Tissue Sarcoma In Adults Research Articles

Prognostic factors of patients with extremity myxoid liposarcomas after surgery

BackgroundExtremity myxoid liposarcoma (MLS) is a rare soft tissue sarcoma in adults. We performed this study to define distinctive clinical features of extremity MLS by assessing prognostic factors.MethodsBetween 1973 and 2015, 1756 patients with extremity MLS who underwent surgical resection were retrieved from the Surveillance, Epidemiology, and End Results (SEER) database of the US National Cancer Institute. Both overall survival (OS) and cancer-specific survival (CSS) were assessed using the Kaplan–Meier method (to obtain OS and CSS curves) and a Cox proportional hazards regression model.ResultsOf the 1756 patients with extremity MLS, the mean and median patient age at diagnosis were 47 and 45 years, respectively. More than half (n = 1027, 58.5%) of the patients were male. In terms of location, 10.5% tumors were located in the upper limbs and 89.5% in lower limbs. All patients received local surgery, and about half of the patients (57.2%) received radiation treatment. The 5- and 10-year OS rates of the entire cohort were 86.4% and 75.9%, respectively. The 5- and 10-year CSS rates were 90.5% and 85.2%, respectively. On multivariate analysis, older age, male gender, high tumor grade, and tumor size > 10 cm were found to be independent risk factors of both decreased OS and CSS. Year of diagnosis ≥ year 2000 was significantly associated with an increased CSS. In addition, radiation treatment failed to become an independent risk factor for either OS or CSS.ConclusionWe identified age, gender, tumor grade, year of diagnosis, and tumor size as independent prognostic factors for OS and CSS in patients with extremity MLS.

Liposarcoma — spectrum of disease

Liposarcomas are the most common soft tissue sarcomas in adults. Diagnosis and treatment of liposarcoma should always be planned and conducted in centres experienced in the treatment of these heterogeneous malignancies with different prognosis and sensitivity to the treatment used. In the following paper, we present a summary of current knowledge about liposarcomas considering the differences between subtypes and new directions in treatment.

Surgical approach on Reccurent Retroperitoneal Liposarcoma with Involvement of Surrounding Organs

Background: Retroperitoneal sarcomas are neoplasm that are only 0,3-3% of all solid tumors. Liposarcomas is most freguent soft tissue sarcomas in adults. Symptoms would only be detected if the liposarcoma presses on the surrounding organs.
 Case presentation: A 58-year-old woman presented with complaint of progressive abdominal distension, loss of appetite, malaise, constipation and weight loss of about 7 kg. Abdominal CT scanning showed the presence of a retroperitoneal tumor that occupies almost the entire right part of abdominal cavity. 16 months earlier, the patient was operated in another surgical facility due to liposarcom, derived from retroperitoneum (Histopathological finding: Poorly differentiated (G3) liposarcoma pleomorphic cell type). The patient was operated under the diagnosis of retroperitoneal liposarcoma.
 Conclusions: Treatment of choice is radical surgical procedure including elimination of all the structures and organs involved by the tumour process - RO resection as a basic principle of surgical treatment.

Adult Pleomorphic Rhabdomyosarcoma of the Peroneal Compartment; Rare Case Report

Pleomorphic Rhabdomyosarcoma (RMS) is a rare form of soft tissue sarcoma in adults. Due to the rarity of adult RMS, information regarding its clinical and biologic characteristics is very limited. I report a case of embryonal RMS of lower extremity involving the right leg peroneal compartment in a 37-year-old man. There was metastasis of the tumor to the ipsilateral inguinal lymph nodes at diagnosis. The patient underwent surgery with enbloc excision of the tumor from the peroneal compartment and ipsilateral inguinal lymphnode biopsy. Histopathological examination revealed high-grade pleomorphic RMS and postoperatively he under went chemotherapy and six cycles of radiotherapy.

Characteristics of Liposarcoma Patients: 5-Year Data

Background : Liposarcoma is the second highest soft tissue sarcoma in adults with prevalence of 15% to 25%. If the size of liposarcoma is very large, it may cause abdominal pain, weight loss, gastrointestinal bleeding, even kidney failure. Therefore, for early detection of liposarcoma, the characteristics of liposarcoma should be investigated. This study aimed to determine liposarcoma characteristics based on age, sex, location and histopathologic type according to WHO (World Health Organization) Classification of Tumors 2013. Methods : A descriptive study was conducted from August to October 2015 at the Department of Anatomic Pathology, Dr. Hasan Sadikin General Hospital, Bandung, Indonesia. Data was obtained from database of patients with liposarcoma at the Department of Anatomic Pathology in the period of January 2010 to December 2014. The collected data was presented in the form of figures and tables. Results : Out of 102 cases, the percentage of liposarcoma was highest between the age of 50 and 59 years (31.37%), followed by the age of 40–49 years (28.43%). The percentage were slightly higher in males (54.9%) compared to females with a ratio of 1.22:1. Femur (23.52%), intraabdomen (22.55%) and retroperitoneum (11.76%) were the most common locations affected. The most common histopathologic subtype was myxoid liposarcoma (52.95%) exceeding a half of the total cases. Conclusions : Liposarcoma most likely occurs in the age range of 40–60 years, the percentage is slightly higher in males, and abdomen and lower extremities are the most common location affected. Myxoid liposarcoma is the most common subtype.

Malignant fibrous histiocytoma of the right upper leg: A case report

Introduction. Malignant fibrous histiocytoma is a fast spreading pleomorphic sarcoma with a high malignant potential. Its spreading is characterized with local invasion and distant metastazes with early onset. Most common localisations of development are extremities, trunk and retroperitoneum. Given the line of rare case and specimen, lack of a clear etiology and mechanisms of this disease, as well as adequate histopathologic findings and intraoperative documentation, we presented current status, discuss putative etiology, histopathology with variant morphology, differential diagnosis and treatment modalities. Case report. We presented a 56-years-old female Serbian with tumor in the thigh that clinically resembles incapsulated hematoma. Computed tomography revealed intramuscular tumor with a heterodense structure and compression on surround tissue. Ex tempore biopsy specimen showed malignant potential of the tumor. Wide and radical excision of the nodule has been done, and definitive histopathological verification revealed malignant fibrous histiocytoma. Conclusion. Malignant fibrous histiocytoma is a most common type of soft tissue sarcomas in adults. Frequent localization is on lower extremities, and every rapidly enlarging nodule in this localization that on computed tomography is like incapsulated hematoma with necrotic zone should alert suspicion on presence of this type of sarcoma.

Soft Tissue and Uterine Leiomyosarcoma.

Leiomyosarcoma (LMS) is one of the most common subtypes of soft tissue sarcoma in adults and can occur in almost any part of the body. Uterine leiomyosarcoma is the most common subtype of uterine sarcoma. Increased awareness of this unique histology has allowed for the development of drugs that are specific to LMS and has begun to shed light on the similarities and possible unique aspects of soft tissue and uterine LMS. In this review, we summarize the current understanding of the epidemiology, diagnosis, genomics, and treatment options for LMS.

Soft tissue sarcomas in adults

Soft tissue sarcomas in adults

MiR-193b-Regulated Signaling Networks Serve as Tumor Suppressors in Liposarcoma and Promote Adipogenesis in Adipose-Derived Stem Cells.

Well-differentiated and dedifferentiated liposarcomas (WDLS/DDLS) account for approximately 13% of all soft tissue sarcoma in adults and cause substantial morbidity or mortality in the majority of patients. In this study, we evaluated the functions of miRNA (miR-193b) in liposarcoma in vitro and in vivo Deep RNA sequencing on 93 WDLS, 145 DDLS, and 12 normal fat samples demonstrated that miR-193b was significantly underexpressed in DDLS compared with normal fat. Reintroduction of miR-193b induced apoptosis in liposarcoma cells and promoted adipogenesis in human adipose-derived stem cells (ASC). Integrative transcriptomic and proteomic analysis of miR-193b-target networks identified novel direct targets, including CRK-like proto-oncogene (CRKL) and focal adhesion kinase (FAK). miR-193b was found to regulate FAK-SRC-CRKL signaling through CRKL and FAK. miR-193b also stimulated reactive oxygen species signaling by targeting the antioxidant methionine sulfoxide reductase A to modulate liposarcoma cell survival and ASC differentiation state. Expression of miR-193b in liposarcoma cells was downregulated by promoter methylation, resulting at least in part from increased expression of the DNA methyltransferase DNMT1 in WDLS/DDLS. In vivo, miR-193b mimetics and FAK inhibitor (PF-562271) each inhibited liposarcoma xenograft growth. In summary, miR-193b not only functions as a tumor suppressor in liposarcoma but also promotes adipogenesis in ASC. Furthermore, this study reveals key tyrosine kinase and DNA methylation pathways in liposarcoma, some with immediate implications for therapeutic exploration. Cancer Res; 77(21); 5728-40. ©2017 AACR.

Diagnostic value of 18F-FDG-PET/CT for the follow-up and restaging of soft tissue sarcomas in adults.

The purpose of this study was to evaluate the clinical utility of 2-[18F] fluoro-2-deoxy-D-glucose (18FDG) positron emission tomography (PET)/computed tomography (CT) (18F-FDG-PET/CT) in the follow-up of adult patients with soft tissue sarcomas. We prospectively evaluated 37 consecutive patients with known soft tissue sarcoma with 18F-FDG-PET/CT examination for suspected recurrence of disease. They were 21 men and 16 women with a mean age of 49.6±10.6 (SD) years (range, 34-75years). The sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV) and accuracy of 18F-FDG-PET/CT examination were calculated on a per patient basis. 18F-FDG-PET/CT showed an overall diagnostic accuracy of 91.8%, sensitivity of 90% and a specificity of 100%. The positive predictive value and negative predictive value were 100 and 70%, respectively. The 18F-FDG-PET/CT interpretations were correct in 34/37 patients (91.8%). Incorrect interpretations occurred in three patients (8.1%). Reasons for false negative findings were low 18F-FDG uptake of local recurrence in one patient and low 18F-FDG uptake of subcentimetric inguinal lymph node metastases. 18F-FDG-PET/CT has a high diagnostic value in the follow-up of patients with soft tissue sarcoma.

Primary Pulmonary Malignant Fibrous Histiocytoma.

Malignant fibrous histiocytoma (MFH) cases are classified within the group of nonclassified sarcomas. The etiopathogenesis is unclear; however, MFH commonly develops in scar tissue and in areas exposed to radiation. MFH is the most common soft tissue sarcoma in adults and may be borne in the lungs, chest wall, mediastinum, or other tissues. Primary MFH of the lung constitutes less than 0.2% of all pulmonary neoplasms; thus, an optimal treatment strategy has not yet been elucidated. We aimed to report a case of MFH of the lung with subsequent treatment administration.

Undifferentiated Pleomorphic Sarcoma Originating in the Porta Hepatis: Report of a Rare Case

Undifferentiated pleomorphic sarcoma (UPS) is a soft tissue sarcoma in adults. Although it can occur in any part of the body, its most common site is the extremities, followed by the trunk and retroperitoneum. Here, we describe a rare case of UPS originating in the porta hepatis of an 83-year-old man that was incidentally detected via contrast-enhanced computed tomography. Because malignancy was suspected, we performed a tumor resection with cholecystectomy. Consistent with a diagnosis of UPS, histopathology revealed highly cellular lesions with scattered pleomorphic spindle cell fascicles arranged in a storiform pattern; further, results of tests for all immunohistochemical markers were negative. One year after surgery, the patient is currently doing well without any evidence of tumor recurrence. To our knowledge, this is the first report of UPS originating in the porta hepatis.

Olaratumab: First Global Approval.

Olaratumab (Lartruvo™) is a fully human IgG1 monoclonal antibody targeted against the human platelet-derived growth factor (PDGF) receptor α (PDGFRα). It was developed by Eli Lilly and Co. (previously ImClone Systems) after PDGFRα was identified as a potential therapeutic target in a variety of cancers. Olaratumab acts by selectively binding PDGFRα, thereby blocking PDGF ligand binding and inhibiting PDGFRα activation and downstream signalling. In October 2016, olaratumab received its first global approval, in the USA, for use in combination with doxorubicin for the treatment of adult patients with soft tissue sarcoma. The approval was granted by the US FDA under its Accelerated Approval Program based on the results of the JGDG phase II trial (NCT01185964). In addition, the EMA granted conditional approval for olaratumab in this indication in November 2016 following a review under the EMA's Accelerated Assessment Program. An international, confirmatory phase III trial in patients with soft tissue sarcoma is ongoing (ANNOUNCE; NCT02451943). Olaratumab has also been investigated in phase II trials in several other cancers. This article summarizes the milestones in the development of olaratumab leading to this first approval, for use in combination with doxorubicin for the treatment of soft tissue sarcoma in adults.

ECCO Essential Requirements for Quality Cancer Care: Soft Tissue Sarcoma in Adults and Bone Sarcoma. A critical review.

ECCO essential requirements for quality cancer care (ERQCC) are checklists and explanations of organisation and actions that are necessary to give high-quality care to patients who have a specific tumour type. They are written by European experts representing all disciplines involved in cancer care. ERQCC papers give oncology teams, patients, policymakers and managers an overview of the elements needed in any healthcare system to provide high quality of care throughout the patient journey. References are made to clinical guidelines and other resources where appropriate, and the focus is on care in Europe. Sarcoma: essential requirements for quality care • Sarcomas - which can be classified into soft tissue and bone sarcomas - are rare, but all rare cancers make up more than 20% of cancers in Europe, and there are substantial inequalities in access to high-quality care. Sarcomas, of which there are many subtypes, comprise a particularly complex and demanding challenge for healthcare systems and providers. This paper presents essential requirements for quality cancer care of soft tissue sarcomas in adults and bone sarcomas. • High-quality care must only be carried out in specialised sarcoma centres (including paediatric cancer centres) which have both a core multidisciplinary team and an extended team of allied professionals, and which are subject to quality and audit procedures. Access to such units is far from universal in all European countries. • It is essential that, to meet European aspirations for high-quality comprehensive cancer control, healthcare organisations implement the requirements in this paper, paying particular attention to multidisciplinarity and patient-centred pathways from diagnosis and follow-up, to treatment, to improve survival and quality of life for patients. Taken together, the information presented in this paper provides a comprehensive description of the essential requirements for establishing a high-quality service for soft tissue sarcomas in adults and bone sarcomas. The ECCO expert group is aware that it is not possible to propose a 'one size fits all' system for all countries, but urges that access to multidisciplinary teams is guaranteed to all patients with sarcoma.

First- and second-line systemic treatments for metastatic and locally advanced soft tissue sarcomas in adults

First- and second-line systemic treatments for metastatic and locally advanced soft tissue sarcomas in adults

Well-Differentiated Liposarcoma of The Larynx: A Case Report and Review of Literature

Summary Liposarcoma is one of the most common soft tissue sarcomas in adults with a relative incidence amongst other sarcomas ranging from 9.8% to 16%. It usually locates in the limbs and retroperitoneum. Primary liposarcomas of the larynx and hypopharynx are rare, comprising less than 20% of all head and neck liposarcomas. According to World Health Organization, these tumors are divided into four histologic types, and well-differentiated liposarcoma is the most common one. It is a tumor of low-grade malignancy that may recur locally, but does not metastasize. We present a case of laryngopharyngeal well- differentiated liposarcoma in an old patient with two previous removals. We also discuss recently published cases with this unusual location of liposarcoma.

Liposarcoma of Hypopharynx and Esophagus: a Unique Entity?

Liposarcoma is the most common soft tissue sarcoma in adults. It represents approximately 20% of all mesenchymal malignancies. It most frequently involves retroperitoneum, trunk, and extremities. Hypopharyngeal and esophageal localization of liposarcoma is extremely rare. We performed a systematic review of literature and reported 26 and 33 cases of hypopharyngeal and esophageal liposarcoma. We analyzed natural history, imaging features, histology, treatment, and prognosis, with a specific focus to similarities and differences between tumors of hypopharynx and esophagus. Hypopharyngeal and esophageal liposarcomas have more similarities than differences. Incidence has a peak at 6th and 7th decades. The diagnostic procedures are barium swallow, endoscopic examination, and CT/MR imaging. Well-differentiated liposarcoma represents the most frequent histological subtype. Surgical excision is the main treatment. Endoscopic resection can be useful for pedunculated tumors of hypopharynx and cervical esophagus. Differences between hypopharyngeal and esophageal liposarcoma are represented by local recurrence rate (greater for hypopharyngeal tumors), number of giant tumors, and time to recurrence (greater for esophageal tumors). Finally, liposarcomas of distal esophagus need more extended approaches. Liposarcomas of hypopharynx and cervical esophagus could be considered a unique pathological entity, with similar features and treatment options. Survival rate is dependent on histological type and location. Local recurrence is common, especially for hypopharyngeal liposarcoma, while the risk of lymph node or distant metastasis is very low. Patients should undergo regular examinations to rule out local recurrence, also for a long time, especially for esophageal tumors.

MLN-8237: A dual inhibitor of aurora A and B in soft tissue sarcomas.

Aurora kinases have become an attractive target in cancer therapy due to their deregulated expression in human tumors. Liposarcoma, a type of soft tissue sarcoma in adults, account for approximately 20% of all adult soft tissue sarcomas. There are no effective chemotherapies for majority of these tumors. Efforts made to define the molecular basis of liposarcomas lead to the finding that besides the amplifications of CDK4 and MDM2, Aurora Kinase A, also was shown to be overexpressed. Based on these as well as mathematic modeling, we have carried out a successful preclinical study using CDK4 and IGF1R inhibitors in liposarcoma. MLN8237 has been shown to be a potent and selective inhibitor of Aurora A. MLN-8237, as per our results, induces a differential inhibition of Aurora A and B in a dose dependent manner. At a low nanomolar dose, cellular effects such as induction of phospho-Histone H3 (Ser10) mimicked as that of the inhibition of Aurora kinase A followed by apoptosis. However, micromolar dose of MLN-8237 induced polyploidy, a hallmark effect of Aurora B inhibition. The dose dependent selectivity of inhibition was further confirmed by using siRNA specific inhibition of Aurora A and B. This was further tested by time lapse microscopy of GFP-H2B labelled cells treated with MLN-8237. LS141 xenograft model at a dose of 30 mg/kg also showed efficient growth suppression by selective inhibition of Aurora Kinase A. Based on our data, a dose that can target only Aurora A will be more beneficial in tumor suppression.

Pedunculated Nodules as a Variant of Dermatofibrosarcoma Protuberans

Dermatofibrosarcoma protuberans (DFSP) is a rare disease of dermal fibroblastic origin that accounts for less than 5% of all soft tissue sarcomas in adults. DFSP grows slowly and is an asymptomatic lesion at the initial diagnosis. Herein, we report a case of multiple pedunculated nodules as a variant of DFSP. A 47-year-old man presented with a 7-month history of multiple well-circumscribed, firm, pedunculated nodules on the inguinal area. Histopathologic examination results showed densely packed uniform spindle cells with a storiform and cartwheel pattern, and positivity for CD34. Wide excision and skin graft were performed and at the 6-month follow-up, there was no evidence of recurrence or metastasis.

Neoadjuvant Chemotherapy and Radiation Therapy for Soft Tissue Sarcoma in Adults: A Single-Institution Retrospective Review

Neoadjuvant Chemotherapy and Radiation Therapy for Soft Tissue Sarcoma in Adults: A Single-Institution Retrospective Review