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).

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.

Mazzucchelli R, Galosi AB, Scarpelli M, et al.
Contemporary update on pathology-related issues of adult renal neoplasms.
Anal Quant Cytol Histol. 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 Cytol Histol. 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

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.

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.

Xie L, X D T, Yang RL, Guo W
Interscapulothoracic resection of tumours of shoulder with a note on reconstruction.
Bone Joint J. 2014; 96-B(5):684-90 [PubMed] Related Publications
We retrospectively reviewed the outcomes of 33 consecutive patients who had undergone an extra-articular, total or partial scapulectomy for a malignant tumour of the shoulder girdle between 1 July 2001 and 30 September 2013. Of these, 26 had tumours which originated in the scapula or the adjacent soft tissue and underwent a classic Tikhoff-Linberg procedure, while seven with tumours arising from the proximal humerus were treated with a modified Tikhoff-Linberg operation. We used a Ligament Advanced Reinforcement System for soft-tissue reconstruction in nine patients, but not in the other 24. The mean Musculoskeletal Tumor Society score (MSTS) was 17.6 (95% confidence interval (CI) 15.9 to 19.4); 17.6 (95% CI 15.5 to 19.6) after the classic Tikhoff-Linberg procedure and 18.1 (95% CI 13.8 to 22.3) after the modified Tikhoff-Linberg procedure. Patients who had undergone a LARS soft-tissue reconstruction had a mean score of 18.6 (95% (CI) 13.9 to 22.4) compared with 17.2 (95% CI 15.5 to 19.0) for those who did not. The Tikhoff-Linberg procedure is a useful method for wide resection of a malignant tumour of the shoulder girdle which helps to preserve hand and elbow function. The method of soft-tissue reconstruction has no effect on functional outcome.

Related: Bone Cancers Chondrosarcoma

Gaston CL, Nakamura T, Reddy K, et al.
Is limb salvage surgery safe for bone sarcomas identified after a previous surgical procedure?
Bone Joint J. 2014; 96-B(5):665-72 [PubMed] Related Publications
Bone sarcomas are rare cancers and orthopaedic surgeons come across them infrequently, sometimes unexpectedly during surgical procedures. We investigated the outcomes of patients who underwent a surgical procedure where sarcomas were found unexpectedly and were subsequently referred to our unit for treatment. We identified 95 patients (44 intra-lesional excisions, 35 fracture fixations, 16 joint replacements) with mean age of 48 years (11 to 83); 60% were males (n = 57). Local recurrence arose in 40% who underwent limb salvage surgery versus 12% who had an amputation. Despite achieving local control, overall survival was worse for patients treated with amputation rather than limb salvage (54% vs 75% five-year survival). Factors that negatively influenced survival were invasive primary surgery (fracture fixation, joint replacement), a delay of greater than two months until referral to our oncology service, and high-grade tumours. Survival in these circumstances depends mostly on factors that are determined prior to definitive treatment by a tertiary orthopaedic oncology unit. Limb salvage in this group of patients is associated with a higher rate of inadequate marginal surgery and, consequently, higher local recurrence rates than amputation, but should still be attempted whenever possible, as local control is not the primary determinant of survival.

Related: Bone Cancers Osteosarcoma

Gogia A, Sharma A, Chopra A, Kumar R
Mediastinal granulocytic sarcoma: poor risk AML?
J Indian Med Assoc. 2013; 111(8):562 [PubMed] Related Publications
Granulocytic sarcoma (GS), a rare extramedullary manifestation of acute myeloid leukaemia (AML) has been frequently reported in the skin, orbits, gingiva, maxilla and lymph nodes. GS in the mediastinum is often mistaken as lymphoblastic lymphoma or mediastinal germ cell tumour. We presented here three cases of AML with normal cytogenetics who presented with mediastinal masses with superior vena caval (SVC) syndrome. All the three cases summarised in this write-up, received standard AML therapy but had, poor outcome.

Related: Acute Myeloid Leukemia (AML)

Ball SL, Kwong FN, Young F, Robson AK
Pharyngeal angiosarcoma following multimodal treatment for oropharyngeal squamous cell carcinoma.
Ann R Coll Surg Engl. 2014; 96(2):e5-6 [PubMed] Related Publications
It is well established that angiosarcoma can develop following radiotherapy. We present an unusual case of angiosarcoma of the pharynx that developed three years after treatment with surgery and adjuvant chemoradiotherapy for a T2N2bM0 squamous cell carcinoma of the oropharynx. The patient was tumour free until developing dysphagia, which was found to be caused by an angiosarcoma. The patient underwent surgery of the pharyngeal angiosarcoma by laryngopharyngectomy, tongue base resection, selective neck dissection and radial forearm microvascular free flap reconstruction. Angiosarcoma following head and neck malignancy is rare but must be considered as part of the differential diagnosis in patients with new symptoms after radiotherapy.

Related: Oropharyngeal Cancer

Aoki M, Nishio J, Iwasaki H, et al.
Osteosarcoma of the patella mimicking giant cell tumor: imaging features with histopathological correlation.
Anticancer Res. 2014; 34(5):2541-5 [PubMed] Related Publications
Patellar tumors represent an uncommon etiology of anterior knee pain and their diagnosis is often delayed. We present an unusual case of conventional osteosarcoma arising in the patella of a 47-year-old man. The patient presented with a 1-year history of increasing anterior knee pain and swelling. Plain radiographs revealed a multi-locular lytic lesion in the inferolateral side of the patella. Computed tomography scans demonstrated an intraosseous lytic lesion with cortical thinning/breakthrough anteriorly. On magnetic resonance imaging, the lesion exhibited low signal intensity on T1-weighted images and heterogeneous high signal intensity on T2-weighted images. Fluid-fluid levels were also observed on T2-weighted images. Contrast-enhanced fat-suppressed T1-weighted images demonstrated strong enhancement of the lesion. These imaging features were suggestive of a benign condition; however, the diagnosis of osteosarcoma was confirmed by histopathology. After neoadjuvant chemotherapy, a wide resection with a free anterolateral thigh flap was performed. The patient subsequently underwent adjuvant chemotherapy and had no evidence of local recurrence or distant metastasis six months after surgery. Our case highlights the difficulty in the diagnosis of patellar osteosarcoma and the importance of performing a biopsy before definitive treatment.

Related: Bone Cancers Osteosarcoma

Kwon HY, Kim KS, An HK, et al.
Triptolide induces apoptosis through extrinsic and intrinsic pathways in human osteosarcoma U2OS cells.
Indian J Biochem Biophys. 2013; 50(6):485-91 [PubMed] Related Publications
Triptolide, a diterpene derived from Tripterygium wilfordii Hook f., a Chinese medicinal herb, has been reported to inhibit cell proliferation and induce apoptosis in various human cancer cells, but its anticancer effects on human osteosarcoma cells have not yet been elucidated. In this study, we investigated whether triptolide induces apoptosis in human osteosarcoma cells and the underlying molecular mechanisms. We firstly demonstrated that triptolide inhibited cell growth and induced apoptosis in U2OS cells. Western blot analysis showed that the levels of procaspase-8, -9, Bcl-2, Bid and mitochondrial cytochrome c were downregulated in triptolide-treated U2OS cells, whereas the levels of Fas, FasL, Bax, cytosolic cytochrome c, cleaved caspase-3 and cleaved PARP were upregulated. These results suggest that triptolide induces apoptosis in U2OS cells by activating both death receptor and mitochondrial apoptotic pathways.

Related: Apoptosis Mitochondrial Mutations in Cancer Osteosarcoma

Makihara N, Maeda T, Ebina Y, et al.
Leiomyosarcoma of the broad ligament: a case report with CT and MRI images.
Eur J Gynaecol Oncol. 2014; 35(2):174-7 [PubMed] Related Publications
Primary leiomyosarcoma of the broad ligament is a very rare and highly malignant gynecological tumor. The authors report a 61-year-old postmenopausal woman with signs and symptoms of malignant ovarian tumor. Preoperative magnetic resonance imaging (MRI) was interpreted as being suspicious for malignant tumors, such as an ovarian cancer or a leiomyosarcoma of the broad ligament, so laparotomy was performed. Macroscopically, the tumor was revealed with a 18 x 13.7 x 9.5 cm degenerated, multiple cystic part and solid whitish part arising from broad ligament which on histopathology proved to be leiomyosarcoma. To the best of the authors' knowledge, primary leiomyosarcoma of the broad ligament has been documented in 21 reports or so, and no imaging findings are available. Here the authors present the MRI findings of primary leiomyosarcoma of the broad ligament.

Related: Gynacological Cancers

Ruivo C, Hopper MA
Spinal chondrosarcoma arising from a solitary lumbar osteochondroma.
JBR-BTR. 2014 Jan-Feb; 97(1):21-4 [PubMed] Related Publications
Chondrosarcoma is a primary malignant neoplasm of cartilage-forming cells that rarely involves the axial skeleton, typically affecting skeletally mature patients. It may arise as a primary bone tumour or as a secondary lesion from a pre-existing benign cartilaginous neoplasm such as an osteochondroma or enchondroma. We report the case of a 68-year-old female who presented with a mildly painful paraspinal mass lesion as a result of malignant degeneration of a previously unknown solitary lumbar osteochondroma into a large chondrosarcoma. The characteristic imaging findings on cross-sectional imaging techniques are reviewed and illustrated, along with an outline of relevant clinical and therapeutic aspects.

Related: Bone Cancers

Agaram NP, Zhang L, Sung YS, et al.
Extraskeletal myxoid chondrosarcoma with non-EWSR1-NR4A3 variant fusions correlate with rhabdoid phenotype and high-grade morphology.
Hum Pathol. 2014; 45(5):1084-91 [PubMed] Article available free on PMC after 01/05/2015 Related Publications
Extraskeletal myxoid chondrosarcomas (EMC) are rare soft tissue sarcomas with distinctive histology and uncertain histogenesis, characterized by Ewing sarcoma breakpoint region 1-nuclear receptor subfamily 4, group A, member 3 (EWSR1-NR4A3) fusion in 75% of the cases. A smaller proportion of cases show NR4A3 fused to other gene partners including TATA binding protein-associated factor 15 (TAF15), transcription factor 12 (TCF12), and TRK-fused gene (TFG). The impact of various gene fusions on morphology and outcome has not been previously evaluated. We investigated 26 consecutive EMCs and correlated the genetic findings with morphology and clinical outcome. There were 5 females and 21 males (median age, 49.5 years). Mean size of the tumors was 11 cm. Fluorescence in situ hybridization analysis showed EWSR1-NR4A3 gene fusion in 16 cases (62%), TAF15-NR4A3 gene fusion in 7 cases (27%), and TCF12-NR4A3 gene fusion in 1 case (4%). Two cases showed only NR4A3 gene rearrangements. Morphologically, most EWSR1-rearranged tumors (10/16) showed low cellularity, minimal cytologic atypia, and low mitotic counts. In contrast, 80% of EMCs with variant (non-EWSR1) NR4A3 gene fusions (TAF15, TCF12) had high-grade morphology with increased cellularity, proliferation, and cytologic atypia, showing a plasmacytoid/rhabdoid morphology in half the cases. Follow-up showed that only 1 of 16 patients with EWSR1-rearranged tumors died of disease, in contrast to 3 (43%) of 7 TAF15-rearranged tumors. In conclusion, EMCs with variant NR4A3 gene fusions show a higher incidence of rhabdoid phenotype, high-grade morphology, and a more aggressive outcome compared with the EWSR1-NR4A3 positive tumors. Furthermore, fluorescence in situ hybridization assay for NR4A3, along with EWSR1, may be an additional ancillary test to confirm diagnosis of EMCs.

Related: Chondrosarcoma EWSR1 gene

Chamberlain BK, McClain CM, Gonzalez RS, et al.
Alveolar soft part sarcoma and granular cell tumor: an immunohistochemical comparison study.
Hum Pathol. 2014; 45(5):1039-44 [PubMed] Related Publications
Although the histologic features of alveolar soft part sarcoma and granular cell tumor are typically distinctive, occasional cases show a significant morphologic overlap. Differentiating these entities is crucial because granular cell tumor is almost always benign and alveolar soft part sarcoma is invariably malignant. We evaluated a panel of immunohistochemical stains (S-100 protein, inhibin, SOX10, nestin, calretinin, and TFE3) in 13 alveolar soft part sarcomas and 11 granular cell tumors. Tissue sections were also stained by the periodic acid-Schiff method after diastase digestion (PAS-D) and evaluated for coarse cytoplasmic granularity or crystalline cytoplasmic inclusions. S-100 protein, inhibin, SOX10, and nestin each distinguished granular cell tumor and alveolar soft part sarcoma with 100% sensitivity and specificity. PAS-D staining also distinguished cases with 100% accuracy, as granular cell tumor consistently demonstrated coarsely granular, PAS-D-positive cytoplasm and alveolar soft part sarcoma showed only focal intracytoplasmic crystalline inclusions. Although all granular cell tumors were calretinin positive, so were 46% of alveolar soft part sarcomas. TFE3 was positive in 91% of granular cell tumors and all alveolar soft part sarcomas. Together with PAS-D, immunohistochemical stains for S-100 protein, inhibin, SOX10, and nestin accurately identify alveolar soft part sarcoma and granular cell tumor. Although TFE3 has been reported as a relatively specific marker for alveolar soft part sarcoma, it should be recalled that it is also expressed in most granular cell tumors.

Takahashi Y, Kohashi K, Yamada Y, et al.
Activation of the Akt/mammalian target of rapamycin pathway in myxofibrosarcomas.
Hum Pathol. 2014; 45(5):984-93 [PubMed] Related Publications
The Akt/mammalian target of rapamycin (mTOR) pathway plays important roles in modulating cellular function in response to extracellular signals such as growth factors and cytokines. The Akt/mTOR signaling pathway is activated in certain kinds of sarcomas. Myxofibrosarcoma is a soft tissue sarcoma, characterized by abundant myxoid stroma and frequent local recurrence. Here, we conducted a large-scale examination of the clinicopathological and activation statuses of the Akt/mTOR pathways in myxofibrosarcoma. The phosphorylation status of Akt, mTOR, S6 ribosomal protein, and the eukaryotic translation initiation factor 4E-binding protein, and mitogen-activated protein kinase were assessed by immunohistochemistry in 101 formalin-fixed, paraffin-embedded samples, including 68 primary tumors in myxofibrosarcoma. Immunohistochemical expressions were confirmed by Western blotting with 20 frozen samples, which were paired with normal tissue samples. PIK3CA and AKT1 gene mutations were also analyzed using 12 primary tumor frozen samples. Immunohistochemically, phosphorylations of Akt, mTOR, S6 ribosomal protein, 4E-binding protein, and mitogen-activated protein kinase 1/2 were observed in 64.7%, 45.6%, 42.6%, 63.2%, and 64.7% of samples. Phosphorylated Akt/mTOR pathway proteins were correlated with one another and were also correlated with the phosphorylation of these proteins in the concordant recurrent tumors. Immunoblotting showed a high degree of phosphorylation in tumor samples, compared with that in normal tissue samples. Activation of the Akt/mTOR pathway was correlated with histologic grade and tumor progression. Mutational analysis failed to reveal any PIK3CA or AKT1 mutations around the hot spots. Activation of the Akt/mTOR pathway was associated with histologic malignancy and tumor progression in primary and recurrent myxofibrosarcoma.

Related: Dermatofibrosarcoma Protuberans AKT1 Signal Transduction

Chung SW, Han I, Oh JH, et al.
Prognostic effect of erroneous surgical procedures in patients with osteosarcoma: evaluation using propensity score matching.
J Bone Joint Surg Am. 2014; 96(8):e60 [PubMed] Related Publications
BACKGROUND: Little is known concerning erroneous surgical procedures of malignant bone tumors, and the prognostic effect of erroneous surgical procedures in osteosarcoma has not been determined.
METHODS: We retrospectively reviewed 240 patients with initially non-metastatic high-grade osteosarcoma of the pelvis and extremities and, of these, identified twenty-six who had undergone previous less appropriate surgical procedures due to misdiagnosis followed by adequate treatment at our institution. We evaluated the clinicopathologic characteristics of these twenty-six patients compared with the remaining 214 patients treated with regular protocol. Subsequently, thirty-eight patients (nineteen in the matched case group and nineteen in the matched control group) were matched for multiple different variables using propensity score matching, and the oncologic results in terms of event-free survival and overall survival were analyzed.
RESULTS: The patients undergoing erroneous surgical procedures were typically older, with small, non-osteoblastic-type tumors that were in an unusual location, showed an osteolytic pattern on radiographs, had a tendency toward marginal or intralesional excision with positive histologic margin, and had not been treated with neoadjuvant chemotherapy (all p < 0.05). After adjustment of confounding variables by propensity score matching, there was no significant difference between matched groups with regard to event-free survival (p = 0.46) and overall survival (p = 0.99).
CONCLUSIONS: Distinct differences existed in the clinicopathologic characteristics of the patients who underwent erroneous surgical procedures due to misdiagnosis. We failed to detect a prognostic relevance of the presence of previous erroneous procedures followed by adequate treatment.

Related: Bone Cancers Osteosarcoma

Mazeron R, Oberlin O, Dumas I, et al.
Brachytherapy in children with rhabdomyosarcomas of the nasolabial fold.
Pediatr Blood Cancer. 2014; 61(7):1162-7 [PubMed] Related Publications
BACKGROUND: Rhabdomyosarcomas (RMS) of the nasolabial fold can be difficult to manage surgically due to functional and cosmetic limitations. Therefore, brachytherapy (BT) has been proposed to improve local control while limiting the volume of irradiation as well as the extent of the surgical excision.
MATERIALS AND METHODS: Sixteen pediatric cases with RMS of the nasolabial fold treated from 1971 to 2005 were retrospectively reviewed.
RESULTS: Median follow-up was 4.4 years (1.7-33). Half of the patients were male and their age at diagnosis ranged from 4 months to 13.5 years. Histological subtypes included 10 embryonal and 6 alveolar RMS. Initial treatment consisted of induction multi-agent chemotherapy in all cases. In 12 patients, BT was combined with local excision (4 complete resections, 1 with macroscopic residual disease, and 7 with microscopic disease). Low dose-rate brachytherapy was performed in all cases according to the Paris system, using plastic catheters implanted per-operatively. The doses delivered ranged from 50 to 70 Gy, depending on chemotherapy response, and surgical margin status. 10 patients relapsed: 4 local, 6 regional, and 2 metastatic failures were reported. The median time to relapse was 6.5 months. At the time of analysis eight patients were alive and four had died. Four cases, under palliative care at last check-up, were lost to follow-up.
CONCLUSION: BT provided an acceptable local control rate, but the poor regional control of these cases may suggest a need for more aggressive management of cervical regional lymph node regions in RMS of the nasolabial fold.

Related: Brachytherapy Rhabdomyosarcoma Skin Cancer

Aslam MI, Abraham J, Mansoor A, et al.
PDGFRβ reverses EphB4 signaling in alveolar rhabdomyosarcoma.
Proc Natl Acad Sci U S A. 2014; 111(17):6383-8 [PubMed] Article available free on PMC after 01/05/2015 Related Publications
Alveolar rhabdomyosarcoma (aRMS) is an aggressive myogenic childhood malignancy, not infrequently presenting as incurable metastatic disease. To identify therapeutic targets, we performed an unbiased tyrosine kinome RNA interference screen in primary cell cultures from a genetically engineered, conditional mouse model of aRMS. We identified ephrin receptor B4 (EphB4) as a target that is widely expressed in human aRMS and that portends a poor clinical outcome in an expression level-dependent manner. We also uncovered cross-talk of this ephrin receptor with another receptor tyrosine kinase, PDGFRβ, which facilitates PDGF ligand-dependent, ephrin ligand-independent activation of EphB4 converging on the Akt and Erk1/2 pathways. Conversely, EphB4 activation by its cognate ligand, EphrinB2, did not stimulate PDGFRβ; instead, apoptosis was paradoxically induced. Finally, we showed that small-molecule inhibition of both PDGFRβ and EphB4 by dasatinib resulted in a significant decrease in tumor cell viability in vitro, as well as decreased tumor growth rate and significantly prolonged survival in vivo. To our knowledge, these results are the first to identify EphB4 and its cross-talk with PDGFRβ as unexpected vital determinants of tumor cell survival in aRMS, with EphB4 at the crux of a bivalent signaling node that is either mitogenic or proapoptotic.

Related: Apoptosis PDGFB gene Signal Transduction Imatinib (Glivec) Dasatinib (Sprycel)

Casey DL, Wexler LH, LaQuaglia MP, et al.
Patterns of failure for rhabdomyosarcoma of the perineal and perianal region.
Int J Radiat Oncol Biol Phys. 2014; 89(1):82-7 [PubMed] Related Publications
PURPOSE: To analyze prognostic factors and patterns of failure for rhabdomyosarcoma of the perineal and perianal region (PRMS), with an emphasis on radiation therapy for locoregional control.
METHODS AND MATERIALS: Detailed records of all 14 patients treated for PRMS at Memorial Sloan-Kettering Cancer Center between 1998 and 2012 were reviewed. The Kaplan-Meier method was used to assess the event-free survival (EFS) and overall survival (OS), and a competing-risks analysis was used to assess the cumulative incidence of local, regional, and distant failures.
RESULTS: Median age was 15.8 years (range, 1.1-31.9 years). High-risk features were identified: 9 of 14 patients (64%) had group 3 disease and 3 of 14 (21%) had group 4; 11 of 14 tumors (78%) were alveolar; 12 of 14 tumors (86%) were ≥5 cm; and 9 of 14 patients (64%) had involved lymph nodes (N1). Of those aged ≥10 years at diagnosis, 9 of 10 (90%) had alveolar histology, all had tumors ≥5 cm, and 8 of 10 (80%) presented with N1 disease. The rates of local, regional, and distant failure at 5 years were 17%, 31%, and 52%, respectively. Although 3 of the 4 patients with regional failure received nodal irradiation, only one of the nodal failures occurred in the radiation therapy field. The 5-year EFS was 33%, and OS was 39%. Age ≥10 years was associated with poor outcomes: EFS was 13% in patients aged ≥10 years, compared with 75% in those aged <10 years (P=.04); the OS was 13% in patients aged ≥10 years, compared with 100% in those aged <10 years (P=.04).
CONCLUSIONS: Patients with PRMS, especially those aged ≥10 years, present with poor prognostic features and continue to have poor outcomes. Given the high incidence of regional node recurrence, we recommend prophylactic ilioinguinal lymph node irradiation for all patients aged ≥10 years. For children aged <10 years, nodal evaluation is essential to determine the role for lymph node irradiation.

Demey K, Reyns LM, Schepers S
Angiosarcoma arising in an arteriovenous fistula in a patient without kidney transplant.
Acta Chir Belg. 2014 Jan-Feb; 114(1):75-8 [PubMed] Related Publications
Angiosarcomas are relatively rare and account for only 1% of all sarcomas. They arise from endothelial cells of blood or lymph vessels. They are usually highly aggressive and long term outcome is poor with an overall 5-year survival rate of 10-20%. We report the case of a 80-year old man with an angiosarcoma arising in a non-functioning arteriovenous fistula. Angiosarcomas arising in an arteriovenous fistula are very rare and only eleven cases were found in the literature. In nine cases (82%) chronic immunosuppression, taken for renal transplant, was one of the causing factors. Our patient however did not receive a kidney transplant and was not on immunosuppressive therapy. Clinicians should be aware that an angiosarcoma can arise in an arteriovenous fistula even without chronic immunosuppression.

Lopez-Beltran A, Montironi R, Carazo JL, et al.
Primary renal osteosarcoma.
Am J Clin Pathol. 2014; 141(5):747-52 [PubMed] Related Publications
OBJECTIVES: To investigate primary osteosarcoma (osteogenic sarcoma) of the kidney, a rare and aggressive neoplasm.
METHODS: We present clinical and pathologic features of three female patients, aged 50, 66, and 78 years, affected by primary osteosarcoma of the kidney. The diagnosis was made by H&E-stained samples from totally (cases 1 and 2) or partially (case 3) embedded tumors.
RESULTS: Reported cases showed histologic features of low-grade (n = 1), chondroblastic (n = 1), and osteoblastic (n = 1) osteosarcoma. Tumor size ranged from 3 to 7 cm, and pT category was pT1a (n = 1), pT1b (n = 1), and pT3a (n = 1). Immunohistochemistry gave focal positive results with PAX2 and CD10 in case 1 and S100 in case 2. On follow-up, two patients were disease free at 25 and 68 months and one died of metastases.
CONCLUSIONS: Surgically treated primary renal osteosarcoma might not be as aggressive as previously thought if diagnosed early with low pT status.

Related: Kidney Cancer Osteosarcoma

Chen EY, DeRan MT, Ignatius MS, et al.
Glycogen synthase kinase 3 inhibitors induce the canonical WNT/β-catenin pathway to suppress growth and self-renewal in embryonal rhabdomyosarcoma.
Proc Natl Acad Sci U S A. 2014; 111(14):5349-54 [PubMed] Article available free on PMC after 08/10/2014 Related Publications
Embryonal rhabdomyosarcoma (ERMS) is a common pediatric malignancy of muscle, with relapse being the major clinical challenge. Self-renewing tumor-propagating cells (TPCs) drive cancer relapse and are confined to a molecularly definable subset of ERMS cells. To identify drugs that suppress ERMS self-renewal and induce differentiation of TPCs, a large-scale chemical screen was completed. Glycogen synthase kinase 3 (GSK3) inhibitors were identified as potent suppressors of ERMS growth through inhibiting proliferation and inducing terminal differentiation of TPCs into myosin-expressing cells. In support of GSK3 inhibitors functioning through activation of the canonical WNT/β-catenin pathway, recombinant WNT3A and stabilized β-catenin also enhanced terminal differentiation of human ERMS cells. Treatment of ERMS-bearing zebrafish with GSK3 inhibitors activated the WNT/β-catenin pathway, resulting in suppressed ERMS growth, depleted TPCs, and diminished self-renewal capacity in vivo. Activation of the canonical WNT/β-catenin pathway also significantly reduced self-renewal of human ERMS, indicating a conserved function for this pathway in modulating ERMS self-renewal. In total, we have identified an unconventional tumor suppressive role for the canonical WNT/β-catenin pathway in regulating self-renewal of ERMS and revealed therapeutic strategies to target differentiation of TPCs in ERMS.

Kang S, Kim HS, Kim S, et al.
Post-metastasis survival in extremity soft tissue sarcoma: a recursive partitioning analysis of prognostic factors.
Eur J Cancer. 2014; 50(9):1649-56 [PubMed] Related Publications
BACKGROUND: Recursive partitioning analysis (RPA) enables grouping of patients into homogeneous prognostic groups in a visually intuitive form and has the capacity to account for complex interactions among prognostic variables. In this study, we employed RPA to generate a prognostic model for extremity soft tissue sarcoma (STS) patients with metastatic disease.
METHODS: A retrospective review was conducted on 135 patients with metastatic STS who had undergone surgical removal of their primary tumours. Patient and tumour variables along with the performance of metastasectomy were analysed for possible prognostic effect on post-metastatic survival. Significant prognostic factors on multivariate analysis were incorporated into RPA to build regression trees for the prediction of post-metastatic survival.
RESULTS: RPA identified six terminal nodes based on histological grade, performance of metastasectomy and disease-free interval (DFI). Based on the median survival time of the terminal nodes, four prognostic groups with significantly different post-metastatic survival were generated: (1) group A: low grade/metastasectomy; (2) group B: low grade/no metastasectomy/DFI ⩾ 12 months or high grade/metastasectomy; (3) group C: low grade/no metastasectomy/DFI < 12 months or high grade/no metastasectomy/DFI ⩾ 12 months; and (4) group D: high grade/no metastasectomy/DFI < 12 months. The 3-year survival rates for each group were: group A, 76.1 ± 9.6%; group B, 42.3 ± 10.3%; group C, 18.8 ± 8.0%; and group D, 0.0 ± 0.0%.
CONCLUSION: Our prognostic model using RPA successfully divides STS patients with metastasis into groups that can be easily implemented using standard clinical parameters.

Stacchiotti S, Pantaleo MA, Astolfi A, et al.
Activity of sunitinib in extraskeletal myxoid chondrosarcoma.
Eur J Cancer. 2014; 50(9):1657-64 [PubMed] Related Publications
BACKGROUND: Extraskeletal myxoid chondrosarcoma (EMC) is a rare soft tissue sarcoma, marked by NR4A3 rearrangement. Herein we report on the activity of sunitinib in a series of 10 patients, strengthening what initially observed in two cases.
PATIENTS AND METHODS: From July 2011, 10 patients with progressive metastatic translocated EMC have been consecutively treated with sunitinib 37.5mg/day, on a named-use basis. In an attempt to interpret the activity of sunitinib in EMC, genotype/phenotype correlations were carried out by fluorescence in situ hybridization (FISH) analyses. Moreover, transcriptome, immunohistochemical and biochemical analyses of a limited set of samples were performed focusing on some putative targets of sunitinib.
RESULTS: Eight of 10 patients are still on therapy. Six patients had a Response Evaluation Criteria in Solid Tumours (RECIST) partial response (PR), two were stable, two progressed. Positron emission tomography (PET) was consistent in 6/6 evaluable cases. One patient underwent surgery after sunitinib, with evidence of a pathologic response. At a median follow-up of 8.5 months (range 2-28), no secondary resistance was detected. Median progression free survival (PFS) has not been reached. Interestingly, all responsive cases turned out to express the typical EWSR1-NR4A3 fusion, while refractory cases carried the alternative TAF15-NR4A3 fusion. Among putative sunitinib targets, only RET was expressed and activated in analysed samples.
CONCLUSIONS: This report confirms the therapeutic activity of sunitinib in EMC. Genotype/phenotype analyses support a correlation between response and EWSR1-NR4A3 fusion. Involvement of RET deserves further investigation.

Related: Angiogenesis Inhibitors Bone Cancers Secondary Bone Cancer (bone metastasis) EWSR1 gene TAF15 gene Sunitinib (Sutent) RET

Valenti MT, Zanatta M, Donatelli L, et al.
Ascorbic acid induces either differentiation or apoptosis in MG-63 osteosarcoma lineage.
Anticancer Res. 2014; 34(4):1617-27 [PubMed] Related Publications
BACKGROUND/AIM: Osteosarcoma originates from mesenchymal stem cells with impaired bone differentiation. In the present study we investigated the effect of ascorbic acid (AsA) on osteogenic differentiation and apoptosis of the MG-63 osteosarcoma cell line.
MATERIALS AND METHODS: We evaluated the expression of runt-related transcription factor-2 (RUNX2) and secreted phosphoprotein 1 (SPP1) genes by real-time Polymerase Chain Reaction (PCR) and of endogenous bone morphogenetic protein-2 (BMP2) and osteocalcin proteins by immunohistochemistry. We analyzed osteoblast maturation by phosphatase alkaline synthesis and calcium deposition, and apoptosis by (TUNEL) test and Annexin staining.
RESULTS: Our results showed that RUNX2 and SPP1 gene expression was increased in cells treated with low concentrations of AsA with respect to untreated cells. At higher concentrations, AsA induced apoptosis of osteosarcoma cells, possibly with the involvement of p21.
CONCLUSION: Our findings support the ability of AsA to induce both differentiation, by affecting the target involved in early and late phases of osteogenic maturation, and apoptosis in poorly-differentiated osteosarcoma cells.

Related: Apoptosis Bone Cancers Osteosarcoma SPP1

Igarashi K, Yamamoto N, Shirai T, et al.
The long-term outcome following the use of frozen autograft treated with liquid nitrogen in the management of bone and soft-tissue sarcomas.
Bone Joint J. 2014; 96-B(4):555-61 [PubMed] Related Publications
In 1999, we developed a technique for biological reconstruction after excision of a bone tumour, which involved using autografts of the bone containing the tumour treated with liquid nitrogen. We have previously reported the use of this technique in 28 patients at a mean follow up of 27 months (10 to 54). In this study, we included 72 patients who underwent reconstruction using this technique. A total of 33 patients died and three were lost to follow-up, at a mean of 23 months (2 to 56) post-operatively, leaving 36 patients available for a assessment at a mean of 101 months 16 to 163) post-operatively. The methods of reconstruction included an osteo-articular graft in 16, an intercalary in 13 and, a composite graft with prosthesis in seven. Post-operative function was excellent in 26 patients (72.2%), good in seven (19.4%), and fair in three (8.3%) according to the functional evaluation system of Enneking. No recurrent tumour occurred within the grafts. The autografts survived in 29 patients (80.6%), and the rates of survival at five and ten years were 86.1% and 80.6 %, respectively. Seven of 16 osteo-articular grafts (44%) failed because of fracture or infection, but all the composite and intercalary grafts survived. The long-term outcomes of frozen autografting, particularly using composite and intercalary grafts, are satisfactory and thus represent a good method of treatment for patients with a sarcoma of bone or soft tissue.

Related: Bone Cancers Osteosarcoma

Lin P, Mobasher ME, Alawi F
Acute dyskerin depletion triggers cellular senescence and renders osteosarcoma cells resistant to genotoxic stress-induced apoptosis.
Biochem Biophys Res Commun. 2014; 446(4):1268-75 [PubMed] Related Publications
Dyskerin is a conserved, nucleolar RNA-binding protein implicated in an increasing array of fundamental cellular processes. Germline mutation in the dyskerin gene (DKC1) is the cause of X-linked dyskeratosis congenita (DC). Conversely, wild-type dyskerin is overexpressed in sporadic cancers, and high-levels may be associated with poor prognosis. It was previously reported that acute loss of dyskerin function via siRNA-mediated depletion slowed the proliferation of transformed cell lines. However, the mechanisms remained unclear. Using human U2OS osteosarcoma cells, we show that siRNA-mediated dyskerin depletion induced cellular senescence as evidenced by proliferative arrest, senescence-associated heterochromatinization and a senescence-associated molecular profile. Senescence can render cells resistant to apoptosis. Conversely, chromatin relaxation can reverse the repressive effects of senescence-associated heterochromatinization on apoptosis. To this end, genotoxic stress-induced apoptosis was suppressed in dyskerin-depleted cells. In contrast, agents that induce chromatin relaxation, including histone deacetylase inhibitors and the DNA intercalator chloroquine, sensitized dyskerin-depleted cells to apoptosis. Dyskerin is a core component of the telomerase complex and plays an important role in telomere homeostasis. Defective telomere maintenance resulting in premature senescence is thought to primarily underlie the pathogenesis of X-linked DC. Since U2OS cells are telomerase-negative, this leads us to conclude that loss of dyskerin function can also induce cellular senescence via mechanisms independent of telomere shortening.

Related: Apoptosis Bone Cancers Osteosarcoma

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