滑膜肉瘤MRI影像特征与组织病理学的相关性研究

背景与目的：磁共振成像(magnetic resonance imaging,MRI)是检查软组织肿瘤最好的影像学方法。滑膜肉瘤是常见的恶性软组织肿瘤之一,本研究探讨滑膜肉瘤的MRI影像特征与病理组织学的相关性．以期提高MRI对该肿瘤的诊断水平。材料和方法：12例滑膜肉瘤均经手术病理证实,位于上肢2例,位于下肢lO例;患者年龄35～50岁,中位年龄45岁;男女比例为3：1。MRI扫描采用PHILIPS GYROSCAN 0．5T超导型磁共振成像系统．扫描序列采用SE、FSE、FFE和Stir等序列,所有病例均作T,WI、T2WI和T1W Gd-DTPA静脉内注射增强扫描检查。取手术标本进行病理学检查,比较滑膜肉瘤MRI影像学表现与病理组织学的关系。结果：MRI可见12例肿块均位于关节旁,其中2例向关节内侵犯,3例侵犯邻近的骨质。在T1W12例肿块均呈以等信号为主的肿块．但其中3例肿块可见部分斑片状和囊状高信号区。在T2W6例肿块信号表现为三重信号;6例肿块表现为多结节状,3例呈不规则状,3例为圆形或椭圆形肿块。5例肿瘤内可见分隔征象,液-液平面征l例,肿块最大直径3～13cm。T1WGd-DTPA增强肿块均呈明显不均匀性强化。12例滑膜肉瘤病理分型：低分化单相梭形细胞为主型7例,高分化3例;双相分化2例。肿块内有大范围出血和囊性变6例,大量瘤组织坏死5例,钙化2例。结论：滑膜肉瘤的MRI具有一些特征性,并和肿瘤的组织学类型具有一定相关性。     

Premonitory Pain Preceding Swelling: A Distinctive Clinical Presentation of Synovial Sarcoma which may Prompt Early Detection

Purpose: The aim of this paper is to document the unusual presentation of long-standing pain at the tumour site before development of a swelling in patients with synovial sarcoma.Patients/methods and results: The clinical presentation of 53 patients with synovial sarcoma was compared with 56 randomly selected patients with other sarcomas of the trunk and extremities. The two groups were similar with regard to age (P = 0.980), sex (P = 0.784) duration of symptoms (P = 0.697), size (P = 0.931) and site of tumour (P = 0.288). Sixteen (30.2%) patients with synovial sarcoma had pain before development of a swelling compared to two (3.6%) patients with other sarcomas (P < 0.001, odds ratio = 11.68, 95% confidence interval 2.53, 53.83). The mean duration of such pain was 37 months (median 24, range 6-120 months). The nature of the pain was variable. Eight patients had sharply localised tenderness. Calcification seen in the X-rays of four patients was initially misdiagnosed as benign lesions. A swelling was ultimately detected by MRI, CT, ultrasound or at physical examination. The mean duration from first presentation with pain till diagnosis of synovial sarcoma was 20 months. In three patients, at explorative surgery there was friable, vascular or necrotic tissue in the absence of a well-defined tumour mass.Discussion: The occurrence of long-standing pain at the tumour site prior to development of a swelling is significantly more common with synovial sarcomas than with other sarcomas. Awareness of this unusual presentation and appropriate investigation may enable detection of synovial sarcoma at a prognostically favourable early stage.     

Surgery for dedifferentiated liposarcoma, presenting two radiologically and pathologically distinctive patterns

In this paper, we report three cases with dedifferentiated liposarcoma in the lower extremities, presenting two patterns that are distinctively different radiologically and pathologically. The first case was a 67-year-old male who had discovered a mass in his left thigh, which was shown as a low and iso density mass between the muscles on computed tomography (CT). The second case was a 61-year-old male who had suffered from an intramuscular tumor with a non-fatty sarcomatous area within the fatty components as shown on magenetic resonance imaging (MRI). The third case was a 52-year-old female who had presented with an intramuscular tumor of the left thigh, consisting of a mass with fat and another soft tissue tumor adjacent to the lipomatous component as detected with CT and MRI. The final pathological diagnoses of the resected specimens in all three cases were concluded to be dedifferentiated liposarcoma, composed of well-differentiated liposarcomas and spindle and/or pleomorphic sarcomas, compatible with malignant fibrous histiocytoma. In all three cases, wide resection of the tumor was successfully carried out, and all patients have been continuously disease-free up to the most recent follow-up. Evaluation of the surgical margins for the resected specimens indicated that the safety margin for a dedifferentiated lesion should be accomplished as an adequate margin or more, and a marginal margin or more could be considered as safe only for the confined part of a well-differentiated liposarcoma.     

Retrospective audit of 957 consecutive <Superscript>18</Superscript>F-FDG PET–CT scans compared to CT and MRI in 493 patients with different histological subtypes of bone and soft tissue sarcoma

Background

The use of ¹⁸F-FDG PET–CT (PET–CT) is widespread in many cancer types compared to sarcoma. We report a large retrospective audit of PET–CT in bone and soft tissue sarcoma with varied grade in a single multi-disciplinary centre. We also sought to answer three questions. Firstly, the correlation between sarcoma sub-type and grade with ¹⁸FDG SUVmax, secondly, the practical uses of PET–CT in the clinical setting of staging (during initial diagnosis), restaging (new baseline prior to definitive intervention) and treatment response. Finally, we also attempted to evaluate the potential additional benefit of PET–CT over concurrent conventional CT and MRI.

Methods

A total of 957 consecutive PET–CT scans were performed in a single supra-regional centre in 493 sarcoma patients (excluding GIST) between 2007 and 2014. We compared, PET–CT SUVmax values in relation to histology and FNCCC grading. We compared PET–CT findings relative to concurrent conventional imaging (MRI and CT) in staging, restaging and treatment responses.

Results

High-grade (II/III) bone and soft tissue sarcoma correlated with high SUVmax, especially undifferentiated pleomorphic sarcoma, leiomyosarcoma, translocation induced sarcomas (Ewing, synovial, alveolar rhabdomyosarcoma), de-differentiated liposarcoma and osteosarcoma. Lower SUVmax values were observed in sarcomas of low histological grade (grade I), and in rare subtypes of intermediate grade soft tissue sarcoma (e.g. alveolar soft part sarcoma and solitary fibrous tumour). SUVmax variation was noted in malignant peripheral nerve sheath tumours, compared to the histologically benign plexiform neurofibroma, whereas PET–CT could clearly differentiate low from high-grade chondrosarcoma. We identified added utility of PET–CT in addition to MRI and CT in high-grade sarcoma of bone and soft tissues. An estimated 21% overall potential benefit was observed for PET–CT over CT/MRI, and in particular, in ‘upstaging’ of high-grade disease (from M0 to M1) where an additional 12% of cases were deemed M1 following PET–CT.

Conclusions

PET–CT in high-grade bone and soft tissue sarcoma can add significant benefit to routine CT/MRI staging. Further prospective and multi-centre evaluation of PET–CT is warranted to determine the actual predictive value and cost-effectiveness of PET–CT in directing clinical management of clinically complex and heterogeneous high-grade sarcomas.

     

Metastasis of sarcomatous lesion in regional lymph node

This is a retrospective study of 390 patients with early sarcoma admitted over a 13-year period. There were 182 bone lesions, 173 soft tissue sarcomas, and 35 visceral sarcomas. Two hundred ninety-two patients had surgical resections, and 77 had specimens of lymph node(s) examined histologically. Among the latter group, 6 of 47 patients with non-osseous sarcomas (13%) had metastasis in the regional node initially (subtypes include fibrohistiocytoma, leiomyosarcoma, rhabdomyosarcoma; synovial, anaplastic, and Kaposi sarcoma). Another two patients developed nodal metastasis subsequently (rhabdomyosarcoma). Among the 30 patients with skeletal sarcomas who had lymph nodes removed during resection, one case with osteogenic sarcoma and one chondrosarcoma had nodal metastasis initially (7%). During follow-up, one patient with Ewing's sarcoma and another with chondrosarcoma developed regional lymphadenopathy. Our findings are discussed and compared with incidences of metastatic sarcomatous lesion in lymph node reported in the literature.     

New concepts for the treatment of pediatric nonrhabdomyosarcoma soft tissue sarcomas

Nonrhabdomyosarcoma soft tissue sarcomas form a group of rare tumors with a different biology and clinical behavior. The recently established European Pediatric Soft Tissue Sarcoma Study Group is organizing a new study devoted specifically to these tumors that were formerly treated according to the principles derived from experience with rhabdomyosarcoma, which is a clearly distinct entity. The new study includes two prospective trials, one for synovial sarcoma and the other for adult-type nonrhabdomyosarcoma soft tissue sarcomas. While surgery remains the mainstay of treatment, the role of adjuvant therapy is not yet clear and our understanding of the biology and natural history of nonrhabdomyosarcoma soft tissue sarcomas is still incomplete. This review presents the latest data on nonrhabdomyosarcoma soft tissue sarcoma treatment and outcome, and the rationale behind a risk-adapted treatment program that investigates the role of full-dose ifosfamide-doxorubicin chemotherapy in improving the response rate of patients with unresectable disease, the chances of avoiding adjuvant chemotherapy in low-risk synovial sarcomas, and the possible role of chemotherapy in high-risk adult-type soft tissue sarcomas.     

Brain metastases in soft tissue sarcomas: case report and literature review

Background and purpose: Brain metastasis is a relatively uncommon event in the natural history of soft tissue sarcomas. The increasing use of chemotherapy may have caused a reduction in local relapses as well as distant failures leading to an improvement in survival, thereby allowing metachronous seeding of the brain, a sanctuary site. The purpose of this report is to increase awareness amongst clinicians regarding such a possibility.Patients and methods: A review of the departmental sarcoma database following the presentation of this index case in the clinic.Results and discussion: An adolescent male who had previously been treated with surgery and radiotherapy for a spindle cell sarcoma of the left thigh developed a space-occupying lesion in the brain within 6 months of treatment of the primary tumor. He subsequently underwent resection of the presumed solitary brain metastasis followed by whole brain radiotherapy. On radiation he was detected to have pulmonary metastases too, for which he was offered palliative chemotherapy. The patient died of brain metastasis within 4 months. A review of the departmental sarcoma database, restricted to soft tissue sarcomas purely, maintained prospectively from 2000 till date, could not identify any other such case.Conclusion: Brain metastases from soft tissue sarcomas are rare. Patients with neurological symptoms, however, should be appropriately investigated. Surgical resection of brain metastasis could be considered for solitary brain metastasis in non-eloquent areas. Palliative radiotherapy is appropriate for patients with multiple brain metastases or co-existing extra-cranial disease.     

New concepts for the treatment of pediatric nonrhabdomyosarcoma soft tissue sarcomas

Nonrhabdomyosarcoma soft tissue sarcomas form a group of rare tumors with a different biology and clinical behavior. The recently established European Pediatric Soft Tissue Sarcoma Study Group is organizing a new study devoted specifically to these tumors that were formerly treated according to the principles derived from experience with rhabdomyosarcoma, which is a clearly distinct entity. The new study includes two prospective trials, one for synovial sarcoma and the other for adult-type nonrhabdomyosarcoma soft tissue sarcomas. While surgery remains the mainstay of treatment, the role of adjuvant therapy is not yet clear and our understanding of the biology and natural history of nonrhabdomyosarcoma soft tissue sarcomas is still incomplete. This review presents the latest data on nonrhabdomyosarcoma soft tissue sarcoma treatment and outcome, and the rationale behind a risk-adapted treatment program that investigates the role of full-dose ifosfamide–doxorubicin chemotherapy in improving the response rate of patients with unresectable disease, the chances of avoiding adjuvant chemotherapy in low-risk synovial sarcomas, and the possible role of chemotherapy in high-risk adult-type soft tissue sarcomas.     

Fluorescence in Situ Hybridization (FISH) for Differential Diagnosis of Soft Tissue Sarcomas

Introduction:Soft tissue sarcomas are rare tumors comprising 1 percent of solid malignancies. The latest editionof WHO soft tissue pathology lists 94 benign and malignant soft tissue tumors. Many of these show a large degreeof morphological overlap. Immunohistochemistry has been shown to be reliable in many cases for differential diagnosisof lesions, although cytogenetic tests are considered the gold standard for many entities.Fluorescence in-situ hybridization(FISH) is a cytogenetic technique that uses fluorescent probes that bind to only those parts of the chromosome whichhave a high degree of sequence complementarity. Many soft tissue tumors show recurrent genetic mutations that arenow being used as diagnostic markers. Knowledge of the molecular identity allows prediction of behavior, prognosisand treatment response. Objective:The aim of this study was to identify genetic mutations in soft tissue sarcomas usingFISH testing and to assess correlations with histological diagnosis. Material and methods:A total of 25 cases of differentsoft tissue sarcomas diagnosed on histology with the help of immunohistochemical staining and for which FISH studieswere requested were included in this study. Three pathologists with a special interest in soft tissue sarcomas reviewedthe cases. FISH tests for EWS, the X:18 translocation, FOXO1 and MDM2 were respectively applied for 8 cases ofEwing sarcoma, 8 cases of synovial sarcoma, 2 cases of rhabdomyosarcoma and 7 cases of dedifferentiated liposarcomaand atypical lipomatous tumors/well differentiated liposarcomas. Results:EWS gene fusion was detected in 7 out of8 cases of Ewing sarcoma and the X:18 translocation was positive in 3 of the 8 cases of synovial sarcoma. FOXO1was not detected in either of the two rhabdomyosarcomas. MDM2 by FISH was detected in only one out of 5 cases ofatypical lipomatous tumors and 1 out of 2 dedifferentiated liposarcomas. Conclusion: FISH is a useful adjunct in thediagnostic assessment of different types of soft tissue sarcomas. It is easy to set up, is relatively inexpensive and hasthe ability to diagnose sarcomas with great accuracy, especially in cases which can not be accurately classified evenafter thorough histological and immunohistochemical evaluation. It may play a very important role in the accuratediagnosis and correct management of patients.     

Dysgerminoma of the ovary with rhabdomyosarcoma. Report of a case

A 14-year-old girl with a large left ovarian mass underwent excision of the tumor which on histologic examination revealed dysgerminoma mixed with elements of rhabdomyosarcoma. A subsequent staging laparotomy revealed a solitary 1-cm nodule along the pelvic wall. This was composed of rhabdomyosarcoma. Review of literature revealed that soft tissue sarcomas of several types may on occasion be seen in association with gonadal and extragonadal mixed germ cell tumors or in spermatocytic seminoma of the testis. However, no previously published report of a sarcoma arising in a pure dysgerminoma was found in the literature.     

Matrix metalloproteinase expression in high grade soft tissue sarcomas

Soft tissue sarcomas comprise a heterogeneous group of mesenchymal tumors with varying biological behavior ranging from indolent tumors with no or minimal metastatic risk to aggressive and frequently metastasizing tumors. Among the more common aggressive adult soft tissue sarcomas are malignant fibrous histiocytoma, synovial sarcoma and liposarcoma. Matrix metalloproteinases are enzymes which perform a homeostatic role in mesenchymal tissue and function in both tumorigenesis and metastasis. The objectives of this study are to determine the presence and relative quantity of matrix metalloproteinases (MMPs) -1, -2, -8, -9, and -13; extracellular matrix metalloproteinase inducer (EMMPRIN); and tissue inhibitors of matrix metalloproteinases (TIMP)-1 and -2 in high grade soft tissue sarcoma tumor specimens using real-time PCR. The second objective is to determine if a relationship exists between quantity of EMMPRIN, MMPs, and TIMPs expressed in tumor tissue and disease-free survival. One hundred and forty patients diagnosed with high grade soft tissue sarcomas between 1995-2003 were identified. Tissue blocks and histologic slides were acquired for 41 specimens. Tumor specimens included 29 malignant fibrous histiocytomas, 3 liposarcomas and 11 synovial sarcomas. RNA was extracted and RT-PCR was performed in triplicate. No significant differences were found between the three types of high grade soft tissue sarcomas studied and the expression of MMPs. Interestingly, no relationship was found between high or low levels of MMPs when compared with disease-free survival. Our data support other research which finds variable correlation between MMP expression in soft tissue sarcomas and disease-free survival. We assert that the difference in correlation between MMP expression in carcinomas and sarcomas and disease-free survival is based on the vast phenotypic and genotypic difference between the cells of origin.     

Primary breast synovial sarcoma: a rare primary breast neoplasm

A primary synovial sarcoma based on the breast is rare. The usual tumours on the breast are carcinomas. Synovial sarcomas account for about 6–9% of soft tissue sarcomas and most commonly develop in the extremity of young adults (80%). The other 20% of synovial sarcomas can arise in non-extremity sites (trunk 8%, retroperitoneal/abdominal 7%, head and neck 5%) but synovial sarcomas can develop in almost any other anatomical location. We report a case of a young woman who presented with a suspected common breast tumour and started treatment of this tumour with carcinoma neoadjuvant chemotherapy. We were surprised when the pathologist identified a synovial sarcoma in the histopathology study.     

A t(X; 18) SYT-SSX2 positive synovial sarcoma in the pelvis of a young adult male: a rare case report with review of literature

Synovial sarcoma is uncommonly documented in the pelvis. Rarely, such cases have dealt with molecular analysis. A 19-year-old boy presented with pain and swelling in his left lower limb of two months duration. He developed acute urinary retention four days prior to his hospital admission, wherein radiological examination unraveled a large soft tissue mass, displacing his pelvic muscles, along with a lytic lesion involving his right pubic bone. Biopsy showed a cellular spindle cell sarcoma, exhibiting hemangiopericytoma-like vascular pattern with focal necrosis. Immunohistochemistry (IHC) showed positivity for vimentin, BCL-2, calponin and MIC 2. Cytokeratin (CK) and epithelial membrane antigen (EMA) were negative. MIB 1 count was 70% (high). P53 was positive. Diagnosis of a poorly differentiated synovial sarcoma was offered and confirmed with a positive t(X; 18) SYT-SSX2 translocation. This case highlights the value of molecular analysis in diagnosis of a synovial sarcoma at rare sites, especially when IHC results are equivocal and the biopsy material is limited.     

Surgical considerations for management of distal extremity soft tissue sarcomas

PURPOSE OF REVIEW: This review focuses on the surgical management of soft tissue sarcomas of the hands and the feet. With recent advances in limb salvage surgery and radiotherapy delivery, local control of soft tissue sarcoma in the extremity has become optimized, and the associated functional results of this treatment have taken on extreme importance. Techniques to limit the amount of normal tissue resected and to reconstruct the resulting defects are critical to the final functional result. RECENT FINDINGS: Several features of soft tissue sarcoma unique to the hand and foot have been reported. Certain histologic subtypes of soft tissue sarcoma have been noted to arise preferentially in the hand and the foot, such as epithelioid sarcoma, clear cell sarcoma, and synovial sarcoma. Patients with hand and foot sarcomas have been described as having improved overall survival, but this is likely a result of the smaller size of tumors arising in these locations. Reconstruction of bone defects using various techniques, vascular reconstruction, tendon transfers, and soft tissue reconstruction using regional flaps in the hand and free flaps in the foot have resulted in good functional outcomes. Amputation and early prosthetic fitting still have a role in management of some soft tissue sarcomas, most frequently in the foot. SUMMARY: Limb salvage remains the standard of care for extremity soft tissue sarcomas. Given the fact that patients have good oncologic and functional outcomes with limb salvage in tumors in the hand and foot, surgical oncologists should have this goal for each patient.     

腺泡状软组织肉瘤的影像学特征及临床病理表现

背景与目的:腺泡状软组织肉瘤罕见,其影像学表现至今尚未见系统研究,本文旨在探讨腺泡状软组织肉瘤(alveolar soft part sarcomas,ASPS)的临床特征和影像学表现,以提高诊断的准确率。方法:回顾性分析10例经病理证实的腺泡状软组织肉瘤的临床特征和影像学表现,所有患者(术前或活检前)均行X线、CT或MR检查。其中,9例行X线平片检查,9例行CT检查,6例行MRI检查。所有切除或活检组织均行HE染色,5例患者有比较完整的免疫组化结果。结果:ASPS发病年龄较轻,80%(8/10)为30岁以下。多表现为无痛性肿块。3例就诊时已有肺转移。70%(7/10)发生于下肢深部软组织以及臀部。另3例分别位于胸壁、颈部及眼眶内。ASPS的CT表现为软组织肿块影,增强后呈明显不均匀强化。MRI表现为T1WI等或略高信号,T2WI高信号,肿瘤内外可见血管流空信号,增强后肿瘤呈不均匀明显强化。镜下ASPS是由嗜伊红色的大多边形上皮样细胞组成,呈特征性的器官样或腺泡状排列,腺泡之间为衬覆单层扁平内皮细胞的裂隙状或血窦样毛细血管网。免疫组化显示3例神经元特异性烯醇化酶(NSE)阳性,两例抗淀粉酶消化染色(PAS)阳性,1例MyoD1横纹肌特异肌调节蛋白阳性(胞质染色),1例Desmin结蛋白阳性。结论:ASPS虽然是少见软组织肉瘤,但影像学很有特点,结合临床、影像和病理表现是诊断的关键。     

Long‐standing Morel‐Lavallée lesion in the proximal thigh: Ultrasound and MR findings with surgical and histopathological correlation

A 65‐year‐old man presented with a soft mass in his proximal right thigh. Ultrasonography showed a well‐defined anechoic lesion with slightly internal echoes. On MRI, the mass was hypointense and minimally hyperintense compared with muscle at T1 and hyperintense at T2, with a hypointense peripheral rim on both sequences. No signal loss was observed on T1‐weighted fat‐suppression MRI. The clinical setting, imaging findings and histopathological features were consistent with a long‐standing Morel‐Lavallée lesion.     

Surgery of soft tissue sarcomas in children

Surgery of soft tissue sarcomas in children has been modified by the introduction of multidisciplinary treatment so that major amputation and exenterations are now rare in the management of these tumors in children. Surgery must still be well planned for total removal of the tumor with preservation of limbs and, in most instances, the function of pelvic organs. The commonest soft tissue sarcomas in children are embryonal rhabdomyosarcoma (RMS), fibrosarcoma, and synovial sarcoma. Treatment of embryonal RMS can now achieve a 2-year survival of 80% at all sites. Surgery and irradiation are used to control the primary tumor and multidrug chemotherapy to control metastases or prevent dissemination of localized tumor. Fibrosarcoma in children, usually a low-grade, extra-abdominal desmoid lesion, does not respond to radiation treatment or chemotherapy, and management is by surgery alone. It shows a marked tendency to local recurrence, and multiple local resections may be necessary for cure. Ninety percent of the children with this tumor can be salvaged by surgery and careful follow-up. The management of synovial sarcoma is surgical and similar to that of RMS. Although not radiosensitive as is RMS, this tumor has responded well to multidisciplinary treatment. Adequate resection is now followed by a chemotherapy protocol similar to that used in osteogenic sarcoma. The smaller numbers of these tumors and their varied natural history make evaluation of treatment difficult. Other soft tissue sarcomas seen with extreme rarity in children are liposarcoma, angiosarcoma, and neurosarcoma. These tumors are treated with the same protocol as that of RMS.     

Incidence, épidémiologie des sarcomes et biologie moléculaire. Résultats préliminaires de l’étude EMS en Rhône-Alpes

Sarcomas comprise a heterogeneous group of mesenchymal neoplasms. They can be grouped into 3 general categories, soft tissue sarcoma, visceral and primary bone sarcoma, which have different staging and treatment approaches. Soft tissue sarcomas are typically classified on the basis of genetic alterations and light-microscopic examination of hematoxylineosin-stained tissue, in which recognizable morphological characteristics of normal tissues are identified. Sarcomas are further characterized by histologic grade. The 3 most important prognostic variables are grade, size, and location of the primary tumor. This review includes a discussion of both soft tissue sarcomas (unclassified sarcoma, liposarcoma, leiomyosarcoma, synovial sarcoma, dermatofibrosarcoma protuberans, angiosarcoma, Kaposi sarcoma, gastrointestinal stromal tumor, rhabdomyosarcoma, …) and primary bone sarcomas (osteosarcoma, Ewing sarcoma and chondrosarcoma). The approach to a patient with a sarcoma begins with a biopsy that obtains adequate tissue for diagnosis without interfering with subsequent optimal definitive surgery. Subsequent treatment depends on the specific type of sarcoma. Due to the absence of clear knowledge for incidence rate, we conducted in 2005 and 2006 an exhaustive analysis of all diagnosed cases in the Rhône-Alpes region. Because sarcomas are relatively uncommon yet comprise a wide variety of different entities, second opinion was systematically performed for all included cases.     

Solitary bone metastasis from myxoid liposarcoma

BACKGROUND: Extrapulmonary metastasis is more common in liposarcoma than in other soft tissue sarcomas. However, osseous metastases are rare. CASE REPORT: We report the case of a 61-year-old woman with a solitary histologically proven bone metastasis of the right femur from primary myxoid liposarcoma of the left thigh. In September 2000 resection of the primary tumor was performed. Histology showed a high-grade liposarcoma with round cell differentiation. Postoperative radiotherapy with 60 Gy was performed. In a follow-up MRI examination 11 months after initial diagnosis, a suspicious formation in the marrow space of the right proximal femur was detected. CT-guided biopsy was performed and histology showed a metastasis from liposarcoma. PET examination confirmed the metastasis as solitary. Curettage of the metastasis was performed followed by radiotherapy with 60 Gy. The patient was without evidence of disease in the last follow-up. CONCLUSIONS: No standard treatment exists for the management of solitary extrapulmonary metastases from soft tissue sarcoma. Interdisciplinary cooperation is advised and the therapy concept should be chosen individually.