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).

Sehgal VN, Verma P, Sharma S
HIV/AIDS Kaposi sarcoma: the Indian perspective.
Skinmed. 2013 Nov-Dec; 11(6):375-7 [PubMed] Related Publications
A 58-year-old, nonalcoholic, nonsmoker, official in private enterprise presented with a confirmed case of human immunodeficiency virus/acquired immune deficiency syndrome (HIV/AIDS) and an itchy, violaceous skin eruption over the lower part of the left leg. He noticed a change in the color and texture of the skin for the first time 2 years ago. Ever since that time, the eruptions had insidiously progressed. He was started on highly active antiretroviral therapy (HAART), comprising 600 mg of zidovudine, 300 mg of lamivudine, and 600 mg of efavirenz for the past year. Despite the therapy, the lesions continued to progress. He claimed to be very compliant with therapy. In addition, he developed pulmonary tuberculosis during the course of the disease. Accordingly, he was treated with a daily schedule' of antitubercular therapy, comprising 450 mg of rifampicin, 300 mg of isoniazid, 1500 mg of pyrazinamide, and 800 mg of ethambutol for 9 months. He experienced a significant loss of weight and appetite. The patient was a divorcee but refused to divulge any details of his married life after a considerate and focused discussion by a trained counselor on HIV/AIDS. He emphatically denied any extramarital sexual contact, blood transfusion, and surgery in the past. Ultimately, the patient's disease status was defined by HIV viral load (< 20 copy/mL) after obtaining informed consent.

Related: Kaposi Sarcoma

Hoellen F, Waldmann A, Benthin S, et al.
The role of lymphadenectomy in uterine sarcoma: a clinical practical approach based on retrospective analysis.
Anticancer Res. 2014; 34(2):985-93 [PubMed] Related Publications
BACKGROUND: Uterine sarcomas are rare, highly aggressive tumors with an unfavorable prognosis. Only limited data on treatment and outcome are available and the role of lymphadenectomy is controversial for this particular tumor type.
PATIENTS AND METHODS: By performing a retrospective analysis, through clinical practice, we analyzed the impact of lymphadenectomy on patient outcome.
RESULTS: Out of 52 patients, 48 women underwent surgical treatment. Among those, lymphadenectomy was performed in 58%. 10% underwent pelvic and paraaortic lymphadenectomy and 48% underwent pelvic lymphadenectomy alone.
CONCLUSION: In our cohort, surgeons tended to perform paraaortic-plus-pelvic or pelvic-alone lymphadenectomy irrespective of the presence or absence of enlarged lymph nodes. Patients who underwent lymphadenectomy showed a better survival. This observation might indicate a potential role for systematic lymphadenectomy in the surgical treatment of uterine sarcoma. We review on clinical operative decisions, critically with regard to current evidence.

Nakamura T, Matsumine A, Iino T, et al.
Role of high-sensitivity C-reactive protein in the differentiation of benign and malignant soft tissue tumors.
Anticancer Res. 2014; 34(2):933-6 [PubMed] Related Publications
The aim of the present study was to determine whether serum high-sensitivity CRP (hs-CRP) levels can be used to predict the differentiation of benign soft tissue tumors and soft tissue sarcomas (STS) and whether there are any links between increased hs-CRP levels and patients' characteristics. Serum samples were collected from 14 healthy subjects, 35 patients with benign soft tissue tumors and 60 patients with STS. The Hs-CRP levels in the patients with STS were statistically higher than those observed in patients with benign soft tissue tumors (p<0.0001) and control subjects (p<0.0001). There were no significant differences in the hs-CRP levels between patients with benign soft tissue tumors and control subjects (p=0.16). In the receiver operating characteristic analysis, a value of 0.95 μg/ml was found to be an appropriate threshold for identifying patients at-risk for STS. The area under the curve was 0.747. The serum hs-CRP level exhibited a sensitivity and specificity STS of 50% and 94.3%, respectively, for identifying. The current analyses showed that an elevated hs-CRP level is associated with the presence of STS and may, therefore, be used as an additional marker for the differential diagnosis of soft tissue tumors.

Ötvös R, Juhasz A, Szalai E, et al.
Molecular typing of human herpesvirus 8 isolates from patients with Kaposi's sarcoma in Hungary.
Anticancer Res. 2014; 34(2):893-8 [PubMed] Related Publications
BACKGROUND: Kaposi's sarcoma (KS) shows a distinct geographical and ethnic distribution. Genes at both ends of the human herpesvirus 8 (HHV-8) genome have been shown to vary considerably. Seven major molecular subtypes of HHV-8 were defined based on the amino acid sequence of the open reading frame K1 (orf-K1). The aim of the present study was to characterize HHV8 isolates from hospitalized patients in Hungary.
MATERIALS AND METHODS: A total of 36 archival paraffin-embedded Kaposi's sarcoma tissue samples were collected. Polymerase chain reaction (PCR) was carried out on the extracted DNA, using specific primers for HHV-8. After identifying the presence of HHV-8 by amplification of its orf26 region, the orf-K1 region was amplified, sequenced and used for phylogenetic analysis.
RESULTS: From the 36 orf26-positive cases, orf-K1 was amplified and was analyzed successfully in 12 cases. Phylogenetic studies, based on the complete K1 gene/protein sequences, indicate that all strains belong to the A subtype. Specifically, six of them were related to the A1 subgroup, six to the A2 subgroup and three previously reported to the A3 subgroup. Nucleotide sequence data are reported and are available in the Genbank database under accession numbers KF829938-KF829947.

Related: Kaposi Sarcoma

Jahangiri FR, Al Eissa S, Jahangiri AF, Al-Habib A
Intraoperative neurophysiological monitoring during sacrectomy procedures.
Neurodiagn J. 2013; 53(4):312-22 [PubMed] Related Publications
Previously intraoperative neurophysiological monitoring (IONM) has not been used along with a computer based navigation system for en bloc resection of a sacral Ewing sarcoma. In order to improve the post-operative neurological outcome of the patient we decided to include IONM in our procedure. A partial or complete resection of a sacral tumor may result in the loss of neurological functions due to close proximity of vascular, neural, and visceral structures. A prolonged two-stage surgical procedure may be a high risk procedure for position related brachial plexus injury. An 18-year-old male presented with left lower extremity weakness, which worsened with gait. His MRI was consistent with a sacral mass causing compression on the left S1 and S2 roots. A surgical resection was planned with anterior and posterior approaches. IONM helped guide the surgical team to prevent damaging the sacral roots on the normal side (right) and position related upper extremity brachial plexus injuries. Our technique involving IONM can be used safely for accurate en bloc removal of a sacral tumor with a safe margin while protecting the neural function and minimizing recurrence. This case report demonstrates that intraoperative neurophysiological monitoring was useful in identifying and reversing impending nerve injury during sacrectomy surgery. Significant changes were seen in ulnar and posterior tibial somatosensory evoked potentials (SSEPs). We recommend that IONM should be considered for safe margin en bloc sacral tumor resection and prevention of injury to the sacral root and brachial plexus.

Related: Ewing's Sarcoma

Ben Kridis W, Khanfir A, Frikha M
Acute pancreatitis induced by anticancer chemotherapy.
Acta Clin Belg. 2013 Jul-Aug; 68(4):309-10 [PubMed] Related Publications
Drug-induced pancreatitis is rare (1.4-2%). This report describes a 20-year-old female patient who developed acute pancreatitis while being treated for neurosarcoma of abdominal wall with the ifosfamide and doxorubicin regimen. Although it is unusual, it is important to consider chemotherapeutic agents as a possible etiology for acute pancreatitis in patients presenting with gastrointestinal symptoms.

Related: Doxorubicin Ifosfamide

Eary JF, Conrad EU, O'Sullivan J, et al.
Sarcoma mid-therapy [F-18]fluorodeoxyglucose positron emission tomography (FDG PET) and patient outcome.
J Bone Joint Surg Am. 2014; 96(2):152-8 [PubMed] Article available free on PMC after 15/01/2015 Related Publications
BACKGROUND: Our previous research investigated the ability of [F-18]fluorodeoxyglucose (FDG) positron emission tomography (PET) imaging results to predict outcome in patients with sarcoma. Tumor uptake of FDG before and after neoadjuvant chemotherapy was predictive of patient outcome. With this background, a prospective clinical study was designed to assess whether tumor FDG uptake levels in the middle of neoadjuvant chemotherapy added additional prognostic information to pre-therapy imaging data.
METHODS: Sixty-five patients with either bone or soft-tissue sarcoma were treated with neoadjuvant-based chemotherapy according to the standard clinical practice for each tumor group. All patients had FDG PET studies before therapy, mid-therapy (after two cycles of chemotherapy), and before resection. Tumor FDG uptake (SUVmax, the maximum standardized uptake value) at each imaging time point, tumor type (bone or soft-tissue sarcoma), tumor size, and histopathologic grade were recorded for each patient. The time from the pre-therapy FDG PET study to events of local tumor recurrence, metastasis, or death were extracted from the clinical records for comparison with the imaging data. Univariate and multivariate analyses of the imaging and clinical data were performed.
RESULTS: Univariate and multivariate data analyses showed that the difference (measured as the percentage reduction) between the pre-therapy and mid-therapy maximum tumor uptake values added prognostic value to patient outcome predictions independently of other patient variables.
CONCLUSIONS: The utility of a tumor pre-therapy FDG PET scan as a biomarker for the outcome of patients with sarcoma was strengthened by a mid-therapy scan to evaluate the interim treatment response.

Related: Bone Cancers

Zin A, Bertorelle R, Dall'Igna P, et al.
Epithelioid rhabdomyosarcoma: a clinicopathologic and molecular study.
Am J Surg Pathol. 2014; 38(2):273-8 [PubMed] Related Publications
Rhabdomyosarcoma (RMS) is the most common pediatric soft tissue sarcoma and is mostly represented by the embryonal (ERMS) and alveolar (ARMS) histotypes. Whereas ERMS shows variable genetic alterations including TP53, RB1, and RAS mutations, ARMS carries a gene fusion between PAX3 or PAX7 and FOXO1. Epithelioid RMS is a morphologic variant of RMS recently described in adults. Five cases of epithelioid RMS were identified after histologic review of 85 cases of ARMS enrolled in Italian therapeutic protocols. Immunostaining analyses (muscle-specific actin, desmin, myogenin, AP-2β, EMA, cytokeratins, INI-1) and reverse transcription polymerase chain reaction assays to detect MyoD1, myogenin, and PAX3/7-FOXO1 transcripts were performed. In 4 cases DNA sequencing of TP53 was performed; and RB1 allelic imbalance and homozygous deletion were analyzed by quantitative real-time polymerase chain reaction. Histologically, epithelioid RMS displayed sheets of large cells without rhabdomyoblastic differentiation or anaplasia in 3 and prominent rhabdoid cells in 2; necrosis was evident in 4, often with a geographic pattern. Immunostainings for INI, desmin, myogenin (scattered cells in 4, diffuse in 1) were positive in all; EMA and MNF116 were positive in 2; AP-2β was negative. PAX3/7-FOXO1 transcripts were absent. In all cases RB1 was wild type, and a TP53 mutation at R273H codon was found in 1. All patients are in complete remission, with a median follow-up of 6 years. Epithelioid RMS may occur in children and is probably related to ERMS, as suggested by lack of fusion transcripts, weak staining for myogenin, negative AP-2β, evidence of TP53 mutation (although only in 1 case), and a favorable clinical course.

Related: Rhabdomyosarcoma

Farahat A, Magdy N, Elaffandi A
Primary myxoid chondrosarcoma of the breast.
Ann R Coll Surg Engl. 2014; 96(1):112E-411E [PubMed] Related Publications
Primary breast chondrosarcoma has been rarely reported in the literature. Conservative breast surgery has never been part of the management of previously reported cases. Surgery remains the mainstay management of such a disease as it is resistant to chemotherapy and radiotherapy. In this report, we present a case of rare primary myxoid chondrosarcoma of the breast that was managed successfully with a conservative approach.

Related: Bone Cancers Breast Cancer Chondrosarcoma

Perut F, Avnet S, Fotia C, et al.
V-ATPase as an effective therapeutic target for sarcomas.
Exp Cell Res. 2014; 320(1):21-32 [PubMed] Related Publications
Malignant tumors show intense glycolysis and, as a consequence, high lactate production and proton efflux activity. We investigated proton dynamics in osteosarcoma, rhabdomyosarcoma, and chondrosarcoma, and evaluated the effects of esomeprazole as a therapeutic agent interfering with tumor acidic microenvironment. All sarcomas were able to survive in an acidic microenvironment (up to 5.9–6.0 pH) and abundant acidic lysosomes were found in all sarcoma subtypes. V-ATPase, a proton pump that acidifies intracellular compartments and transports protons across the plasma membrane, was detected in all cell types with a histotype-specific expression pattern. Esomeprazole administration interfered with proton compartmentalization in acidic organelles and induced a significant dose-dependent toxicity. Among the different histotypes, rhabdomyosarcoma, expressing the highest levels of V-ATPase and whose lysosomes are most acidic, was mostly susceptible to ESOM treatment.

Theriot C, Hughes K, Mitchell J, Patterson B
Pelvic synovial sarcoma of unknown primary origin: case report and literature review.
J Miss State Med Assoc. 2013; 54(11):308-11, 313 [PubMed] Related Publications
A case report of a 26-year-old woman with pelvic pain for one-month duration is presented. The physical exam was unremarkable, but a right pelvic mass was found on ultrasound. Computed tomography (CT) of the abdomen/pelvis confirmed a heterogeneous 6 x 4.4 cm mass within the right pelvis adjacent to the superior-lateral aspect of the uterus. Laparoscopy was performed, and the mass was found to be friable and necrotic. The origin of the mass was not able to be determined due to the collapse of the mass upon instrumentation. Histology revealed spindle cells. Further studies, including immunochemical staining, revealed a synovial sarcoma. Patient underwent laparotomy for staging and in hopes of obtaining clear tumor margins. Surgery revealed subsequent tumors involving the mesentery of the small intestine and the peritoneal wall. Biopsies taken at surgery did not reveal the primary origin of the original pelvic tumor.

Related: Cancer of Unknown Primary

Gajewski P, Błaszak-Ciećwierska W, Chojnowski S, et al.
Angiosarcoma of the heart - a diagnostic pitfall.
Kardiol Pol. 2013; 71(12):1296-8 [PubMed] Related Publications
Primary tumours of the heart are extremely rare. Sarcomas are considered to be the most frequent histological type. This study presents two cases with a two-week and two-month history of fatigue, fever and shortness of breath. In both cases, cardiac tumours with pericardial effusion were diagnosed by means of echocardiography, being responsible for the above-mentioned symptoms. At the beginning of the diagnostic process, sarcomas seemed to be the likeliest cause of these symptoms. Establishment of the histopathological diagnosis based on the tumour biopsy turned out to be very difficult, and this delayed further therapeutic procedures.

Liu Y, Ishibashi H, Sako S, et al.
A giant mesentery malignant solitary fibrous tumor recurring as dedifferentiated liposarcoma- a report of a very rare case and literature review.
Gan To Kagaku Ryoho. 2013; 40(12):2466-9 [PubMed] Related Publications
We report a case of a 59-year-old woman with a very rare giant mesentery malignant solitary fibrous tumor that recurred as dedifferentiated liposarcoma. The woman was admitted to the hospital because of low abdominal pain. Radiological and biopsy findings revealed a multi-lobulated giant malignant solitary fibrous tumor that had invaded the inferior vena cava, abdominal aorta, and superior mesentery vessels. The tumor was completely removed during the first cytoreductive surgery. Histopathologically, tumor had a heterogeneous cell population, composed of spindle cells with fibrous collagen proliferation. The spindle cells were not arranged in a specific pattern. Immunohistochemistry revealed that the tumor cells were positive for CD34, CD99, Bcl-2, and smooth muscle actin( SMA) and negative for CD117, epithelial membrane antigen (EMA), CAM5.7, S100, desmin, and caldesmon. The tumor recurred 9 months after surgery, and another cytoreductive surgery was then performed. The postoperative histopathological appearance of the invaded area indicated a well-differentiated liposarcoma. Formation of tumorous bone was also noted in the same area, in addition to atypical mesenchymal cells and multi-vacuolated lipoblasts in the area of the well-differentiated liposarcoma. Proliferated spindle cells arranged in a storiform pattern were found in the area adjacent to the tumor. Immunohistochemical analysis revealed that the tumors cells were positive for SMA, HHF-35, and caldesmon and negative for CD117, CD34, and S100. A diagnosis of dedifferentiated liposarcoma was made.

Azzariti A, Porcelli L, Mangia A, et al.
Irradiation-induced angiosarcoma and anti-angiogenic therapy: a therapeutic hope?
Exp Cell Res. 2014; 321(2):240-7 [PubMed] Related Publications
Angiosarcomas are rare soft-tissue sarcomas of endothelial cell origin. They can be sporadic or caused by therapeutic radiation, hence secondary breast angiosarcomas are an important subgroup of patients. Assessing the molecular biology of angiosarcomas and identify specific targets for treatment is challenging. There is currently great interest in the role of angiogenesis and of angiogenic factors associated with tumor pathogenesis and as targets for treatment of angiosarcomas. A primary cell line derived from a skin fragment of a irradiation-induced angiosarcoma patient was obtained and utilized to evaluate cell biomarkers CD31, CD34, HIF-1 alpha and VEGFRs expression by immunocytochemistry and immunofluorescence, drugs cytotoxicity by cell counting and VEGF release by ELISA immunoassay. In addition to previous biomarkers, FVIII and VEGF were also evaluated on tumor specimens by immunohistochemistry to further confirm the diagnosis. We targeted the VEGF-VEGFR-2 axis of tumor angiogenesis with two different class of vascular targeted drugs; caprelsa, the VEGFR-2/EGFR/RET inhibitor and bevacizumab the anti-VEGF monoclonal antibody. We found the same biomarkers expression either in tumor specimens and in the cell line derived from tumor. In vitro experiments demonstrated that angiogenesis plays a pivotal role in the progression of this tumor as cells displayed high level of VEGFR-2, HIF-1 alpha strongly accumulated into the nucleus and the pro-angiogenic factor VEGF was released by cells in culture medium. The evaluation of caprelsa and bevacizumab cytotoxicity demonstrated that both drugs were effective in inhibiting tumor proliferation. Due to these results, we started to treat the patient with pazopanib, which was the unique tyrosine kinase inhibitor available in Italy through a compassionate supply program, obtaining a long lasting partial response. Our data suggest that the study of the primary cell line could help physicians in choosing a therapeutic approach for patient that almost in vitro shows chances of success and that the anti-angiogenetic agents are a reliable therapeutic opportunity for angiosarcomas patients.

Related: Angiogenesis Inhibitors Breast Cancer

Bruckner BA, Rodriguez LE, Bunge R, et al.
Large cardiac tumor managed with resection and two ventricular assist devices.
Ann Thorac Surg. 2014; 97(1):321-4 [PubMed] Related Publications
Symptomatic cardiac tumors can lead to a rapid clinical deterioration and death. Prompt surgical resection is ideal in this situation as it is the only proven treatment to date. We report the radical resection of a large malignant cardiac tumor that obstructed the right ventricular outflow tract. Extensive resection precluded reconstruction and limited the ability to implant a total artificial heart; thus, 2 paracorporeal devices were implanted instead.

Choi LE, Healey JH, Kuk D, Brennan MF
Analysis of outcomes in extraskeletal osteosarcoma: a review of fifty-three cases.
J Bone Joint Surg Am. 2014; 96(1):e2 [PubMed] Related Publications
BACKGROUND: Extraskeletal osteosarcoma is a rare soft-tissue sarcoma about which little is known. The objectives of this study were to describe the clinical features and natural history of extraskeletal osteosarcoma and to investigate factors affecting outcomes.
METHODS: A retrospective review of a prospectively maintained database of patients diagnosed with soft-tissue sarcoma was conducted. Patients with pathologically confirmed extraskeletal osteosarcoma from 1982 to 2012 were identified and were included in the analysis. Medical records were reviewed for clinical features, treatment, and outcomes.
RESULTS: Fifty-three patients were identified from the database: forty-two presented with localized disease, two presented with metastatic disease, and nine presented with recurrent (local and/or distant) disease. The median patient age at diagnosis was sixty-four years, with a median follow-up time of thirty-four months (range, one to 290 months) for survivors. Of the fifty-three patients who were identified, forty-one had lesions in the extremities, fifty-one had high-grade lesions, forty had lesions >5 cm, and forty-two had deep lesions. For patients presenting with localized disease, the median survival was 45.8 months with a three-year cumulative incidence of death due to disease of 39%. All patients with localized disease were managed with surgical resection of the primary tumor: nineteen with surgery only, ten with adjuvant radiation, five with adjuvant chemotherapy, and eight with both radiation and chemotherapy. Eighteen patients relapsed: two patients had local recurrences, ten patients had distant metastases, and six patients had local recurrences and distant metastases. In log-rank analysis, patients with superficial tumors and negative margins at resection had a higher three-year event-free survival. No significant association of disease-specific or event-free survival was found with the addition of radiation, chemotherapy, or both to surgery.
CONCLUSIONS: For patients presenting with localized extraskeletal osteosarcoma, three-year event-free survival was higher for patients with superficial tumors and negative margins at resection. Radiation and chemotherapeutic treatment were not associated with a lower incidence of death due to disease or a longer event-free survival.

Related: Osteosarcoma

Fasina O
Pattern of presentation and outcome of ophthalmic rhabdomyosarcoma in Ibadan.
Afr J Med Med Sci. 2013; 42(2):165-9 [PubMed] Related Publications
BACKGROUND: Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma, and the commonest primary orbital malignancy in children. Studies on ophthalmic RMS are scanty in Nigeria, and other parts of Africa. This study thus describes the pattern of ophthalmic RMS in Ibadan and treatment outcome.
METHOD: A retrospective, non-comparative, consecutive, interventional analysis of patients with ophthalmic RMS in an ocular oncology unit of a teaching hospital over 20 years was carried out.
RESULTS: Records of 22 patients, 15 males and 7 females, aged 2 months to 50 years (Median 7 years, Range 5-13 years) were analyzed. All patients presented with visual loss, orbital swelling and proptosis. Histological examination revealed embryonal RMS in 16 (72.7%) patients, alveolar in three (13.6%), pleomorphic in two (9.1%) and "not otherwise stated" in one (4.5%). Treatment outcome was poor as only two (9.1%) patients remained tumour-free 12 and 36 months after diagnosis. Three (13.6%) patients died on admission, while two (9.1%) patients are presently undergoing treatment. Fifteen (68.2%) patients were lost to follow-up, financial constraint and perceived poor response to treatment being the main reasons for incomplete and inconsistent treatment regimen and resultant poor outcome.
CONCLUSION: Demography and morphology of ophthalmic RMS in Ibadan is comparable to other studies. However, treatment outcome remains poor, mainly due to late presentation, incomplete and inconsistent treatment. Strengthening the national health insurance scheme to cater for the indigent and increased public enlightenment on early presentation of cases may go a long way in improving the outcome in our patients.

Related: Rhabdomyosarcoma

Zhang Y, Ma Q, Liu T, et al.
Tumor self-seeding by circulating tumor cells in nude mouse models of human osteosarcoma and a preliminary study of its mechanisms.
J Cancer Res Clin Oncol. 2014; 140(2):329-40 [PubMed] Related Publications
PURPOSE: The purpose of this study is to determine whether and how tumor self-seeding by circulating tumor cells (CTCs) plays a role in the initiation and progression of osteosarcoma.
METHODS: Two different nude mouse models of human osteosarcoma were established for detecting tumor self-seeding by fluorescently labeled CTCs. Various tumor growth indicators were quantitated for seeded and unseeded groups. Growth mechanisms were characterized using cell proliferation assays and immunohistochemical staining. Conditioned media of primary osteosarcoma cells was characterized in a Transwell migration assay and enzyme-linked immunosorbent assay. The effect of cytokines secreted by primary tumor cells was verified by small interfering RNA and recombinant human cytokine experiments.
RESULTS: Red fluorescent protein-labeled CTCs seeded primary tumors in both models. Seeded primary tumors groups grew faster than control groups (P < 0.05), which was partially attributed to the CTCs having a higher proliferation rate and higher vascular endothelial growth factor expression after self-seeding. Conditioned media of primary osteosarcoma cells attracted CTCs, through an IL-6-dependent mechanism.
CONCLUSIONS: CTC tumor self-seeding occurs in osteosarcoma and promotes the growth of primary osteosarcoma. CTCs appear to be recruited by cytokines secreted by primary osteosarcoma cells, particularly IL-6.

Related: Bone Cancers Osteosarcoma VEGFA

Kohen D, Dross P
Angiosarcoma of the scalp.
Del Med J. 2013; 85(9):269-72 [PubMed] Related Publications
Cutaneous angiosarcoma of the scalp is a rare malignant neoplasm of endothelial cells which occurs predominantly in elderly Caucasian males. The etiology is unknown; however the most common association is prior radiation exposure. The diagnosis is often delayed due to misinterpretation for other lesions, including trauma-related bruising and infectious processes such as abscess. Radiologic imaging is non-diagnostic but can be helpful in defining the extent of local disease, as well as distant metastases. Prognosis is poor with a five-year survival rate of less than 30 percent. Treatment guidelines are not well established; however the mainstay is surgical resection with wide margins followed by adjuvant radiation therapy. Prognosis remains poor with a local recurrence rate of greater than 80 percent. Adjuvant chemotherapy is also controversial but may play a role in short term palliation of metastatic or locally advanced disease. Current investigations using molecular targeted therapy are underway with the goal of eliminating systemic side effects.

Related: Head and Neck Cancers Head and Neck Cancers - Molecular Biology Skin Cancer

Franceschini D, Scotti V, Simontacchi G, et al.
Application of helical tomotherapy for the treatment of a right atrium angiosarcoma: a case report.
Tumori. 2013 Sep-Oct; 99(5):e233-6 [PubMed] Related Publications
We report the case of a 48-year-old woman affected by inoperable primary angiosarcoma of the right atrium who was treated with 4 cycles of induction chemotherapy and radical radiotherapy. We present the application and the result of helical tomotherapy for this patient. We also describe the technical aspects of the simulation, planning, setup and delivery of radiotherapy. At 16 months after the diagnosis the patient is still in good condition without any symptoms and with a partial response of the lesion. This case shows the feasibility of treatment with high doses of radiation for a primary unresectable cardiac sarcoma.

Related: Epirubicin Ifosfamide

Ravo V, Falivene S, De Chiara A, et al.
Treatment of cutaneous angiosarcoma of the face: efficacy of combined chemotherapy and radiotherapy.
Tumori. 2013 Sep-Oct; 99(5):e211-5 [PubMed] Related Publications
INTRODUCTION: Cutaneous angiosarcoma (AS) is a rare form of soft tissue sarcoma. It is aggressive and has a poor prognosis. The aim of our report is to show that with combined chemotherapy and radiotherapy it is possible to obtain good results in terms of local control, complete response, and aesthetic outcome.
CASE REPORT: We present the case of a 60-year-old man affected by AS covering the entire surface of the nose. Surgery, although indicated, was excluded because it was considered mutilating and would give a poor cosmetic result. The patient was treated with chemotherapy consisting of paclitaxel 80 mg/m2 for 6 cycles followed by radiotherapy at a dose of 50 Gy. Then 3 additional cycles of chemotherapy were administered according to the same scheme. A complete response was obtained. At 40 months after treatment, the patient did not show any signs of late toxicity, all lesions had disappeared, and all laboratory tests were negative.
CONCLUSIONS: Our experience shows that concomitant chemoradiotherapy can be delivered safely and can be tolerated with low toxicity and good results in terms of local control and complete response. We obtained an excellent aesthetic result with improvement of the patient's quality of life.

Related: Paclitaxel Skin Cancer

Torretta S, Gaffuri M, Recalcati S, et al.
Pharyngolaryngeal location of Kaposi's sarcoma with airway obstruction in an HIV-negative patient.
Tumori. 2013 Sep-Oct; 99(5):e208-10 [PubMed] Related Publications
Kaposi's sarcoma (KS) is a human herpes virus-8 (HHV-8)-associated angioproliferative disorder, and its occurrence may be favored by human immunodeficiency virus (HIV) infection and iatrogenic immunosuppression. It has also been postulated that a chronic inflammatory disease of the skin can pave the way to its development. KS generally involves mucosal and cutaneous sites, including the head and neck. An oropharyngeal location is quite common, but laryngeal involvement with possible upper airway obstruction and respiratory distress requiring tracheotomy is rare, and no hypopharyngeal locations have yet been reported. We describe the case of a 68-year-old male patient who developed KS after immunosuppressive treatment for pemphigus vulgaris, an autoimmune bullous disease presenting with blisters and erosions on the skin and the oral mucosa. KS was initially localized to the oral cavity and oropharynx, but subsequent involvement of the laryngeal and hypopharyngeal tract led to acute airway obstruction and the need for tracheotomy. This unique case of pharyngolaryngeal KS suggests that clinicians faced with purple nodular lesions should consider a differential diagnosis of KS in immunocompromised patients, even if they are HIV negative, and should carefully manage the patency of the upper airways.

Related: Cancer of the Larynx Laryngeal Cancer - Molecular Biology Kaposi Sarcoma

Elbashier SH, Nazarina AR, Looi LM
Cytokeratin immunoreactivity in Ewing sarcoma/ primitive neuroectodermal tumour.
Malays J Pathol. 2013; 35(2):139-45 [PubMed] Related Publications
Ewing sarcoma (ES)/ primitive neuroectodermal tumour (PNET) is an aggressive malignant neoplasm affecting mainly children and young adults. The tumour is included with other primitive neoplasms under the category of small round cell tumour. Cytokeratin expression in ES/PNET has been described in sporadic case reports as well as a few systemic series. We studied this feature in Malaysian patients diagnosed in University Malaya Medical Centre on the basis of typical morphology and immunohistochemical assays. Immunohistochemical staining for AE1/AE3 and MNF116 were performed in 43 cases. Cytokeratin was expressed in 17 cases (39.5%) in focal, intermediate or diffuse patterns. There was no significant association between cytokeratin immunoreactivity and the following parameters: patient age, sex, skeletal and extraskeletal primary location as well as primary, metastastic or recurrent tumours or chemotherapy treatment. A significant association between cytokeratin and neuron specific enolase (NSE) expression was demonstrated. Our study supports evidence of epithelial differentiation in ES/PNET and emphasizes that the expression of cytokeratin does not exclude ES/PNET in the differential diagnosis of small round cell tumours.

Related: Ewing's Sarcoma

Karamanou M, Antoniou C, Stratigos AJ, et al.
The eminent dermatologist Moriz Kaposi (1837-1902) and the first description of idiopathic multiple pigmented sarcoma of the skin.
J BUON. 2013 Oct-Dec; 18(4):1101-5 [PubMed] Related Publications
In 1872, the Hungarian born dermatologist Moriz Kaposi that was practicing in Vienna first described a rare endemic disease that bears his name, among elderly persons of Central European or Mediterranean origin named "idiopathic multiple pigmented sarcoma of the skin". Ten years later the Italian dermatologist Tommaso de Amicis confirms Kaposi's findings. For more than a century the disease was known as a rare low grade malignancy till the 1980s AIDS epidemic.

Related: Kaposi Sarcoma Skin Cancer

Wu Z, Ma C, Shan Z, et al.
Histone deacetylase inhibitors suppress the growth of human osteosarcomas in vitro and in vivo.
J BUON. 2013 Oct-Dec; 18(4):1032-7 [PubMed] Related Publications
PURPOSE: Although the antitumor efficacy of histone deacetylase inhibitors (HDACIs) has been referred to as a promising new treatment strategy in malignancies, how they exert their effects on human osteosarcoma in vitro and in vivo is yet not well understood. In this study, we employed HDACIs suberoylanilide hydroxamic acid (SAHA) and sodium butyrate (SB) to investigate their effects on human osteosarcoma in vitro and in vivo.
METHODS: The in vitro effects of HDACIs SAHA and SB were evaluated in SaOS2 and U2OS human osteosarcoma cell lines. Cell growth, cell cycle progression and histone acetylation were investigated by MTS, flow cytometry and western blotting, respectively. In addition, SAHA or SB was administered for 4 weeks in mice xenograph models for assessing the in vivo effects.
RESULTS: MTS assays revealed that SAHA and SB significantly suppressed the growth of SaOS2 and U2OS cells in a concentration-dependent manner. Western blotting analysis indicated that the levels of acetylated H3 were increased after HDACIs treatment. Flow cytometry showed that SAHA arrested the cell cycle in G1 and G2/M phase, while SB arrested the cell cycle in G2/M phase. The tumor growth of mice xenograph models with SaOS2 was inhibited by SAHA and SB compared with vehicle control.
CONCLUSION: HDACIs SAHA and SB significantly inhibit the growth of human osteosarcoma cells and induce cell cycle arrest. The tumor inhibitory effects were also validated in mice xenograft models.

Related: Bone Cancers Osteosarcoma

Stemme S, Ghaderi M, Carlson JW
Diagnosis of endometrial stromal tumors: a clinicopathologic study of 25 biopsy specimens with identification of problematic areas.
Am J Clin Pathol. 2014; 141(1):133-9 [PubMed] Related Publications
OBJECTIVES: To assess the difficulties associated with diagnosing endometrial stromal tumors (ESTs) on endometrial biopsy.
METHODS: We examined 25 endometrial biopsy specimens from 19 consecutive women diagnosed with either endometrial stromal nodule (n = 3) or endometrial stromal sarcoma (n = 16).
RESULTS: Rereview of the biopsy specimens revealed a stromal fragment suspicious for an EST in 16, of which eight had received a benign diagnosis on initial review. Most ESTs had an aglandular stromal fragment that was 5 mm or larger. Stromal fragments of this size were not encountered in the control material. Problematic areas included highly cellular leiomyoma and a lack of attention to the stromal compartment.
CONCLUSIONS: Most endometrial stromal tumors present with large aglandular stromal fragments (≥5 mm). These fragments are large enough that difficulties in diagnosis appear to be due to a lack of attention to the stromal compartment.

Related: Endometrial (Uterus) Cancer Endometrial Cancer

Dei Tos AP
A current perspective on the role for molecular studies in soft tissue tumor pathology.
Semin Diagn Pathol. 2013; 30(4):375-81 [PubMed] Related Publications
Soft tissue sarcomas represent an heterogenous group of malignancies. They represent a diagnostic challenge, and their accurate classification impact over treatment options. Sarcomas, similarly to hematologic neoplasm, often harbor relatively specific genetic aberrations, the recognition of which can be used to improved diagnostic accuracy. This review will focus on the clinical relevance of molecular analysis in soft tissue sarcomas, trying to elucidate its role as a diagnostic tool as well as a potential prognostic/predictive marker.

Gaber MA, Bakry OA, Shehata WA
Isolated oral Kaposi sarcoma in an HIV-negative patient: a case report.
Anal Quant Cytol Histol. 2013; 35(4):237-40 [PubMed] Related Publications
BACKGROUND: Kaposi sarcoma is a well-known vascular tumor first described by Moriz Kaposi in 1872. Oral involvement is seen as an AIDS-related malignant neoplasm but is rarely described in HIV-negative and non-immunosuppressed individuals.
CASE: We report a case of oral Kaposi sarcoma in a 75-year-old, HIV-negative woman. Diagnosis was achieved according to clinical, histopathological and positive polymerase chain reaction for human herpes virus 8. The tumor was surgically excised and no recurrence was detected in the following 6 months.
CONCLUSION: Oral Kaposi sarcoma is rare in HIV-negative patients and is associated with HHV-8 infection. Lesions are usually solitary and can be treated surgically. It should be included in the differential diagnoses of oral lesions that are clinically suspicious and resistant to therapy.

Related: Oral Cancer Kaposi Sarcoma

Yagishita S, Horinouchi H, Yorozu T, et al.
Secondary osteosarcoma developing 10 years after chemoradiotherapy for non-small-cell lung cancer.
Jpn J Clin Oncol. 2014; 44(2):191-4 [PubMed] Related Publications
A 53-year-old female patient was admitted with pain and a progressively enlarging mass in the right upper chest. Chest computed tomography revealed a mass lesion in the region of the right upper ribs. Ten years prior to this admission, the patient had undergone right lobectomy for lung adenocarcinoma. One year after the surgery, follow-up computed tomography had revealed tumor recurrence in the mediastinal and supraclavicular lymph nodes, and the patient had been treated by chemoradiotherapy. Thereafter, regular follow-up had revealed no evidence of recurrence of the non-small-cell lung cancer. Histopathological findings revealed proliferation of spindle-shaped malignant tumor cells in a background of osteoid, consistent with the diagnosis of osteosarcoma. The location of the tumor was consistent with the radiation field. Based on the clinicopathological findings, the patient was diagnosed as having secondary osteosarcoma occurring as a result of the chemoradiotherapy administered previously for the recurrent non-small-cell lung cancer. Unfortunately, the patient died of rapid progression of the osteosarcoma within a week of admission to the hospital. The autopsy revealed contiguous invasion by the tumor of the heart, with massive thrombus formation. The peripheral pulmonary arteries were diffusely occluded by metastatic tumors. Our case serves to highlight the risk of development of secondary sarcoma as a life-threatening late complication after chemoradiotherapy for locally advanced non-small-cell lung cancer, even after complete cure of the primary tumor.

Related: Bone Cancers Non-Small Cell Lung Cancer Lung Cancer Osteosarcoma

Kiuru M, McDermott G, Coit DC, et al.
Basal cell carcinosarcoma with PTCH1 mutations in both epithelial and sarcomatoid primary tumor components and in the sarcomatoid metastasis.
Am J Surg Pathol. 2014; 38(1):138-42 [PubMed] Related Publications
Basal cell carcinosarcoma is a rare biphenotypic malignant skin tumor, in which one tumor component has light microscopic features of basal cell carcinoma, whereas the other has features of sarcoma. Clinical experience with this tumor is limited, and associated molecular genetic alterations are unknown. Herein, we report a unique case of metastatic basal cell carcinosarcoma, in which we analyzed the 2 components of the primary tumor as well as the metastasis by next-generation sequencing. The patient was a 72-year-old man who presented with a 7-year history of a large tumor of the left forearm. The tumor showed mixed features of basal cell carcinoma and undifferentiated sarcoma. The patient underwent a wide local excision and sentinel lymph node biopsy, which revealed microscopic subcapsular deposits of metastatic sarcomatoid tumor. One year later, intra-abdominal metastatic tumor was detected and resected. It had sarcomatoid features by light microscopy and failed to stain for epithelial markers by immunohistochemistry. DNA was extracted separately from the epithelial and sarcomatoid component of the primary tumor, intra-abdominal metastasis, and normal tissue. All exons of 230 cancer-associated genes were sequenced to an average read depth of >500-fold. This revealed multiple identical mutations in epithelial and sarcomatoid tumor compartments. Both compartments harbored 2 identical mutations, a truncating and a missense mutation, in the patched gene (PTCH1). This finding is not only of interest for a shared heritage of different subpopulations in a biphenotypic tumor, but also relevant clinically. It provides a rationale for the clinical use of hedgehog pathway inhibitors for treatment of patients affected by this tumor. Unfortunately, the patient reported herein died of metastatic disease before targeted therapy could be initiated.

Related: Skin Cancer

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