(1,0,0,-1,-71.95,63.5)">
				<tspan style="font-size: 12.50px; " x="0" y="0">𝑛</tspan>
				<tspan style="font-size: 12.50px; " x="9.6773224" y="0">=</tspan>
				<tspan style="font-size: 12.50px; " x="21.71771" y="0">2</tspan>
			</text>

		</g>
	</g>
</svg>), humeral diaphysis (<svg style="vertical-align:-0.10033pt;width:35.099998px;" id="M2" height="10.8125" version="1.1" viewBox="0 0 35.099998 10.8125" width="35.099998"  xmlns="http://www.w3.org/2000/svg">
	<g transform="matrix(1.25,0,0,-1.25,0,10.8125)">
		<g transform="translate(72,-63.35)">
			<text transform="matrix(1,0,0,-1,-71.95,63.5)">
				<tspan style="font-size: 12.50px; " x="0" y="0">𝑛</tspan>
				<tspan style="font-size: 12.50px; " x="9.6773224" y="0">=</tspan>
				<tspan style="font-size: 12.50px; " x="21.71771" y="0">2</tspan>
			</text>

		</g>
	</g>
</svg>), humeral proximal diaphysis and epiphysis (<svg style="vertical-align:-0.10033pt;width:35.099998px;" id="M3" height="10.8125" version="1.1" viewBox="0 0 35.099998 10.8125" width="35.099998"  xmlns="http://www.w3.org/2000/svg">
	<g transform="matrix(1.25,0,0,-1.25,0,10.8125)">
		<g transform="translate(72,-63.35)">
			<text transform="matrix(1,0,0,-1,-71.95,63.5)">
				<tspan style="font-size: 12.50px; " x="0" y="0">𝑛</tspan>
				<tspan style="font-size: 12.50px; " x="9.6773224" y="0">=</tspan>
				<tspan style="font-size: 12.50px; " x="21.71771" y="0">1</tspan>
			</text>

		</g>
	</g>
</svg>), femoral diaphysis (<svg style="vertical-align:-0.10033pt;width:35.099998px;" id="M4" height="10.8125" version="1.1" viewBox="0 0 35.099998 10.8125" width="35.099998"  xmlns="http://www.w3.org/2000/svg">
	<g transform="matrix(1.25,0,0,-1.25,0,10.8125)">
		<g transform="translate(72,-63.35)">
			<text transform="matrix(1,0,0,-1,-71.95,63.5)">
				<tspan style="font-size: 12.50px; " x="0" y="0">𝑛</tspan>
				<tspan style="font-size: 12.50px; " x="9.6773224" y="0">=</tspan>
				<tspan style="font-size: 12.50px; " x="21.71771" y="0">1</tspan>
			</text>

		</g>
	</g>
</svg>), ulnar diaphysis (<svg style="vertical-align:-0.10033pt;width:35.099998px;" id="M5" height="10.8125" version="1.1" viewBox="0 0 35.099998 10.8125" width="35.099998"  xmlns="http://www.w3.org/2000/svg">
	<g transform="matrix(1.25,0,0,-1.25,0,10.8125)">
		<g transform="translate(72,-63.35)">
			<text transform="matrix(1,0,0,-1,-71.95,63.5)">
				<tspan style="font-size: 12.50px; " x="0" y="0">𝑛</tspan>
				<tspan style="font-size: 12.50px; " x="9.6773224" y="0">=</tspan>
				<tspan style="font-size: 12.50px; " x="21.71771" y="0">1</tspan>
			</text>

		</g>
	</g>
</svg>), and tibial diaphysis (<svg style="vertical-align:-0.10033pt;width:35.099998px;" id="M6" height="10.8125" version="1.1" viewBox="0 0 35.099998 10.8125" width="35.099998"  xmlns="http://www.w3.org/2000/svg">
	<g transform="matrix(1.25,0,0,-1.25,0,10.8125)">
		<g transform="translate(72,-63.35)">
			<text transform="matrix(1,0,0,-1,-71.95,63.5)">
				<tspan style="font-size: 12.50px; " x="0" y="0">𝑛</tspan>
				<tspan style="font-size: 12.50px; " x="9.6773224" y="0">=</tspan>
				<tspan style="font-size: 12.50px; " x="21.71771" y="0">1</tspan>
			</text>

		</g>
	</g>
</svg>). One patient with Ewing's sarcoma had an early hip disarticulation, developed multiple metastases, and died 9 months after the operation. The remaining patients (<svg style="vertical-align:-0.10033pt;width:35.099998px;" id="M7" height="10.6" version="1.1" viewBox="0 0 35.099998 10.6" width="35.099998"  xmlns="http://www.w3.org/2000/svg">
	<g transform="matrix(1.25,0,0,-1.25,0,10.6)">
		<g transform="translate(72,-63.52)">
			<text transform="matrix(1,0,0,-1,-71.95,63.67)">
				<tspan style="font-size: 12.50px; " x="0" y="0">𝑛</tspan>
				<tspan style="font-size: 12.50px; " x="9.6773224" y="0">=</tspan>
				<tspan style="font-size: 12.50px; " x="21.71771" y="0">7</tspan>
			</text>

		</g>
	</g>
</svg>) are all alive 50 months (range 26&#x2013;75 months) after surgery. During the follow-up the following major complications were seen: 1-2 fractures (<svg style="vertical-align:-0.10033pt;width:35.099998px;" id="M8" height="10.8125" version="1.1" viewBox="0 0 35.099998 10.8125" width="35.099998"  xmlns="http://www.w3.org/2000/svg">
	<g transform="matrix(1.25,0,0,-1.25,0,10.8125)">
		<g transform="translate(72,-63.35)">
			<text transform="matrix(1,0,0,-1,-71.95,63.5)">
				<tspan style="font-size: 12.50px; " x="0" y="0">𝑛</tspan>
				<tspan style="font-size: 12.50px; " x="9.6773224" y="0">=</tspan>
				<tspan style="font-size: 12.50px; " x="21.71771" y="0">4</tspan>
			</text>

		</g>
	</g>
</svg>), pseudarthrosis (<svg style="vertical-align:-0.10033pt;width:35.099998px;" id="M9" height="10.8125" version="1.1" viewBox="0 0 35.099998 10.8125" width="35.099998"  xmlns="http://www.w3.org/2000/svg">
	<g transform="matrix(1.25,0,0,-1.25,0,10.8125)">
		<g transform="translate(72,-63.35)">
			<text transform="matrix(1,0,0,-1,-71.95,63.5)">
				<tspan style="font-size: 12.50px; " x="0" y="0">𝑛</tspan>
				<tspan style="font-size: 12.50px; " x="9.6773224" y="0">=</tspan>
				<tspan style="font-size: 12.50px; " x="21.71771" y="0">2</tspan>
			</text>

		</g>
	</g>
</svg>), and hip dislocation (<svg style="vertical-align:-0.10033pt;width:35.099998px;" id="M10" height="10.8125" version="1.1" viewBox="0 0 35.099998 10.8125" width="35.099998"  xmlns="http://www.w3.org/2000/svg">
	<g transform="matrix(1.25,0,0,-1.25,0,10.8125)">
		<g transform="translate(72,-63.35)">
			<text transform="matrix(1,0,0,-1,-71.95,63.5)">
				<tspan style="font-size: 12.50px; " x="0" y="0">𝑛</tspan>
				<tspan style="font-size: 12.50px; " x="9.6773224" y="0">=</tspan>
				<tspan style="font-size: 12.50px; " x="21.71771" y="0">1</tspan>
			</text>

		</g>
	</g>
</svg>). 
Limb-sparing surgery with reconstruction of bone defects using vascularized fibular grafts in BS cases is feasible with acceptable clinical results, but fractures should be expected in many patients.

Michael M&#xf8;rk Petersen

Dorrit Hovgaard

Jens J&#xf8;rgen Elberg

Catherine Rechnitzer

S&#xf8;ren Daugaard

Aida Muhic

Vascularized Fibula Grafts for Reconstruction of Bone Defects after Resection of Bone Sarcomas

Multiple Osteochondromas (MO) is a disease of benign bony growths with a low incidence of malignant transformation. Secondary chondrosarcoma in children is rare even in children with MO. Making a diagnosis of malignancy in low-grade cartilage tumors is challenging and requires consideration of clinical, radiographic, and histopathological factors. We report two cases of skeletally immature patients with MO who presented with rapidly enlarging and radiographically aggressive lesions consistent with malignant transformation. Both underwent allograft reconstruction of the involved site with no signs of recurrence or metastatic disease at a minimum of four-year follow-up.

Gregory A. Schmale

Douglas S. Hawkins

Joe Rutledge

Ernest U. Conrad

Malignant Progression in Two Children with Multiple Osteochondromas

<i  >Purpose</i>. The aim of this study was to prospectively evaluate whether FDG-PET allows an accurate assessment of histopathologic response to neoadjuvant treatment in adult patients with primary bone sarcomas. <i  >Methods</i>. Twelve consecutive patients with resectable, primary high grade bone sarcomas were enrolled prospectively. FDG-PET/CT imaging was performed prior to the initiation and after completion of neoadjuvant treatment. Imaging findings were correlated with histopathologic response. <i  >Results</i>. Histopathologic responders showed significantly more pronounced decreases in tumor FDG-SUVmax from baseline to late follow up than non-responders (<svg style="vertical-align:-1.09097pt;width:49.037498px;" id="M1" height="12.175" version="1.1" viewBox="0 0 49.037498 12.175" width="49.037498"  xmlns="http://www.w3.org/2000/svg">
	<g transform="matrix(1.25,0,0,-1.25,0,12.175)">
		<g transform="translate(72,-62.26)">
			<text transform="matrix(1,0,0,-1,-71.95,63.4)">
				<tspan style="font-size: 12.50px; " x="0" y="0">6</tspan>
				<tspan style="font-size: 12.50px; " x="6.2515001" y="0">4</tspan>
				<tspan style="font-size: 12.50px; " x="15.278666" y="0">±</tspan>
				<tspan style="font-size: 12.50px; " x="26.618887" y="0">1</tspan>
				<tspan style="font-size: 12.50px; " x="32.870388" y="0">9</tspan>
			</text>

		</g>
	</g>
</svg>&#x25; versus <svg style="vertical-align:-1.09097pt;width:49.037498px;" id="M2" height="12.05" version="1.1" viewBox="0 0 49.037498 12.05" width="49.037498"  xmlns="http://www.w3.org/2000/svg">
	<g transform="matrix(1.25,0,0,-1.25,0,12.05)">
		<g transform="translate(72,-62.36)">
			<text transform="matrix(1,0,0,-1,-71.95,63.5)">
				<tspan style="font-size: 12.50px; " x="0" y="0">2</tspan>
				<tspan style="font-size: 12.50px; " x="6.2515001" y="0">9</tspan>
				<tspan style="font-size: 12.50px; " x="15.278666" y="0">±</tspan>
				<tspan style="font-size: 12.50px; " x="26.618887" y="0">3</tspan>
				<tspan style="font-size: 12.50px; " x="32.870388" y="0">0</tspan>
			</text>

		</g>
	</g>
</svg> &#x25;, resp.; <svg style="vertical-align:-0.17555pt;width:48.337502px;" id="M3" height="10.9125" version="1.1" viewBox="0 0 48.337502 10.9125" width="48.337502"  xmlns="http://www.w3.org/2000/svg">
	<g transform="matrix(1.25,0,0,-1.25,0,10.9125)">
		<g transform="translate(72,-63.27)">
			<text transform="matrix(1,0,0,-1,-71.95,63.5)">
				<tspan style="font-size: 12.50px; " x="0" y="0">𝑃</tspan>
				<tspan style="font-size: 12.50px; " x="10.890113" y="0">=</tspan>
				<tspan style="font-size: 12.50px; " x="22.930502" y="0">.</tspan>
				<tspan style="font-size: 12.50px; " x="26.056252" y="0">0</tspan>
				<tspan style="font-size: 12.50px; " x="32.307751" y="0">3</tspan>
			</text>

		</g>
	</g>
</svg>). Using a 60&#x25; decrease in tumor FDG-uptake as a threshold for metabolic response correctly classified  3 of 4 histopathologic responders and 7 of 8 histopathologic non-responders as metabolic responders and non-responders, respectively (sensitivity, 75&#x25;; specificity, 88&#x25;). <i  >Conclusion</i>. These results suggest that changes in FDG-SUVmax at the end of neoadjuvant treatment can identify histopathologic responders and non-responders in adult primary bone sarcoma patients.

Matthias R. Benz

Johannes Czernin

William D. Tap

Jeffrey J. Eckardt

Leanne L. Seeger

Martin S. Allen-Auerbach

Sarah M. Dry

Michael E. Phelps

Wolfgang A. Weber

Fritz C. Eilber

FDG-PET/CT Imaging Predicts Histopathologic Treatment Responses after Neoadjuvant Therapy in Adult Primary Bone Sarcomas

Sclerosing epithelioid fibrosarcoma (SEF) is a rare soft tissue sarcoma mostly occurring in extraosseous sites. SEF represents a clinically challenging entity especially because no standardized treatment regimens are available. Intraosseous localization is an additional challenge with respect to the therapeutical approach. We report on a 16-year-old patient with SEF of the right proximal tibia. The patient underwent standardized neoadjuvant chemotherapy analogous to the EURAMOS-1 protocol for the treatment of osteosarcoma followed by tumor resection and endoprosthetic reconstruction. Histopathological analysis of the resected tumor showed <svg style="vertical-align:-0.30096pt;width:30.799999px;" id="M1" height="11.0625" version="1.1" viewBox="0 0 30.799999 11.0625" width="30.799999"  xmlns="http://www.w3.org/2000/svg">
    <g transform="matrix(1.25,0,0,-1.25,0,11.0625)">
        <g transform="translate(72,-63.15)">
            <text transform="matrix(1,0,0,-1,-71.95,63.5)">
                <tspan style="font-size: 12.50px; " x="0" y="0">&gt;</tspan>
                <tspan style="font-size: 12.50px; " x="12.027886" y="0">9</tspan>
                <tspan style="font-size: 12.50px; " x="18.279387" y="0">0</tspan>
            </text>

        </g>
    </g>
</svg>&#x25; vital tumor cells suggesting no response to chemotherapy. Therefore, therapy was reassigned to the CWS 2002 High-Risk protocol for the treatment of soft tissue sarcoma. To date (22 months after diagnosis), there is no evidence of relapse or metastasis. Our data suggest that SEF may be resistant to a chemotherapy regimen containing Cisplatin, Doxorubicin, and Methotrexate, which should be considered in planning treatment for patients with SEF.

Thomas G. P. Grunewald

Irene von Luettichau

Gregor Weirich

Angela Wawer

Uta Behrends

Peter M. Prodinger

Gernot Jundt

Stefan S. Bielack

Reiner Gradinger

Stefan Burdach

Sclerosing Epithelioid Fibrosarcoma of the Bone: A Case Report of High Resistance to Chemotherapy and a Survey of the Literature

Unlike other soft tissue sarcomas, myxoid/round cell liposarcoma (MRCL) has a tendency to spread to extrapulmonary sites but bone metastases are thought to be uncommon. In case reports, negative bone scintigraphy has been noted in patients with myxoid/round cell liposarcoma and bone metastases but  the prevalence and optimal method of diagnosis of bone metastases in this common subtype of liposarcoma are unclear. In an attempt to answer these questions, data were obtained from a prospective database of patients with sarcoma, including MRCL, and the diagnostic imaging used was examined. A variety of imaging tools were used including plain X-rays, bone scintigraphy, computed tomography (CT), and magnetic resonance imaging (MRI). Eight patients (4.3%) developed skeletal metastases all of which were positive on MRI. Bone scintigraphy was negative in two out of four cases, CT was negative in six out of seven, and X-rays were negative in four. Radiography and CT measure mainly cortical bone involvement, whereas MRI examines bone marrow. When investigating patients with MRCL for bone pain, negative X-rays and bone scans do not rule out bone metastases. In our experience, MRI provides the most sensitive technique for the diagnosis of bone metastases in MRCL.

J. L. Noble

E. Moskovic

C. Fisher

I. Judson

Imaging of Skeletal Metastases in Myxoid Liposarcoma

Aggressive fibromatosis (AF) or desmoid tumor is a rare condition, characterized by deep tissue invasion by a monoclonal fibroblastic neoplasm, developed from musculoaponeurotic structures. Surgery is the treatment of choice, but negative margins can hardly been achieved in large tumors, and can lead to major functional disability. AF medical therapy includes nonsteroids anti-inflammatory drugs, tamoxifen, with inconsistent results. Several reports of imatinib efficacy in AF appear in the literature. Here, we describe for the first time a V530I <i>KIT</i> exon 10 mutant that was associated to a dramatic imatinib response in an extraabdominal aggressive fibromatosis. The previously discovered V530I substitution was characterized in the core binding factor AML, but had never been reported in any other condition, so far. In this paper, we discuss the <i>KIT</i> exon 10 mutations or polymorphisms that have been described in a variety of KIT-related conditions, including acute myelogenous leukemia, mastocytosis, and aggressive fibromatosis.

Jean-Emmanuel Kurtz

Ir&#xe8;ne Asmane

Anne-Claire Voegeli

Agn&#xe8;s Neuville

Armelle Dufresne

Val&#xe8;re Litique

Christine Chevreau

Jean-Pierre Bergerat

A V530I Mutation in c-KIT Exon 10 Is Associated to Imatinib Response in Extraabdominal Aggressive Fibromatosis

Resection of large lipomatous tumours in the subdeltoid region remains technically challenging due to the risk of injury to the axillary neurovascular bundle. We describe a novel deltoid release and reinsertion technique for resection of large lipomatous tumours of the sub-deltoid region and report the functional and oncologic outcomes of six patients who underwent this procedure. Three cases were diagnosed histologically as atypical lipoma and three cases were diagnosed as lipoma. There was one local recurrence in a case of an atypical lipoma. Rotator cuff function was comparable to that of the contralateral side in all cases and the average Constant Score adopted by the European Shoulder and Elbow Society was 84 (range 81 to 92) out of 100. We conclude that patients with large sub-deltoid lipomatous tumours who undergo resection through a previously undescribed deltoid release and reinsertion technique have excellent functional outcome with a low risk for recurrence.

Emad Al Absi

Tamanna Karim

Nigel Colterjohn

Michelle Ghert

A Novel Surgical Approach to Lipomatous Tumours of the Deltoid Region

Radiotherapy (RT) is our preferred modality for local palliation of metastatic soft tissue sarcoma (STS). A short and intense course of RT is usually needed for rapid palliation and local control of metastatic disease. 
Seventeen patients at a median age of 61 had symptomatic metastatic sarcoma and required rapid palliation. The symptoms related to the metastases were either pain or discomfort. 
All patients were treated by a short and intensive course of administration: 39 Gy were given in 13 fractions of 3 Gy/day, 5 times a week.   
Median follow-up period was 25 weeks. The treatment was well tolerated. Acute side effects included grade one skin toxicity. No wound complications were noted among those undergoing surgery. Late side effects included skin pigmentation and induration of irradiated soft tissues.  Durable pain control was achieved in 12 out 15 cases treated for gross metastases.  Tumor progression was seen in the 3 other cases within a period of two to nine months. Among 5 lesions which were irradiated as an adjunctive treatment following resection, no local recurrence was observed. 
The results of this series, although limited in size, point to the safety and feasibility of hypofractionated RT for palliation of musculoskeletal metastases from sarcoma

Viacheslav Soyfer

Benjamin W. Corn

Yehuda  Kollender

Haim Tempelhoff 

Isaac Meller

Ofer Merimsky

Radiation Therapy for Palliation of Sarcoma Metastases: A Unique and Uniform Hypofractionation Experience

Extraosseous osteosarcoma (EOO) is a malignant mesenchymal neoplasm that is located in the soft tissues without direct attachment to the skeletal system and that produces osteoid, bone, or chondroid material. EOO is an extremely rare disease, accounting for only 1&#37; of soft tissue sarcomas, and typically presents in either an extremity or the retroperitoneum. This paper presents the case of a 45-year-old Caucasian male with extraosseous osteosarcoma of the esophagus.

Rodney E. Wegner

Kevin M. McGrath

James D. Luketich

David M. Friedland

Extraosseous Osteosarcoma of the Esophagus: A Case Report

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<p><em>Sarcoma</em> covers all aspects of connective tissue oncology research. It brings together work from scientists and clinicians carrying out a broad range of research in this field, including the basic sciences, molecular biology and pathology etc.</p>


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Sarcoma covers all aspects of connective tissue oncology research. It brings together work from scientists and clinicians carrying out a broad range of research in this field, including the basic sciences, molecular biology and pathology etc.

Sarcoma