Common Malignant Soft Tissue Tumor Research Articles

Pleomorphic Rhabdomyosarcoma of the Uterine Corpus in an Adult Who Underwent Multi-gene Panel Testing.

Rhabdomyosarcoma (RMS) is the most common malignant soft tissue tumor in children. Adult primary RMS of gynecological origin is a rare condition and uterine RMS is an aggressive malignancy with a poor prognosis. The genetic variants associated with uterine RMS in adults have yet to be fully elucidated, and there is no established therapeutic strategy for rare tumors. A 69-year-old Japanese woman was referred to our hospital with abdominal bloating. Imaging examination revealed a tumor with diameter of 85 mm located in the uterus and multiple regional lymph node metastases. Biopsy of the uterine corpus indicated possible uterine carcinosarcoma or RMS. Following debulking surgery, the patient was diagnosed with stage IVB pleomorphic RMS. The patient was treated with two courses of doxorubicin every three weeks and one course of combination chemotherapy with vincristine, actinomycin, and cyclophosphamide. Because of rapid progression of the disease, we decided to perform multi-gene panel testing to determine the most effective therapeutic strategy. However, no therapeutic plan based on genetic information was identified. The patient with chemotherapy-refractory RMS died 11 weeks after surgery. Our patient had advanced uterine RMS with an unresectable tumor that was resistant to chemotherapy, resulting in poor outcomes. Despite conducting multi-gene panel testing, no tailored therapeutic approach based on genetic information was found. This case highlights the challenges in managing uterine RMS in adults and underscores the urgent need for further research to identify effective treatment modalities.

Embryonal rhabdomyosarcoma of hard palate: a case report and review of literature

Rhabdomyosarcomas are the most common malignant soft tissue tumors in children and adolescents. Around 40% of cases are diagnosed in head and neck region with orbit and face being the commonly involved areas. Oral lesions account for less than 10%. We report a case of embryonal RMS in a 4-year-old boy who presented with a polypoid mass in the hard palate. Though majority of the oral lesions encountered in children are benign, a small but significant percentage may be representative of an aggressive malignant tumor. Differentiating benign and malignant oral lesions is mandatory for optimal diagnostic strategies.

Neonatal Rhabdomyosarcoma: About a new observation

Rhabdomyosarcoma is a cancer that develops from so-called "soft" tissue. In neonates, as well as in older children, rhabdomyosarcoma is the most common malignant soft tissue tumor. Malignant mesenchymal tumors are, after neuroblastoma, the second most common type of cancer in the newborn. Our case is a female neonate, 2nd of 2 siblings, full term, eutrophic, admitted at H4 of life with respiratory distress rated 2/10 according to the silverman score with left choanal atresia. The oral examination revealed several solid pinkish masses and the cervico-facial CT scan showed an oval retro pharyngeal collection associated with infiltration of the superficial and deep left neck and upper mediastinum spaces. The histopathological study found a morphological and immunohistochemical aspect of an embryonal rhabdomyosarcoma. Rhabdomyosarcoma is rare in the neonatal period, it is located mainly in the limbs, genitourinary tract and head and neck region. As was the case with our newborn who presented with an oropharyngeal location. Their diagnosis can be difficult and requires the advice of an expert anatomopathologist. Their management must be multidisciplinary.

Does the apparent diffusion coefficient from diffusion-weighted MRI imaging aid in the characterization of malignant soft tissue tumors and sarcomas.

To identify trends in apparent diffusion coefficient (ADC) measurements from diffusion-weighted imaging (DWI) with respect to tumor type classification and other tumor characteristics whether common malignant soft tissue tumors can be distinguished. A consecutive series of extremity malignant soft tissue tumors and soft tissue sarcomas (STS) among 78 adult patients with conventional MRI and DWI were included. Each case was evaluated with respect to T1/T2 signal alterations and heterogeneity, presence of peritumoral edema, necrosis, cystic changes, internal hemorrhage, and maximum longitudinal dimension blinded to the histology. The ADC mean and minimum were obtained using a free-hand region of interest of the whole tumor and the darkest (lowest signal area) ADC area of the tumor. Kruskal-Wallis and Wilcoxon Rank-Sum Tests were used to determine associations and significance between tumor subtypes. Intraclass correlation (ICC) and kappa calculations were utilized to assess inter-reader agreements for ADC values and reader diagnosis. Liposarcomas showed more heterogenous T1W images with hyperintense T1W signal when compared to tumors not classified as liposarcoma (P = 0.046 and P = 0.010, respectively). Liposarcomas were relatively consistent in demonstrating an absence of hemorrhage (81.8%) while undifferentiated pleomorphic sarcomas consistently showed intralesional hemorrhage (90%). When comparing individual tumor classifications against the rest of the samples, lymphomas registered lower mean and minimum ADC values in the whole tumor and in themost hypointense area of the tumor for both readers (P < 0.05). The interobserver agreement between the two readers was good to excellent for all four ADC measurements (ICC = 0.65-0.98). Diffusion-weighted imaging generated ADC measurements are reproducible but currently offer limited insight in being able to differentiate among different malignant soft tissue tumor and sarcoma histologies.T1W and T2W signal characteristics also offer limited insight indifferentiating between soft tissue malignancies.

Наиболее распространенные диагнозы в ортопедической онкологии: регион Юго-Восточной Анатолии, Турция

The purpose of this study: Musculoskeletal tumors are very rare, and their distribution varies regionally. We aim to share the musculoskeletal system tumors and tumor-like lesions that orthopedic surgeons in the region commonly encounter in their daily practice.Methods. In this retrospective study, 339 patients diagnosed with tumor and tumor-like lesions in the musculoskeletal system who consulted a single orthopedic surgeon between 2022 and 2023 in a tertiary hospital in the Southeast of the country were included. Age, gender, size, type of lesions, tumor diagnoses, and anatomical sites were examined.Results. Bone localization (62.8%) is more common than soft tissue. Benign lesions are most commonly seen in both bone and soft tissue. Osteochondroma (21.7%) and chondrosarcoma (54%) are the most common benign and malignant bone tumors, while osteomyelitis (47.6%) is the most common tumor-like lesion. Hemangioma (27%) and peripheral nerve sheath tumor (62.5%) are the most common benign and malignant soft tissue tumors, while ganglion cyst (63.8%) is the most common tumor-like lesion. Benign bone tumors are larger in males than females (p:0.008). Lower extremity located of musculoskeletal tumors and tumor-like lesions is more common than upper extremity.Conclusions. In the Southeastern Anatolia region, benign bone and soft tissue tumors (osteochondroma and hemangioma) are more likely. The most common malignant tumors are chondrosarcoma and peripheral nerve sheath tumors. Additionally, it should not be forgotten that musculoskeletal system tumors are often located in the lower extremities.Keywords: Benign tumors, bone tumors, malign tumors, musculoskeletal tumors, soft tissue tumors, tumor-like lesions.Corresponding author: Özlem Orhan, Assistant professor of the Department of Orthopedics and Traumatology, Medical Faculty, Harran University, Sanlıurfa, Turkey. Postal code: 63300Address: Turkey, Sanlıurfa, Osmanbey campusPhone: +905057475401Email: droorhan@gmail.comJ Trauma Ortho Kaz 2023; 3 (69): 12-18Recieved: 28-07-2023Accepted: 11-09-2023 This work is licensed under a Creative Commons Attribution 4.0 International License

S3039 Hepatic Undifferentiated Pleomorphic Sarcoma Mimicking Liver Abscess

Introduction: Undifferentiated pleomorphic sarcoma (UPS) has been recognized as one of the most common malignant soft tissue tumors. It usually affects the elderly population and involves the extremities. However, hepatic UPS is rare. Fewer than 200 cases have been reported. Primary hepatic UPS occurs in late adulthood. It typically presents with a solid hepatic mass with regional lymph node involvement. Occasionally, UPS mimics hepatic infection clinically. Here we report a case of hepatic UPS, mimicking the presentations of liver abscess. Case Description/Methods: A 44-year-old female with a past medical history of hypertension presented at the emergency department (ED) for right upper abdominal pain for one week. The pain was described as dull, 5/10 on a pain scale, non-radiating, and associated with nausea, fever, and chills. The pain was worsened by sitting up and alleviated by lying down. At ED, the patient’s vital signs were within normal range. Physical examination showed right upper quadrant tenderness, without rebound, guarding, or rigidity. She was found to have slightly elevated liver enzymes and alkaline phosphate. CT of the abdomen revealed an irregular hypodense mass at the right hepatic lobe measuring 14.3 x 11.6 cm and a 2.6cm enlarged porta hepatis lymph node. The patient was admitted for further evaluation. Concerning possible liver abscess, the blood culture was collected, and the patient was treated with empiric antibiotics. Tumor markers including AFP, and CEA were within normal range. The blood culture was negative for infection. To rule out malignancy, PET scan was performed and showed a hypermetabolic mass with necrotic components in the liver. The patient underwent a liver biopsy. The pathology revealed extensively necrotic pleomorphic round cells with eosinophilic cytoplasm and variably sized, oval nuclei, indicating hepatic undifferentiated pleomorphic sarcoma. Oncology was consulted and recommended doxorubicin and ifosfamide for neoadjuvant chemotherapy. The patient was discharged to home to follow up with oncology as outpatient for chemotherapy and surgery for tumor resection. (Figure) Discussion: Hepatic UPS is a rare malignant mesenchymal tumor. Nonspecific clinical symptoms, unmarkable laboratory findings, and rapid tumor growth lead to poor prognosis. Physicians should be aware of this rare disease as a differential diagnosis for liver mass. Surgical resection is the preferred treatment. Chemotherapy could be used as neoadjuvant or adjuvant treatment to improve the survival rate.Figure 1.: a large liver mass noticed on CT of abdomen with contrast.

Deep Learning of Rhabdomyosarcoma Pathology Images for Classification and Survival Outcome Prediction

Rhabdomyosarcoma (RMS), the most common malignant soft tissue tumor in children, has several histologic subtypes that influence treatment and predict patient outcomes. Assistance with histologic classification for pathologists as well as discovery of optimized predictive biomarkers is needed. A convolutional neural network for RMS histology subtype classification was developed using digitized pathology images from 80 patients collected at time of diagnosis. A subsequent embryonal rhabdomyosarcoma (eRMS) prognostic model was also developed in a cohort of 60 eRMS patients. The RMS classification model reached a performance of an area under the receiver operating curve of 0.94 for alveolar rhabdomyosarcoma and an area under the receiver operating curve of 0.92 for eRMS at slide level in the test data set (n=192). The eRMS prognosis model separated the patients into predicted high- and low-risk groups with significantly different event-free survival outcome (likelihood ratio test; P =0.02) in the test data set (n=136). The predicted risk group is significantly associated with patient event-free survival outcome after adjusting for patient age and sex (predicted high- versus low-risk group hazard ratio,4.64; 95% CI,1.05-20.57; P =0.04). This is the first comprehensive study to develop computational algorithms for subtype classification and prognosis prediction for RMS histopathology images. Such models can aid pathology evaluation and provide additional parameters for risk stratification.

Primary Dedifferentiated Liposarcoma of the Colon - Report of a Rare Case with Review of Literature

Liposarcoma is the most common malignant soft tissue tumor in adults. Extremities and retroperitoneum are the common sites of involvement of liposarcoma. Liposarcoma of the gastrointestinal tract is rare and the colon is an extremely uncommon site. Mesenchymal tumors of the bowel are mainly represented by gastrointestinal stromal tumors (GIST). A sixty-one-year-old male patient presented with abdominal pain and distension. Contrast-enhanced computerized tomography (CECT) showed a circumferential

Synovial Sarcoma in Children, Adolescents, and Young Adults: A Report From the Children's Oncology Group ARST0332 Study.

Synovial sarcoma (SS) is the second most common malignant soft tissue tumor in children. ARST0332 evaluated a risk-based treatment strategy for young patients with soft tissue sarcoma designed to limit therapy for low-risk (LR) disease and to test neoadjuvant chemoradiotherapy for unresected higher-risk disease. Newly diagnosed patients with SS age < 30 years were assigned to four treatment arms based on disease features: A (surgery only), B (55.8 Gy radiotherapy [RT]), C (ifosfamide and doxorubicin [ID] chemotherapy plus 55.8 Gy RT), and D (neoadjuvant ID and 45 Gy RT, then surgery and RT boost based on margins followed by adjuvant ID). Patients treated in Arms A and B were considered LR, arms C and D without metastases as intermediate-risk (IR), and those with metastases as high-risk (HR). Of the 146 patients with SS enrolled, 138 were eligible and evaluable: LR (46), IR (71), and HR (21). Tumors were 80% extremity, 70% > 5 cm, 70% high-grade, 62% invasive, 95% deep, and 15% metastatic. Treatment was on arm A (29.7%), B (3.6%), C (16.7%), and D (50%). There were no toxic deaths and four unexpected grade 4 adverse events. By risk group, at a median follow-up of 6.8 years, estimated 5-year event-free survival was LR 82%, IR 70%, and HR 8%, and overall survival was LR 98%, IR 89%, and HR 13%. After accounting for the features that defined risk category, none of the other patient or disease characteristics (age, sex, tumor site, tumor invasiveness, and depth) improved the risk stratification model. The risk-based treatment strategy used in ARST0332 produced favorable outcomes in patients with nonmetastatic SS relative to historical controls despite using RT less frequently and at lower doses. The outcome for metastatic SS remains unsatisfactory and new therapies are urgently needed.

Neoplastic Elbow Diseases and Mimickers.

Tumors around the elbow are infrequent, and delayed diagnosis is a common theme because of the low incidence and lack of familiarity. However, just like any other site, the radiologic work-up of musculoskeletal tumors around the elbow remains the same, with plain films the first investigation in a patient with a suspected bone tumor and ultrasound the first modality to evaluate a soft tissue lump. The management of both bone and soft tissue tumors around the elbow is unique because of a large number of important structures in an anatomically confined space and little normal tissue to spare without severely compromising the joint's function. Many benign nonneoplastic entities can mimic bone and soft tissue tumors on imaging. It is important to keep the characteristic imaging appearance in mind while formulating a differential diagnosis to avoid an unnecessary additional work-up. This article reviews the most common benign and malignant bone and soft tissue tumors around the elbow, mimickers, imaging features, and current therapeutic concepts.

The role and efficacy of multidisciplinary council in the bone and soft tissue tumor patients

Objectives: Bone and soft tissue tumors have become more common and recognized diseases with developing medical technologies. The life span of the patients has been prolonged with more effective treatment methods and developing technology. Regardless of their area of expertise, the concept of multidisciplinary tumor approach has emerged in recent years, since it is difficult for a single physician to manage a malignant mass treatment process. For this purpose, we aimed to share our multidisciplinary bone and soft tissue tumor council data results. Methods: Patients who were evaluated at the Department of Orthopedics and Traumatology of Ondokuz Mayis University between January 2004 and June 2017 were evaluated retrospectively. For this study, the weekly archived tumor council forms were evaluated and the data were transferred to the computer via Microsoft Excel and SPSS programs. The database for the specified years was created and the results were evaluated and the database was evaluated. Results: A total of 2788 patients were included in the study. After the patients with data deficiency were removed, 2397 patients were the subject of the study. In the evaluation of the first 1960 patients, 658 primary bone tumors, 577 primary soft tissue tumors, 356 cases of metastases and 374 non-tumoral cases were detected. The most common benign bone tumor was enchondroma, while the most common malignant bone tumor was osteosarcoma. The most common benign soft tissue tumor was lipoma, whereas the most common malignant soft tissue tumor was malignant mesenchymal tumor. The most common non-tumor cause was chronic infection. The diagnosis of 203 patients was different from the definitive diagnosis. Conclusions: In this study, we determined the epidemiological distribution of the cases evaluated in the multidisciplinary tumor council in the Middle Black Sea and the role of multidisciplinaryapproach in treatment and survival has a positive effect especially in selected patient groups.

Childhood soft tissue tumors, one center experience

Aim: Studies on childhood soft tissue tumors have generally been conducted with malignant tumors. There are few studies in the literature comprising all soft tissue tumors. The current study aimed to examine the incidence of all benign and malignant soft tissue tumors during childhood in East Mediterranean region.Materials and Methods: This study retrospectively analyzed the data gathered from patients diagnosed with soft tissue tumors according to the WHO 2020 classification. It focused on those aged 0–18 years that underwent excisional biopsy at Adana City Training and Research Hospital (Adana, Turkey) between 2014 and 2020.Results: Of the total patients, 47.5% were female and 52.5% male. Of these, 87.2% of the cases were benign, 9.2% malignant, and 3.6% intermediate. Hemangioma (55%) was the most common benign soft tissue tumor, and the most common location was the head–neck. Other benign soft tissue tumors were neurofibroma (8.8%), lipoma (6.1%), schwannoma (5.1%), and neuroma (4.4%). Rhabdomyosarcoma (53.2%) was the most common malignant soft tissue tumor. The embryonal subtype was the most common (36.6%). Other malignant soft tissue tumors were extra-osseous PNET/Ewing’s sarcoma (16.6%), synovial sarcoma (13.3%), and leiomyosarcoma (3.3%).Conclusion: Similar to the literature, in this study, the most common benign soft tissue tumor in children was hemangioma, and the most common malignant soft tissue tumor was rhabdomyosarcoma. It is hoped that this study will contribute to the literature as it is the first study to cover all childhood soft tissue tumors at national and regional scale.

Battle Against Musculoskeletal Tumors: Descriptive Data of Military Hospital Experience.

Background: Management of musculoskeletal tumors remains challenging for orthopedic surgeons. The aim of this cross-sectional study was to present the prevalence and localization of musculoskeletal disorders diagnosed and treated at a tertiary referral military hospital.Method: A total of 552 patients' medical records who presented to our clinic between 2009 and 2014 with the diagnosis of musculoskeletal tumors were retrospectively analyzed according to age, gender, bone/soft tissue localization, histopathological diagnosis, incidence, and treatment.Results: Of the cases diagnosed with tumor, 225 were soft tissue localized, 317 bone localized, and 10 tumor-like lesions. The most common primary benign soft tissue tumors were lipoma, ganglion, and giant cell tumor of tendon sheath, while the most common malignant soft tissue tumors were liposarcoma and synovial sarcoma, respectively. The most common primary bone tumors were osteochondroma, enchondroma, and simple bone cyst, while the most common malignant bone tumors were osteosarcoma, Ewing sarcoma and chondrosarcoma, respectively. Myositis ossificans was found as the most common tumor-like lesion.Conclusion: Descriptive data in musculoskeletal tumors is crucial in terms of improving treatment and reducing mortality. In this study, no significant difference was found between the data of tertiary military hospital regarding epidemiology of musculoskeletal system tumors and those from the literatures around Turkey.

Sonographic Appearances of Liposarcoma: Correlations with Pathologic Subtypes

Liposarcoma is the second most common malignant soft-tissue tumor. This entity is pathologically categorized into 4 subtypes: well-differentiated, myxoid, dedifferentiated and pleomorphic. Although features on magnetic resonance imaging and computed tomography for these 4 subtypes have been reported quite precisely, those on sonography have not been fully investigated. The present study was therefore aimed at clarifying the sonographic appearances of each liposarcoma subtype and assessing correlations with histopathology. The study population was made up of 35 cases, including 21 cases of well-differentiated liposarcoma, 6 cases of myxoid liposarcoma, 6 cases of dedifferentiated liposarcoma and 2 cases of pleomorphic liposarcoma. Compared with the other subtypes, well-differentiated liposarcoma was characterized by the high frequency of the following findings: isoechogenicity, tiny hyperechoic lines and hypovascularity (p < 0.01, in each). Myxoid liposarcomas were characterized by low echogenicity, intermingled with anechoic areas and moderate vascularity (p < 0.01, in each). Dedifferentiated liposarcomas showed a specific biphasic pattern of hyperechoic and hypoechoic areas and hypervascularity (p < 0.01, in each). Pleomorphic liposarcomas showed a specific gyrus-like mixture of hyperechoic and hypoechoic areas (p < 0.01). In conclusion, the present study revealed different characteristics of sonographic appearance among the 4 histopathologic subtypes of liposarcoma.

Https://www.pacificejournals.com/journal/index.php/apalm/article/view/2293

Background: Soft tissue tumors are a highly heterogeneous group of tumors that are classified by the line of differentiation, according to the adult tissue they resemble. Objective of this study was to assess the utility of fine needle aspiration cytology (FNAC) in diagnosing soft tissue tumors and to do the histo-pathological correlation wherever possible. Methods: Conventional May-Grunwald Giemsa (MGG) staining was done in all the 100 cases and cytodiagnosis was rendered. Histopathology was available in 41 cases. The cytological diagnosis was correlated with histology in these cases. The accuracy rate, sensitivity, specificity, the positive and negative predictive value were calculated. P value was found by applying Fisher’s exact test. Results: The study was carried out on aspirates of 100 patients. Out of which 65 cases were benign, 30 were malignant while 5 cases were inadequate. Lipoma was the most common cytologically diagnosed benign lesion. Dermatofibrosarcoma protuberans (DFSP) and synovial sarcoma were found to be the most common malignant soft tissue tumors. Cyto-histological correlation could be achieved in 41 cases, with an accuracy of 92.7%. Conclusion: FNAC is a well-tolerated and cost-effective procedure. It provides predictive diagnosis of benign or malignant soft tissue tumors and also specific tumor type, especially after correlation with clinical and radiological findings.

Toosendanin induces the apoptosis of human Ewing's sarcoma cells via the mitochondrial apoptotic pathway.

Toosendanin, a triterpenoid extracted from the root bark of Melia toosendan, has its origin from traditional Chinese medicine and has been used as a non-polluting and pesticide-free plant insecticide in China for fruit and vegetable production. In recent years, toosendanin has been found to inhibit tumor cell proliferation and promote tumor cell apoptosis. Ewing's sarcoma (ES) is the second most common primary malignant bone and soft tissue tumor in children and adolescents. Although the overall prognosis of ES has improved, the 5-year survival rate has not significantly increased. To analyze the role of toosendanin on ES progression, CCK-8 viability assay, flow cytometry, Hoechst 33258 staining and western blotting were performed. The present results suggested that toosendanin suppressed cell viability and induced apoptosis in human SK-ES-1 cells compared with DMSO treatment. In addition, in the present study, toosendanin was found to upregulate the expression of Bax and downregulate the expression of Bcl-2, altering the Bax/Bcl-2 ratio. Additionally, toosendanin promoted the release of cytochrome c, resulting in the activation of the mitochondrial apoptotic pathway, thus inducing the activation of caspase-9 and caspase-3, and the cleavage of PARP. Our results demonstrated that toosendanin inhibited the growth of ES cells in a dose-dependent manner and triggered mitochondrial apoptotic pathway to induce apoptosis. Therefore, toosendanin can potentially be utilized as an anticancer botanical drug for the treatment of ES.

Distribution and evaluation of bone and soft tissue tumors operated in a tertiary care center

ObjectiveThe aim of this study was to retrospectively evaluate the patients who were operated in our orthopedics and traumatology clinic with the suspection of bone and soft tissue tumors. MethodsA total of 3133 patients (1146 (46.5%) female and 1318 (53.5%) male) who presented to our tertiary clinic from different regions of Turkey between January 2002 and July 2013 with the presumed diagnosis of bone and soft tissue tumors were analyzed according to age, gender, bone/soft tissue localization, tumoral localization, histopathological diagnosis, tumor size and incidence. ResultsOf all operated patients, 2464 (78%) were diagnosed with tumor, while non-tumoral causes were found in 669 (22%) patients. Of the cases diagnosed with tumor, 1139 were bone localized, 1004 soft tissue localized, and 321 metastasis. The most common benign bone tumors were osteochondroma (130, 20%), enchondroma (96, 15%), and simple bone cysts (90, 14%), while the most common malignant bone tumors were osteosarcoma (241, 44%), ewing's sarcoma (89, 16%), and chondrosarcoma (77, 14%); respectively. The most common benign soft tissue tumors were lipoma (141, 22%), giant cell tumors (108, 16%) and ganglion (107, 16%), while the most common malignant soft tissue tumors were liposarcoma (55, 16%), synovial sarcoma (53, 16%) and malignant mesenchymal tumors (45, 13%); respectively. ConclusionMusculoskeletal tumors are rare, but descriptive data in any region are important in order to reduce mortality and improve treatment. No significant difference was found between the data of our hospital regarding epidemiology of the musculoskeletal system tumors and those from the other regions around the world. Level of evidenceLevel IV, Therapeutic study.

Analysis of clinicopathological characteristics and prognostic factors of foot and ankle soft tissue and bone tumors

Objective: To evaluate the clinicopathological characteristics of foot and ankle soft tissue and bone tumor, and to analyze the prognosis and the related factors of malignant tumors in this site. Methods: 74 patients with soft tissue and bone tumors of foot and ankle from January 2006 to February 2017 were retrospectively analyzed. The clinicopathological characteristics, the treatment and survival status of malignant tumors were followed up, and the clinical and therapeutic factors related to prognosis were analyzed. Results: Of the 74 patients, 34 were males and 40 were females. The male to female ratio was 1∶1.18; the age ranged from 12 to 64 years and the median age was 42 years. Tumors located in forefoot of 22 cases, 22 in midfoot, 10 in hind foot, 14 in ankle joint and 6 in multiple sites. 14 cases were bone tumors, including 7 benign and 7 malignant, and 60 cases were soft tissue tumors, including 14 benign and 46 malignant. The most common malignant soft tissue tumors were synovial sarcomas (13 cases), and the most common benign soft tissue tumors were hemangiomas (4 cases). 44 cases of malignant tumors underwent surgery were followed up, of which were 7 bone and 37 soft tissue malignant tumors. Limb salvage surgeries were performed in 33 cases and amputation in 11 cases. The median follow-up time was 69.8 months, and the median survival time was 40.7 months. The 1-year, 3-year and 5-year survival rate of soft tissue malignant tumors was 88.0%, 73.0%, and 63.0%, respectively. The 1-year, 3-year and 5-year survival rate of bone malignant tumors was 86.0%, 57.0% and 57.0%, respectively. Univariate analysis showed that the prognostic factors affecting 5-year survival rate were tumor size and adjuvant therapy (P<0.05). Patient's gender, age, tumor location, histological type and surgical procedure had no effect on overall survival(P>0.05). Multivariate analysis showed that tumor size was an independent prognostic factor (RR=7.262, P=0.005). Conclusions: Forefoot and midfoot are more common in foot and ankle soft tissue and bone tumors. Synovial sarcoma is the most common diagnosis in malignant soft tissue tumors, and hemangioma is the most common diagnosis in benign soft tissue tumors. The prognostic factor of malignant soft tissue and bone tumors in foot and ankle is tumor size. Patients with the tumor size of 5 cm or more have a worse prognosis.

Myxoid Liposarcoma: When the Diagnosis is Not Obvious: Case Report and Literature Review

Sarcomas are a rare and heterogeneous group of malignant tumors of mesenchymal origin that comprise less than 1% of all adult malignancies and 12% of pediatric cancers. Liposarcoma is defined as a malignant mesenchymal neoplasm that is composed of lipogenic tissue, and is a common malignant soft tissue tumor, accounting for 10% to 16% of all sarcomas. This case is about a 47-year-old male, who visited his family doctor with complaints of asymmetrically enlarged left thigh mass with a week of evolution, associated with light left thigh pain that appeared 1 month before presentation. Physical examination showed a difference of 11.5 centimeters (cm) in the measurement between both thighs. An ultrasound was requested and showed a massive neoformation on the posterior aspect of the left thigh, compatible with liposarcoma. The patient was referred to the General Surgery Service where he performed magnetic resonance imaging (MRI), which confirmed the presence of bulky neoformation, compatible with undifferentiated liposarcoma. As part of the protocol, the patient was then referred to the Portuguese Oncology Institute in Oporto, where he performed genetic tests and therapeutic guidance. Soft tissue sarcomas are a heterogeneous group of tumors with a large spread in biological behavior, prognosis, and requested treatment modalities. Myxoid liposarcoma show a good prognosis in most cases. However, it is biologically different from other liposarcomas, with the presence of the t (12;16) translocation, high radio and chemosensitivity and a high prevalence of extrapulmonary metastases.

Soft tissue tumors : Epidemiology, classification and staging

Benign, intermediate and malignant soft tissue tumors can be differentiated histologically. Furthermore, the tumors can be subdivided according to their linear differentiation. In the new World Health Organization (WHO) classification of soft tissue tumors from 2013 changes have been made relating to the allocation of known entities, e. g. undifferentiated sarcomas have been formed into anew subgroup and are no longer assigned to the fibrohistiocytic tumors. The term malignant fibrous histiocytoma has been replaced by the undifferentiated sarcoma. Furthermore, two new subgroups were incorporated, the nerve sheath tumors and gastrointestinal stromal tumors. These were previously included in the tumor classification of other organ systems. These changes in the new classification are related to the rapid increase in knowledge of the genetics and the cell biology of soft tissue tumors. Malignant soft tissue tumors only represent 1% of all malignant tumors in adults. The largest subgroup of soft tissue tumors in adults is the adipocytic tumors. The liposarcoma, which belongs to this subgroup is one of the most common malignant soft tissue tumors in adults. In childhood malignant soft tissue tumors represent 15% of malignant tumors and rhabdomyosarcoma is the most common malignant soft tissue tumor.