Soft Tissue Sarcomas
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Soft tissue sarcomas are malignant tumours that may arise in any of the mesodermal tissues (muscles, tendons, vessels that carry blood or lymph, joints, and fat). Sarcomas are a diverse range of tumours, they are named after the type of soft tissue cell they arise from. Types of soft tissue sarcomas include; alveolar soft-part sarcoma, angiosarcoma, fibrosarcoma, leiomyosarcoma, liposarcoma, malignant fibrous histiocytoma, hemangiopericytoma, mesenchymoma, schwannoma, peripheral neuroectodermal tumours, rhabdomyosarcoma, synovial sarcoma, and other types. In terms of treatment these different sub-types are usually treated in the same way using a uniform soft tissue protocol.

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Information for Patients and the Public
Information for Health Professionals / Researchers
Latest Research Publications
Childhood Soft Tissue Sarcoma
Uterine Sarcoma
Kaposi Sarcoma
Gastrointestinal Stromal Tumors

Information Patients and the Public (16 links)

Information for Health Professionals / Researchers (19 links)

Latest Research Publications

This list of publications is regularly updated (Source: PubMed).

Donfut AL, Lemaitre J, Van de Walle H, et al.
Primitive pulmonary "malignant epithelioid hemangioendothelioma" versus epithelioid angiosarcoma. A case report and review of the literature.
Acta Chir Belg. 2014 Mar-Apr; 114(2):143-5 [PubMed] Related Publications
We describe the case of a 56-year-old man presenting a primary pulmonary epithelioid angiosarcoma versus malignant epithelioid hemangioendothelioma still alive, without recurrence at nearly two years after the beginning of the symptoms. The primary pulmonary angiosarcoma is extremely rare, being reported only in a handful of cases. Metastatic involvement of the lung (90%) is far more common than primary pulmonary involvement (10%). Various predisposing condition for the development of angiosarcoma have been described. Early diagnosis is not common, because of the rarity of angiosarcoma in the lung and consequent low index of suspicion. Due to the paucity of cases, there are no defined treatment regimens for this entity. However, there is a tendency for surgical intervention in all reported cases.

Related: Lung Cancer

Nappi L, Mele G, Angioni S, et al.
Uterine leiomyosarcoma: report of three cases and review of the literature.
Eur J Gynaecol Oncol. 2014; 35(3):328-31 [PubMed] Related Publications
This is the report of three cases of unsuspected uterine leiomyosarcoma diagnosed by pathologist after hysteroscopic resection. The literature on this issue has been reviewed. Mesenchymal uterine tumors are rare malignancies, occurring in only 17 per one million women annually. The three most common variants of uterine sarcoma are endometrial stromal sarcoma, leiomyosarcoma, and malignant mixed Müllerian tumour. Less than one percent of women believed to have a leiomyoma actually have a sarcoma at hysterectomy. According to the authors' experience and the available literature reviewed, the removal of the whole myomatosus lesion, even if its appearance suggests a typical submucosal myoma, represents the only method to definitively rule out the presence of sarcomatous tissue.

Barone A, Ambrosio MR, Rocca BJ, et al.
Myxoid leiomyosarcoma of the uterus: a case report.
Eur J Gynaecol Oncol. 2014; 35(3):322-4 [PubMed] Related Publications
Only 30 cases of myxoid leiomyosarcomas (MLMS) have been reported to date. The authors describe a further case in a 66-year-old woman. The main differential diagnoses include: myxoid inflammatory myofibroblastic tumours, mixoid leiomyoma, and endometrial stromal tumours. Surgery remains the appropriate treatment. However, in spite of an aggressive surgical approach and local and systemic control, recurrences and metastasis are frequent.

Wu PC, Yue CT, Huang SC
Complete response after MAID treatment for advanced primary ovarian angiosarcoma: case report and literature review.
Eur J Gynaecol Oncol. 2014; 35(3):318-21 [PubMed] Related Publications
The patient presented in this case report was a 45-year-old female, with a Stage IIIA ovarian angiosarcoma combined with mature teratoma, that underwent debulking surgery and achieved complete remission for 11 months after six cycles of MAID chemotherapy (mesna, adriamycin/doxorubicin, ifosfamide, and dacarbazine). Thereafter, she had tumor recurrence with peritoneal seeding and massive pleural effusion; hence she received chemotherapy again. Although she had been undergoing a series of chemotherapies, the tumor continued to progress. Hence, she refused further chemotherapy since September 2012. Unfortunately, she passed away in January 2013 due to severe dyspnea with wide spread tumor progression. She had the longest survival period (31 months) and complete remission period than the other advanced primary ovarian angiosarcoma cases ever reported in the literature.

Related: Dacarbazine Doxorubicin Ifosfamide Mesna Ovarian Cancer

Kozakiewicz B, Dmoch-Gajzlerska E, Roszkowska-Purska K
Carcinomas and sarcomas of Bartholin gland. A report of nine cases and review of the literature.
Eur J Gynaecol Oncol. 2014; 35(3):243-9 [PubMed] Related Publications
UNLABELLED: The greater vestibular gland, also called Bartholin's gland after the Danish anatomist Caspar Bartholin the Younger who first described it in the 17th century, is the site of tumours arising from different types of epithelium and characterized by a different clinical course. In the years 1980-2009, 1,296 patients with vulvar carcinoma were treated at the Oncology Centre in Warsaw, Poland and nine of them had carcinoma of Bartholin's gland, including three patients with squamous cell carcinoma (SCC), three patients with adenoid cystic carcinoma (ACC) and three patients with sarcoma. In this paper the authors present the signs and symptoms, clinical course, treatment outcomes, and recurrence of these three malignant tumours of different histopathology. Own observations and evaluation of treatment results are compared with published reports from other centres. Interestingly, there is no consensus regarding diagnostic criteria or a uniform approach to management. Relatively poor knowledge of malignant tumours of Bartholin's gland seems to be responsible for delays in proper diagnosis and hence optimal management. When instituted, the treatment is usually aggressive and involves adjuvant radio- and chemotherapy, while the chances of longer disease-free survival after treatment may be compromised.
CONCLUSION: Bartholin sarcomas grow fast and invasive, SCC, and ACC infiltrate slowly and systematic. All types are curable at high interest rates if they are originally from the surgically removed lymph nodes on both sides and irradiated.

Related: Vulva Cancer

Brunetti AE, Delcuratolo S, Lorusso V, et al.
Third-line trabectedin for a metastatic desmoplastic small round cell tumour treated with multimodal therapy.
Anticancer Res. 2014; 34(7):3683-8 [PubMed] Related Publications
BACKGROUND: Desmoplastic small round cell tumour (DSRCT) is a rare and aggressive cancer that usually develops in the peritoneal cavity of young males. Its prognosis is dismal, with current treatment options including the combination of multi-agent chemotherapy, aggressive surgery, radiation therapy, and autologous stem cell transplantation. Hyperthermic intraperitoneal chemotherapy (HIPEC) may also be an option.
CASE REPORT: Herein we report the administration of the marine-derived multi-target antineoplastic agent, trabectedin, in a patient with DSRCT, heavily pre-treated with conventional multi-agent chemotherapy, HIPEC, and surgery.
RESULTS: The patient achieved a prolonged partial response and an extended period of stable disease with third-line trabectedin, following disease progression after conventional multi-agent chemotherapy, HIPEC, and surgery.
CONCLUSION: Trabectedin may be a treatment option in multimodal therapy for the management of DSRCT and warrants further research to explore the impact of trabectedin in the treatment of this disease.

Related: Desmoplastic Small Round Cell Tumor Trabectedin

Gurjar V, Bharaney R
Giant phyllode tumour (cystosarcoma) of breast: a rare size recurrent breast lump.
J Indian Med Assoc. 2013; 111(9):618, 620 [PubMed] Related Publications
Phyllode tumour is a rare fibro-epithelial lesion that accounts for less than 1% of all neoplasms. It occurs in females in the 3rd and 4th decades. In 15% of patients local recurrence occurs and in 20% of patients distal metastasis is seen. Here we are presenting a case of a very rare size recurrent phyllode tumour with malignant changes which was associated with contralateral breast fibro-adenoma. Patient was investigated and operated successfully for right sided mastectomy for recurrent giant phyllode tumour and wide excision for opposite side fibro-adenoma.

Related: Breast Cancer

Horevoets J, Devos B
Angiosarcoma of the breast: case report and review of the literature.
Acta Chir Belg. 2013 May-Jun; 113(3):223-7 [PubMed] Related Publications
Skin lesions in a post-radiated breast should always be considered as possible angiosarcoma even if they mimic a traumatic lesion. Early detection by excisional biopsy followed by a mastectomy is necessary because of the high numbers of relapse if locally treated and possible deadly metastasis. We present a case of a cutaneous angiosarcoma occurred at lumpectomy site in a patient with a history of breast cancer and radiation of the breast and provide a review of the literature.

Related: Breast Cancer

Naik S, Rao D, Hegde S
Malignant fibrous histiocytoma clinically mimicking dental abscess in a child: report of an unusual case.
J Mass Dent Soc. 2014; 63(1):32-4, 36 [PubMed] Related Publications
Swellings of the orofacial tissues arising from infections of dental origin are relatively common. Occasionally a similar presentation may be due to a malignant tumor, and oral clinicians must always bear this possibility in mind with facial swellings that do not respond to conventional therapy Malignant fibrous histiocytoma is a high-grade aggressive sarcoma, which is relatively rare in children and uncommon in both the head-and-neck and mandibular regions. This article describes an unusual case of malignant fibrous histiocytoma involving the mandible of an 11-year-old child. The purpose of this article is to emphasize the importance of diagnosing such lesions in the early stage to improve prognosis and limit complications.

Hisaoka M
Lipoblast: morphologic features and diagnostic value.
J UOEH. 2014; 36(2):115-21 [PubMed] Related Publications
Lipoblasts are conceptually a precursor or immature form of adipocytes and histologically defined as lipid-containing, mono- or multivacuolated cells possessing hyperchromatic, indented or often scalloped nuclei. They are essentially identified in neoplastic conditions and assumed to recapitulate, to some extent, the differentiation process of normal fat (adipogenesis) like their potential normal counterpart, preadipocyte or preadipose cell. Traditionally, great emphasis has been placed on the identification of lipoblasts in diagnostic pathology, particularly of liposarcoma. However, it is not always an easy task for pathologists because of a variety of histological mimics such as Lochkern cells, brown fat cells and pseudolipoblasts. Currently, lipoblasts are not a prerequisite for the diagnosis of liposarcoma partly because of some benign tumors harboring lipoblasts or lipoblast-like cells such as spindle cell/pleomorphic lipoma and chondroid lipoma, although their presence is still crucial for proper diagnosis. This review summarizes the clinicopathologic features of lipoblasts, their histological mimics and representative benign tumors carrying lipoblasts to facilitate routine pathology practice and to avoid erroneous diagnosis of liposarcoma.

Fu W, Asp P, Canter B, Dynlacht BD
Primary cilia control hedgehog signaling during muscle differentiation and are deregulated in rhabdomyosarcoma.
Proc Natl Acad Sci U S A. 2014; 111(25):9151-6 [PubMed] Article available free on PMC after 24/12/2014 Related Publications
The primary cilium acts as a cellular antenna, transducing diverse signaling pathways, and recent evidence suggests that primary cilia are important in development and cancer. However, a role for cilia in normal muscle development and rhabdomyosarcoma (RMS) has not been explored. Here we implicate primary cilia in proliferation, hedgehog (Hh) signaling, and differentiation of skeletal muscle cells. Cilia and Hh signaling are highly dynamic during the differentiation of myoblasts. We show that cilia are assembled during the initial stages of myogenic differentiation but disappear as cells progress through myogenesis, concomitant with the destruction of proteins critical for cilia assembly and shortly after the Hh effector, Gli3, leaves the cilium. Importantly, we show that ablation of primary cilia strongly suppresses Hh signaling and myogenic differentiation while enhancing proliferation. Interestingly, our data further indicate that both cilia assembly and Hh signaling are deregulated in RMS, and cilia respond to Hh ligand in certain subsets of RMS cells but not others. Together, these findings provide evidence for an essential role for both primary cilia assembly and disassembly in the control of Hh signaling and early differentiation in muscle cells. We suggest that the temporally orchestrated destruction of centrosomal and ciliary proteins is a necessary antecedent for removal of the primary cilium and cessation of Hh signaling during myogenic differentiation. Additionally, our results further stratify RMS populations and highlight cilia assembly and disassembly as potential RMS drug targets.

Related: Rhabdomyosarcoma Signal Transduction

Park KS, Kim HS, Cho YJ, et al.
Vascular soft-tissue sarcomas: a prognostic model from a retrospective single-center study.
Oncology. 2014; 86(5-6):329-35 [PubMed] Related Publications
BACKGROUND: Vascular soft-tissue sarcomas are a rare form of sarcoma. Malignant subtypes exhibit a variety of biological behaviors. We describe the clinicopathological characteristics and patient survival outcomes of malignant vascular soft-tissue sarcomas.
METHODS: We conducted a retrospective study on a cohort of 84 patients diagnosed with vascular tumors by histological examination at the Yonsei University College of Medicine between April 1987 and August 2011. The primary end point was overall survival (OS).
RESULTS: The angiosarcoma patients had a significantly shorter OS than the patients with other subtypes of sarcomas (59.0 and 142.7 months, respectively; p < 0.001). Upon multivariate analysis of survival in patients who underwent surgical resection, the following independent prognostic factors were identified: primary site (trunk, p = 0.001), age (older than 65 years, p < 0.001), pathology (angiosarcoma, p = 0.006) and R2 resection (p = 0.002).
CONCLUSION: The independent prognostic factors for shorter survival are the trunk as the primary site, malignant angiosarcoma and age (>65 years). Complete excision should be attempted for providing a survival advantage in the patients with localized disease. In addition, bleeding episodes are much more frequent in patients with a poor survival outcome.

Kimura H, Yamamoto N, Nishida H, et al.
Synovial sarcoma in knee joint, mimicking low-grade sarcoma confirmed by molecular detection of SYT gene split.
Anticancer Res. 2014; 34(6):3105-11 [PubMed] Related Publications
A 10-year-old boy underwent arthroscopic curettage for an intra-articular mass in knee joint. The tumor was diagnosed as low-grade fibrous sarcoma. Five years later, the patient presented with a recurrent tumor. The patient underwent a marginal excision with knee joint preservation and without adjuvant therapy. Two years after the last surgery, the patient is thriving with no evidence of recurrent or metastatic disease. The final diagnosis was synovial sarcoma confirmed via a SYT gene split performed with fluorescent in situ hybridization (FISH), although the tumor appeared as a low-grade fibrous type in a hematoxylin-eosin section. The first curetted specimen was also confirmed to bear a SYT gene split. Synovial sarcoma has been conventionally recognized as a high-grade sarcoma. Our patient had a tumor that exhibited the characteristics of both a histologically and clinically low-grade tumor. From the present case, we consider that low-grade variants of synovial sarcoma do exist although their existence remains controversial.

Related: FISH Synovial Sarcoma SS18 gene

Quan P, Moinfar F, Kufferath I, et al.
Effects of targeting endometrial stromal sarcoma cells via histone deacetylase and PI3K/AKT/mTOR signaling.
Anticancer Res. 2014; 34(6):2883-97 [PubMed] Related Publications
AIM: Endometrial stromal sarcoma (ESS) is a rare gynecological mesenchymal malignancy with only few therapeutic options. This study aimed to investigate the efficacy of the histone deacetylase (HDAC) inhibitor suberanilohydroxamic acid (SAHA) combined with inhibitors of the phosphoinositid-3-Kinase (PI3K) pathway in ESS therapy.
MATERIALS AND METHODS: The effects of SAHA combined with inhibitor of PI3K (LY294002, LY), mammalian target of rapamycin mTOR (rapamycin), and their combination on cell growth and the PI3K pathway in two ESS cell lines (ESS-1 and MES-SA) and one non-neoplastic cell line HESC, were investigated.
RESULTS: SAHA reduced growth of the three cell lines by inhibiting protein kinase B AKT and mTOR/p70S6K cascade activation. SAHA combined with LY or rapamycin, or both, synergistically reduced p-p70S6K and p-4E-BP1 levels. SAHA combined with LY and rapamycin led to the strongest growth inhibition and slowest growth recovery among the combination treatments.
CONCLUSION: SAHA combined with inhibition of PI3K and mTOR could represent an efficient therapy option for patients with ESS.

Related: Apoptosis Endometrial (Uterus) Cancer Endometrial Cancer AKT1 Signal Transduction

Nason GJ, Baker JF, Seoighe D, et al.
Congenital-infantile fibrosarcoma of the foot--avoidance of amputation.
Ir Med J. 2014; 107(5):148-9 [PubMed] Related Publications
Congenital-infantile fibrosarcoma is a rare entity with a five year survival rate of over 90%. Surgery is still the most common treatment modality with amputation often necessary. There have been reports supporting the use of neoadjuvant chemotherapy to debulk the tumour in an effort to facilitate limb sparing surgery. We report a case of a newborn who presented with a life threatening haemorrhage from a fibrosarcoma of the foot, successfully treated with Vincristine, Actinomycin and Cyclophosphamide (VAC) chemotherapy alone.

Related: Cyclophosphamide Dactinomycin Vincristine

Mazzucchelli R, Galosi AB, Scarpelli M, et al.
Contemporary update on pathology-related issues of adult renal neoplasms.
Anal Quant Cytopathol Histpathol. 2014; 36(1):1-8 [PubMed] Related Publications
This review gives an update on selected issues on renal neoplasia with special references to emerging new tumor entities (thyroid-like follicular renal cell carcinoma, succinic dehydrogenase B deficiency-associated renal cell carcinoma, and anaplastic lymphoma kinase [ALK] translocation renal cell carcinoma), tumor grading (the International Society of Urological Pathology grading system), and assessment of tumoral involvement of the renal sinus structures, including the sinus fat, the loose connective tissue, or any sinus-based endothelium-lined space.

Related: Kidney Cancer

Nicolas MM, Nazarullah A, Guo CC
Sarcomatoid urothelial carcinoma with chondrosarcomatous differentiation of the ureter: a case report.
Anal Quant Cytopathol Histpathol. 2014; 36(2):111-6 [PubMed] Related Publications
BACKGROUND: Sarcomatoid urothelial cell carcinoma of the urinary tract has a poor prognosis. Most of the reported cases of sarcomatoid urothelial cell carcinomas are those from the urinary bladder. A limited number of these tumors originate from the ureter.
CASE: We describe a ureteral sarcomatoid urothelial carcinoma in a 63-year-old man who underwent nephroureterectomy with bladder cuff. The malignant epithelial elements consisted of undifferentiated polygonal cells and areas of glandular formation. Urothelial carcinoma in situ was present in the overlying mucosa. The mesenchymal components were pleomorphic spindle cells and atypical chondrocytes within lacunae with multinucleation and mitoses. The tumor extended beyond the muscularis into the periureteral adipose tissue. The tumor recurred after 6 months in the retroperitoneum and presacral area. The patient received chemotherapy and radiotherapy but died 16 months after the initial diagnosis.
CONCLUSION: Sarcomatoid urothelial carcinoma of the ureter is uncommon. Even rarer is the presence of malignant heterologous elements such as chondrosarcoma. The case described here underscores the aggressive nature of these neoplasms.

Related: Chondrosarcoma

Goedhart LM, Ploegmakers JJ, Kroon HM, et al.
The presentation, treatment and outcome of periosteal chondrosarcoma in the Netherlands.
Bone Joint J. 2014; 96-B(6):823-8 [PubMed] Related Publications
In this case study, we describe the clinical presentation and treatment of 36 patients with periosteal chondrosarcoma collected over a 59-year period by the archive of the Netherlands Committee on Bone Tumours. The demographics, clinical presentation, radiological features, treatment and follow-up are presented with the size, location, the histological grading of the tumour and the survival. We found a slight predominance of men (61%), and a predilection for the distal femur (33%) and proximal humerus (33%). The metaphysis was the most common site (47%) and the most common presentation was with pain (44%). Half the tumours were classified histologically as grade 1. Pulmonary metastases were reported in one patient after an intra-lesional resection. A second patient died from local recurrence and possible pulmonary and skin metastases after an incomplete resection. It is clearly important to make the diagnosis appropriately because an incomplete resection may result in local recurrence and metastatic spread. Staging for metastatic disease is recommended in grade II or III lesions. These patients should be managed with a contrast-enhanced MRI of the tumour and histological confirmation by biopsy, followed by en-bloc excision.

Related: Bone Cancers Chondrosarcoma

Kazarin O, Ilan N, Naroditzky I, et al.
Expression of heparanase in soft tissue sarcomas of adults.
J Exp Clin Cancer Res. 2014; 33:39 [PubMed] Article available free on PMC after 24/12/2014 Related Publications
BACKGROUND: Heparanase is an endo-β-D-glucuronidase that cleaves heparan sulfate chains of proteoglycans, resulting in the disassembly of the extracellular matrix. Heparanase has a central role in the development of various tumors, and its expression has been associated with increased tumor growth, angiogenesis and metastasis, but there is insufficient information about the function of heparanase in sarcomas.
STUDY AIMS: 1) To evaluate heparanase levels in adult soft tissue sarcomas (STS); 2) To examine the correlation between heparanase levels and pathological and clinical parameters and treatment outcome.
METHODS: Pathological specimens of primary or metastatic STS were subjected to immunohistochemical analysis applying an anti-heparanase antibody. The clinical and the pathological data, together with the data of heparanase levels, were evaluated in a logistic regression model for tumor recurrence and survival.
RESULTS: One hundred and one samples were examined, 55 from primary tumors and 46 from metastatic sites. A high expression of heparanase was observed in 29 (52.7%) and 22 specimens (47.8%), respectively. There was no statistically significant difference between heparanase expressions in the primary vs. metastatic sites of tumors. Moreover, no correlation was observed between heparanase staining and tumor aggressiveness, tumor recurrence or patient survival in various groups of patients.
CONCLUSION: Expression of heparanase was observed in 50% of the STS, in various histological subtypes. A larger study with homogenous groups of specific sub-types of STS or stages of disease is required to validate over-expression of heparanase as a marker of disease aggressiveness.

Davis JL, Grenert JP, Horvai AE
Loss of heterozygosity and microsatellite instability are rare in sporadic dedifferentiated liposarcoma: a study of 43 well-characterized cases.
Arch Pathol Lab Med. 2014; 138(6):823-7 [PubMed] Related Publications
CONTEXT: Defects in mismatch repair proteins have been identified in Lynch syndrome-associated liposarcomas, as well as in rare sporadic sarcomas. However, it is unclear if mismatch repair defects have a role in sarcoma tumorigenesis. Microsatellite instability is a surrogate marker of mismatch repair defects.
OBJECTIVES: To determine whether sporadic dedifferentiated liposarcomas display microsatellite instability and, if so, to evaluate whether such instability differs between the lipogenic and nonlipogenic components of these tumors.
DESIGN: The diagnoses of conventional dedifferentiated liposarcoma were confirmed by a combination of morphologic, immunophenotypic, and molecular studies. Standard fluorescence-based polymerase chain reaction, including 5 mononucleotide microsatellite markers (BAT25, BAT26, NR21, NR24, and MONO27), as well as 2 pentanucleotide repeat markers (Penta C and Penta D), was used to test for instability and loss of heterozygosity.
RESULTS: We demonstrated only a single case (1 of 43) with microsatellite instability at one mononucleotide marker. No sarcomas showed high-level microsatellite instability. However, loss of heterozygosity at the pentanucleotide markers was observed in 8 of 43 cases. The presence of loss of heterozygosity was overrepresented in the nonlipogenic (dedifferentiated) components compared with the paired lipogenic (well differentiated) components.
CONCLUSIONS: Mismatch repair defects do not contribute to sporadic dedifferentiated liposarcoma tumorigenesis. Whether the observed loss of heterozygosity drives tumorigenesis in liposarcoma, for example by affecting tumor suppressor or cell cycle regulator genes, remains to be determined.

Biswas B, Rastogi S, Khan SA, et al.
Outcomes and prognostic factors for Ewing-family tumors of the extremities.
J Bone Joint Surg Am. 2014; 96(10):841-9 [PubMed] Related Publications
BACKGROUND: There are few published studies describing the clinical results of patients uniformly treated for a Ewing-family tumor of an extremity.
METHODS: We performed a review of patients who had received uniform treatment consisting of neoadjuvant chemotherapy, surgery and/or radiation therapy as local treatment, and then adjuvant chemotherapy from June 2003 to November 2011 at a single institution.
RESULTS: There were 158 patients included in the study. The median age was fifteen years. Sixty-nine (44%) of the patients had metastatic disease at presentation. Fifty-seven patients underwent surgery, and forty-one received radical radiation therapy following neoadjuvant chemotherapy. After a median of 24.3 months (range, 1.6 to ninety-seven months) of follow-up, the five-year event-free survival, overall survival, and local control rates (and standard error) were 24.1% ± 4.3%, 43.5% ± 6%, and 55% ± 6.8%, respectively, for the entire cohort and 36.4% ± 6.2%, 57.6% ± 7.4%, and 58.2% ± 7.9%, respectively, for patients without metastases. In the multivariate analysis, metastases predicted inferior event-free survival (p = 0.02) and overall survival (p = 0.03) rates in the entire cohort, whereas radical radiation therapy predicted an inferior local control rate in the entire cohort (p = 0.001) and in patients without metastases (p = 0.04). In the group with localized disease, there was no difference between the patients who received radical radiation therapy and those who underwent surgery with regard to tumor diameter (p = 0.8) or post-neoadjuvant chemotherapy response (p = 0.1). A white blood cell count (WBC) of >11 × 109/L predicted inferior event-free survival (p = 0.005) and local control (p = 0.02) rates for patients without metastases.
CONCLUSIONS: To our knowledge, this is the largest study on extremity Ewing-family tumors treated with uniform chemotherapy and either surgical resection or radical radiation therapy in Asia. All possible efforts should be made to resect a primary tumor after neoadjuvant chemotherapy, as radical radiation therapy alone results in a poor local control rate despite a good post-neoadjuvant chemotherapy response. Patients without metastases but with a high WBC had inferior event-free survival and local control rates and may require more aggressive therapy.
LEVEL OF EVIDENCE: Therapeutic Level IV. See Instructions for Authors for a complete description of levels of evidence.

Related: Bone Cancers Ewing's Sarcoma

Poos K, Smida J, Nathrath M, et al.
Structuring osteosarcoma knowledge: an osteosarcoma-gene association database based on literature mining and manual annotation.
Database (Oxford). 2014; 2014 [PubMed] Article available free on PMC after 24/12/2014 Related Publications
Osteosarcoma (OS) is the most common primary bone cancer exhibiting high genomic instability. This genomic instability affects multiple genes and microRNAs to a varying extent depending on patient and tumor subtype. Massive research is ongoing to identify genes including their gene products and microRNAs that correlate with disease progression and might be used as biomarkers for OS. However, the genomic complexity hampers the identification of reliable biomarkers. Up to now, clinico-pathological factors are the key determinants to guide prognosis and therapeutic treatments. Each day, new studies about OS are published and complicate the acquisition of information to support biomarker discovery and therapeutic improvements. Thus, it is necessary to provide a structured and annotated view on the current OS knowledge that is quick and easily accessible to researchers of the field. Therefore, we developed a publicly available database and Web interface that serves as resource for OS-associated genes and microRNAs. Genes and microRNAs were collected using an automated dictionary-based gene recognition procedure followed by manual review and annotation by experts of the field. In total, 911 genes and 81 microRNAs related to 1331 PubMed abstracts were collected (last update: 29 October 2013). Users can evaluate genes and microRNAs according to their potential prognostic and therapeutic impact, the experimental procedures, the sample types, the biological contexts and microRNA target gene interactions. Additionally, a pathway enrichment analysis of the collected genes highlights different aspects of OS progression. OS requires pathways commonly deregulated in cancer but also features OS-specific alterations like deregulated osteoclast differentiation. To our knowledge, this is the first effort of an OS database containing manual reviewed and annotated up-to-date OS knowledge. It might be a useful resource especially for the bone tumor research community, as specific information about genes or microRNAs is quick and easily accessible. Hence, this platform can support the ongoing OS research and biomarker discovery. Database URL:

Related: CDKN1A Osteosarcoma

Altinay S, Kusaslan R
Gastrointestinal autonomic nerve tumour of jejunum presenting as a perforated mass.
J Pak Med Assoc. 2014; 64(4):461-4 [PubMed] Related Publications
Gastrointestinal autonomic nerve tumour (GANT) is a rare mesenchymal neoplasm of the gastrointestinal tract arising from the neural plexus of the intestinal wall. Herein, we present a 70-year-old male patient presenting with a clinical picture of acute abdomen. Examination of the specimen obtained from the small bowel by means of complete resection revealed a relatively soft submucosal mass measuring 4.5 x 3 cm in size with spindle morphology and high mitotic activity (> 10 mitoses per 50 high-power fields). The tumour cells were strong positive for c-kit (CD117), S-100 protein and glial fibrillary acidic protein (GFAP), but did not harbour mutations in the c-kit and PDGFR genes. The diagnosis was based on light microscopy and immunohistochemical verification. We started tyrosine kinase inhibitor 400 mg/day. The patient is currently alive without metastasis at 28 months postoperatively. He is under close follow-up and survival data of the patient will be presented in the later studies.

Related: Gastrointestinal Stromal Tumors Imatinib (Glivec)

Stucky CC, Wasif N, Ashman JB, et al.
Excellent local control with preoperative radiation therapy, surgical resection, and intra-operative electron radiation therapy for retroperitoneal sarcoma.
J Surg Oncol. 2014; 109(8):798-803 [PubMed] Related Publications
PURPOSE: To examine the value of surgical resection combined with preoperative external beam radiation therapy and intraoperative radiation therapy (Surg-RT) for retroperitoneal sarcoma (RPS).
METHODS: Review of 63 consecutive patients with RPS from 1996 to 2011.
RESULTS: Thirty-seven patients (59%) underwent Surg-RT and 26 (41%) had surgery alone. 51% of tumors were high grade and 36% of patients had locally recurrent disease. Final margin status was: R0 73%, R1 16%, R2 6%, and unknown 5%. Of those with R0 resections, 67% received Surg-RT. Median follow-up was 45 months. The 5-year local control rate was 89% for Surg-RT patients and 46% for surgery alone patients (P = 0.03). On multivariate analysis, Surg-RT was the only variable associated with a lower risk of LR (HR 0.19; CI 0.05-0.69, P = 0.003). The actuarial 5-year OS was 60% for patients receiving either Surg-RT or surgery alone.
CONCLUSIONS: The combination of pre-operative radiation, surgical resection, and intraoperative radiation produces excellent local disease control for RPS. Combination therapy was associated with improved local control but not with overall survival.

Alam MM, Naser MF, Islam MF, Rahman MA
Primary adrenal leiomyosarcoma in an adult female.
Mymensingh Med J. 2014; 23(2):380-3 [PubMed] Related Publications
Primary leiomyosarcoma of the adrenal gland is a rare tumour and we describe such a case in a 35 year old female who had dull pain in the left flank for 6 months. There was no weight loss or fever only mild tenderness was in the left subcostal region. She was normotensive and there was no evidence of virilization. CT scan revealed left adrenal mass, after adrenalectomy it was 8.5×7.5×6.2cm³ in size. There was no evidence of local extension or metastasis. Histopathology confirmed adrenal leiomyosarcome.

Shirazian S, Agha-Hosseini F
Oral osteosarcoma: a case report and analysis of previously reported cases.
N Y State Dent J. 2014; 80(2):50-4 [PubMed] Related Publications
Osteosarcoma is the most common malignancy of mesenchymal cells after hematopoietic neoplasms. Most originate within bones, but the occurrence of this malignancy in the jaw bones is rare. There is controversy about the characteristics of this tumor in the literature. The aim of this paper was to collect the previous reported data and provide a statistical analysis of them. Additionally, we have reported a case of mandibular osteosarcoma.

Related: Osteosarcoma

Lantos JE, Hwang S, Panicek DM
Benign mural nodules within fluid collections at MRI after soft-tissue sarcoma resection.
AJR Am J Roentgenol. 2014; 202(6):1297-302 [PubMed] Related Publications
OBJECTIVE: The purpose of this study was to determine the prevalence and clinical significance of nodules within fluid collections on MRI after surgical resection of soft-tissue sarcoma.
MATERIALS AND METHODS: This retrospective study included 175 patients who underwent resection of primary soft-tissue sarcoma and whose postoperative MRI reports mentioned fluid. Images were reviewed to determine the presence of fluid collections of 1 cm or greater in diameter in the surgical bed and any nodule (measuring ≥ 0.7 cm) within the collection. Signal intensity and characteristics of each collection and rim and presence of septa or blood products were recorded. Size, signal intensity, and contrast enhancement of nodules were reviewed. Nodules were classified as benign or malignant on the basis of histologic results or clinical or MRI follow-up.
RESULTS: Fluid collections were present in 75 patients. Of those, 45 collections (60%) showed homogeneous fluid signal intensity and 30 (40%) were heterogeneous; septa were present in 45 (60%) and blood products in 12 (16%). Most collections showed a thin rim (59%) and rim enhancement (88%). Nodules were present along the inner wall of six (8%) collections. Four (66%) nodules enhanced and two (33%) were T1 hyperintense. At follow-up MRI, two nodules were stable in size, one decreased, and three resolved. Nodules in three patients were biopsied; all were benign. Two other patients had no recurrence at follow-up, and another died at 3 months.
CONCLUSION: A nodule within a postoperative fluid collection at MRI after soft-tissue sarcoma resection generally does not represent tumor recurrence; short-interval follow-up MRI is recommended rather than immediate biopsy.

Kim JM, Im SA, Oh SN, Chung NG
Alveolar soft part sarcoma arising from the kidney: imaging and clinical features.
Korean J Radiol. 2014; 15(3):381-5 [PubMed] Article available free on PMC after 24/12/2014 Related Publications
Alveolar soft part sarcoma (ASPS) is an extremely rare malignant soft tissue sarcoma primarily affecting young patients. It usually occurs in the lower extremities, although it can occur in soft tissue anywhere in the body. However, to our knowledge, there has been no case of primary ASPS originating from the kidney in the literature. We herein present the imaging and clinical features of an ASPS which occurred in a 16-year-old male presented as a palpable mass in the left side of the abdomen.

Related: Kidney Cancer

Hong JB, Cho KH, Choi JH
Periosteal osteosarcoma arising from the rib and scapula: imaging features in two cases.
Korean J Radiol. 2014; 15(3):370-5 [PubMed] Article available free on PMC after 24/12/2014 Related Publications
Periosteal osteosarcoma is an extremely rare chondroblastic osteosarcoma in the flat bone. There were authors reporting of two cases of periosteal osteosarcoma in the highly unusual sites. One of them arose from the rib, in a 17-year-old male, which appeared as a hypodense juxtacortical mass with periosteal reaction on CT. The other one arose from the scapula, in a 17-year-old female, which showed the intermediate signal intensity (SI) on T1-weighted image (WI), heterogeneous high SI on T2WI, and rim-enhancement on contrast-enhanced T1WI with cortical destruction on MRI.

Related: Bone Cancers Osteosarcoma

Brasme JF, Chalumeau M, Oberlin O, et al.
Time to diagnosis of Ewing tumors in children and adolescents is not associated with metastasis or survival: a prospective multicenter study of 436 patients.
J Clin Oncol. 2014; 32(18):1935-40 [PubMed] Related Publications
PURPOSE: The time to diagnosis (TtD) of Ewing tumors is one of the longest among pediatric tumors. Its precise consequences, however, have not been studied well. We analyzed the distribution of TtD for Ewing tumors in children and adolescents and its association with clinical features, tumor characteristics, surgical outcome, and long-term survival.
PATIENTS AND METHODS: We analyzed prospectively collected data from two multicenter clinical trials of patients younger than 21 years old who had Ewing bone tumors treated in France between 1988 and 2000. Clinical and tumoral features, TtD, and outcome associations were studied by univariable and multivariable analyses.
RESULTS: The median TtD for the 436 patients was 70 days (interquartile range, 27 to 146 days), with no significant decrease during the study period (P > .2). The factors associated with long TtD were older age and some tumor sites (pelvis, extremities of limbs). Increased tumor volume and decreased histologic response to chemotherapy were associated with long TtD on univariable analysis (P < .05) but not after adjustment. Presence of a nerve or spinal-cord compression at diagnosis, presence or site of metastasis, surgical treatment, mutilating surgery, complete resection, or survival were not associated with TtD.
CONCLUSION: TtD of Ewing tumors was long, especially for adolescents and for certain tumor sites, and did not improve over time. But TtD was not associated with metastasis, surgical outcome, or survival. These findings could be used to comfort parents at diagnosis and in expert testimony produced for malpractice claims.

Related: Bone Cancers France

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