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Objective: To examine the efficacy and safety of anlotinib as firstline therapy to treat locally advanced or metastatic soft-tissue sarcoma. Methods: This is a single-arm trial. Treatment-naïve patients (≥14 years) with locally advanced or metastatic soft tissue sarcoma were eligible. Each treatment cycle lasted for 3 weeks, and included liposomal doxorubicin (40-50 mg/m2) on day 1 and anlotinib (12 mg) on days 8-21. Starting from the 9th cycle, treatment consisted of only anlotinib. Treatment continued until disease progression or intolerable toxicities. The primary efficacy end point was progression-free survival (PFS). Results: Eight patients were enrolled between July 25, 2019 and January 8, 2020. The median number of treatment cycles was 5.5. Within 5.9 months median follow-up, PFS events occurred in 4 (4/8, 50%) patients. The median PFS was 11.3 months and the 6-month PFS rate was 56%. No patients attained complete response and 2 patients (fibrosarcoma, 1 patient and undifferentiated pleomorphic sarcoma, 1 patient) achieved partial response. Three patients (fibrosarcoma, 2 patients and synovial sarcoma, 1 patient) had stable disease. The objective response rate was 25% (2/8) for the study population, and the disease control rate was 75% (6/8). No new safety concerns emerged. Conclusions: Anlotinib plus liposomal doxorubicin demonstrated antitumor activities in previously untreated locally advanced or metastatic soft tissue sarcomas. Due to the small sample size, further investigations with a larger population should be undertaken to confirm the study findings. [ABSTRACT FROM AUTHOR]

Context: Malignant peripheral nerve sheath tumor (MPNST) is a rare and aggressive soft-tissue sarcoma.Aims: The aim of this study was to analyze various prognostic factors and treatment outcome of patients with MPNST.Settings and Design: This was a retrospective study.Subjects and Methods: Ninety-two patients, who presented with MPNST at a tertiary care cancer center from 2011 to 2018, were included in this study. The median follow-up of all living patients was 33 months. Neurofibromatosis 1 (NF1) was seen in 12 (13%) patients. Sixty (65.2%) patients received curative-intent treatment.Statistical Analysis Used: Kaplan-Meier method was used for survival analysis. Log-rank test was used for univariate analysis, and multivariate analysis was done by Cox proportional hazard ratio method.Results: The 5-year overall survival (OS) of all patients was 47.2% and the 5-year disease-free survival (DFS) of operated patients was 41.5%. On univariate analysis, association with NF1 (P = 0.009), grade (P = 0.017), and margin status (P = 0.002) had a significant effect on DFS, whereas association with NF1 (P = 0.025), metastatic disease on presentation (P < 0.0001), palliative intent of treatment (P < 0.0001), grade (P = 0.049), and margin status (P = 0.036) had a significant effect on OS. On multivariate analysis for patients who were treated with curative-intent treatment, grade (P = 0.015), and margin status (P = 0.028) had a significant effect on DFS, whereas association with NF1 (P = 0.00026) and location of tumor (P = 0.040) had a significant effect on OS.Conclusions: The presence of distant metastasis, palliative intent of treatment, association with NF1, location of the tumor in the head and neck, high tumor grade, and positive margin status were the risk factors associated with poor survival for the patients with MPNST. Wide local excision with negative resection margin is the highly recommended treatment. [ABSTRACT FROM AUTHOR]

Purpose: Although soft tissue constitutes half of the body weight, soft-tissue sarcomas (STSs) are less common than any other types of tumors.Materials and Methods: In this retrospective study, the prognostic factors and clinical courses of 64 patients with extremity STSs treated at our clinic between 1996 and 2012 were investigated.Results: Of the 64 patients included in this study, 35 (55%) were male and 29 (45%) were female. By the end of follow-up, 29 (45%) of the patients remained alive while 35 (55%) deceased. The overall survival (OS) time of the patients was 89.1 months, and their 1-, 3-, 5-, and 10-year survival rates were 82.8%, 69.3%, 51.6%, and 39.4%, respectively. Univariate analysis revealed the following variables as prognostic factors: tumor stage (P < 0.001), surgical method applied (P = 0.009), radiotherapy (RT) application (P = 0.018), RT dose (P < 0.001), and development of metastasis during follow-up (P < 0.001). Multivariate analysis revealed only type of surgery to be a prognostic factor (P = 0.016).Conclusion: Besides surgery, RT plays a crucial role in the multimodal treatment of STSs and increases local control rates and OS. In our study, stage, surgery, and adjuvant RT were found to be effective factors indicating OS. However, more prospective work in this area is necessary. [ABSTRACT FROM AUTHOR]

Aims: Neonatal tumors (NTs) include a group of diverse neoplasms. In this study, we reviewed our data for clinical presentations, management options, and outcome. Materials and Methods: All patients from 0- to 1-month age presenting with solid tumors, from 2006 to 2018 were studied. The gender, presentation, location, type of tumor, and management were analyzed. The final diagnosis was made with histopathology in all cases. Hemangiomas and lymphangiomas were excluded from the study. Results: A total of 32 neonates were studied. The most common tumor was sacrococcygeal teratoma (SCT,16) followed by teratoma at other sites including two cases of fetus-in-fetu, soft-tissue sarcoma (STS, 4), mesenchymal hamartoma (2), hemangioendothelioma (2), and other rare tumors. Three tumors were diagnosed antenatally; of whom, two were neither visible externally nor palpable. Complete surgical excision was done for all except in a case of ovarian cyst where near-total cystectomy was done. No patient received chemotherapy or radiotherapy. Six patients had postoperative complications, including two who had local recurrence requiring excision. There was one mortality. All the other patients are doing well during follow-up. Conclusion: NTs have varied presentations. SCT and STS were the most common benign and malignant tumor, respectively. Early diagnosis and complete surgical excision are often curative for all, regardless of the pathology with the minimal role of chemotherapy or radiotherapy. [ABSTRACT FROM AUTHOR]

Introduction: Primary bone and soft tissue sarcomas are rare, but diagnostically and therapeutically challenging group of tumors, requiring multidisciplinary management. There are limited documented studies from multidisciplinary teams , in the form of comprehensive analysis of these tumors, from our country. This study is an analysis of cases of osteosarcomas, Ewing sarcomas (ESs), chondrosarcomas (CSs), and soft-tissue sarcomas (STSs), registered at our institution during 2012.Methods: Clinical details, including outcomes of cases of bone and STSs, during the year 2012, were retrieved from the medical records of our institution and were further analyzed.Results: Ninety-five high-grade, extremity-based, treatment-naïve cases of osteosarcomas were treated with a novel, dose-dense, nonhigh-dose methotrexate-based OGS-12 protocol. Good histopathologic response (necrosis ≥90%) was achieved in 59% nonmetastatic and 56% metastatic patients. At a median follow-up of 48 months, the estimated 5-year event-free survival and overall survival (OS) were 67% and 78%, respectively. In the metastatic cohort at a median follow-up of 51 months, the 5-year estimated progression-free survival was 24% and OS was 26%. Among 87 (73.2%) nonmetastatic and 32 (26.8%) metastatic, analyzable cases of ES, at a median follow-up of 40 months, the disease-free survival (DFS) and OS in the nonmetastatic group were 62% and 83%; in the metastatic group, they were 37.5% and 65.6%, respectively. Among 40 cases of CSs (33 nonmetastatic and 7 metastatic), 21 had limb salvage surgery while 5 had amputation. Microscopically, 90.4% were Grade II CSs. Five-year OS and DFS were 84.6% and 71%, respectively. Among 189 high-grade, extremity-based STSs (89% nonmetastatic), synovial sarcoma was the most common subtype (31%). Eighty-five percent had limb preservation surgery; a majority were offered adjuvant radiation with or without chemotherapy. At a median follow-up of 51 (1-63) months, 3-year local control, DFS, and OS were 81%, 48%, and 64%, respectively.Conclusions: The novel OGS 12 and Ewing Family of Tumors 2001 protocols have shown comparable outcomes to international standards in cases of osteosarcoma and ES, respectively, and merit wider applications, especially in low- and middle-income countries (LMICs). Outcomes in STS and CSs were also comparable and underscore the importance of a multidisciplinary approach for the management of sarcomas in LMICS. [ABSTRACT FROM AUTHOR]

The Southwest coastal plain of Taiwan is an endemic area of arsenic contamination. Residents who lived there before the 1970s and who used raw groundwater for drinking have a higher risk of arsenic poisoning. In 1968, Tseng et al. described Blackfoot disease as a peripheral vascular disease caused by chronic exposure to arsenic, thereby introducing the concept of arsenic-induced systemic illness in Taiwan. Multiple Bowen's disease (BD) is one of the characteristic consequences of chronic arsenic poisoning and it usually presents as cutaneous carcinoma in situ. Multiple BD can also be associated with squamous cell carcinoma and basal cell carcinoma of the skin, as well as lung, liver, gastrointestinal, and bladder cancers. We encountered a 79-year-old male from Yun-Lin, a county in Southwest Taiwan, who presented with a progressing tumor in his right anterior chest wall. In addition, numerous keratoses and scaly skin lesions were noted on his trunk and extremities, some of which were combined with erosions. The patient was diagnosed with chronic arsenic poisoning with multiple BD and the huge tumor was confirmed as an epithelioid malignant peripheral nerve sheath tumor. [ABSTRACT FROM AUTHOR]

Synovial sarcoma is one of the common soft-tissue tumors of the body and is usually found on the lower extremity, head-and-neck regions. Reports of monophasic variant of synovial sarcoma in the palm are a rare entity. It can present as a challenge for the surgeons mimicking other conditions. Very few cases of this malignancy are reported in the palm. Painful palmar mass is an unusual presentation for this variant of sarcoma. Preoperative suspicion and planned surgical approach pave the way for the proper management. Neoplasm of the hand is very challenging, as it needs surgical resection balancing with resection margin and functionality of the hand. These cases are notorious for late recurrences and metastasis. Long-term follow-up is of utmost importance. We are reporting a case of palmar monophasic synovial sarcoma with its management and follow-up. [ABSTRACT FROM AUTHOR]

OBJECTIVE: The primary aim of intensity-modulated radiation therapy (IMRT) for treating soft tissue sarcoma of an extremity is the reduction of morbidity and maintenance of local control. METHODS: We evaluated the outcomes and toxicity of adjuvant IMRT following function-preserving surgery to treat patients with soft tissue sarcoma and metastasis of the extremities. We retrospectively reviewed prospective databases at Kure Medical Center and Aichi Medical University between 2013 and 2016 and identified 10 patients with lower extremity soft tissue sarcoma who underwent both limb-sparing surgery and postoperative IMRT at one of our institutions. RESULTS: There were 7 men and 3 women (mean age, 67.2 years; range, 48-87 years) included in the study. Of these, four patients were continuously disease-free, two showed no evidence of disease, and four died due to disease. The average functional score was 82% (range, 53-100%). Among the 10 patients, 2 (22%) had grade 1-2 edema and 1 (11%) had grade 2 joint stiffness. Another patient developed grade 2 acute dermatitis. There were no severe complications such as infection, tissue necrosis, fracture, or nerve palsy. CONCLUSION: Although the number of patients in this study was small, our results show that adjuvant IMRT following function-preserving surgery for soft tissue sarcoma of the extremity can be valuable for treating unmanageable tumors. [ABSTRACT FROM AUTHOR]

Synovial sarcomas are aggressive malignant soft-tissue tumors that often involve large joints of the lower extremities. Cricopharynx is a rare site for primary synovial sarcoma with no case reported until date. We report a case of a 45-year-old male presented with progressive dysphagia and hoarseness of voice. On examination by computed tomography revealed a soft-tissue mass in the cricopharyngeal region with involvement of posterior wall of the laryngeal framework. The endoscopic biopsy was suggestive of biphasic synovial sarcoma, which was confirmed by immunohistochemistry. The patient underwent surgery followed by radiotherapy and chemotherapy. Despite multimodality, management patient developed lung metastasis, and survived for 10 months only. There are no available guidelines for treatment of such rare tumors, and each case needs to be reported. [ABSTRACT FROM AUTHOR]

Nonrhabdmyosarcoma soft tissue sarcomas (NRSTS) include a cluster of different types of soft tissue sarcomas clubbed together due to the rarity of individual subtypes. The diagnostic accuracy is lately reinforced due to the availability of immunohistochemical and molecular markers. Surgery is the central modality of treatment since many of them are insensitive to chemotherapy. With the availability of rational risk stratification system, efforts are in progress to evaluate the role of neoadjuvant chemotherapy and radiotherapy to improve outcomes especially for the locally advanced disease. The survival remains dismal for metastatic disease. This review highlights the current status of NRSTS and also describes the experience from a single centre in treatment of NRSTS. [ABSTRACT FROM AUTHOR]

Summary Background Malignant fibrous histiocytoma (MFH) is a common soft-tissue sarcoma in adults, which commonly affects the extremities, trunk, head, and neck. Of these regions, the extremities are more often affected. However, only a few studies have specifically investigated the clinical behavior and prognosis of this malignancy, especially in East Asian populations. Aims and objectives We collected data on MFH cases for a period of 10 years and present our experience here in treating patients with extremity MFH. Materials and methods This study was a retrospective analysis of patients with extremity MFH treated in the Department of Plastic Surgery at National Cheng Kung University Hospital between January 2004 and December 2013. Data on patient profiles, surgical resection, radiotherapy, and chemotherapy were all reviewed. The patients were followed up regarding their local recurrences and survivals. Results Thirteen patients (6 men and 7 women) were enrolled in this study. The mean age of the patients was 57.9 years (range, 33–82 years). The sizes of the clinical tumors ranged from 1.7 to 15 cm in a maximal linear dimension (mean, 7.3 cm), and 76.9% of the tumors developed in the lower extremities. The average follow-up time was 63.5 months. One patient received limb amputation, and 12 patients were treated with limb salvage including marginal or wide excisions. Adjuvant radiotherapy was performed in 10 patients whose tumor sizes exceeded 5 cm or who exhibited inadequate excision or recurrence. Chemotherapy was performed in six patients who exhibited a distant metastasis or vessel invasion. The local and distant recurrence rates were 53.8% and 30.8%, respectively. A total of 42.9% of patients with local recurrence developed distant metastases. The surgical margin was the only significant factor of local disease control, and distant metastasis was the only factor relevant to the overall survival (OS). Conclusion An adequate resection margin during treatment for the primary MFH tumor achieves optimal results for local disease control. It influences the OS rate, because local-recurrence-related distant metastasis is significantly associated with high mortality. [ABSTRACT FROM AUTHOR]

Purpose: The aim of this study was to evaluate the role of positron emission tomography-computed tomography (PET-CT) and magnetic resonance imaging (MRI) in characterization and pre-operative staging of soft-tissue sarcoma (STS) and correlating with operative and histopathological findings. Materials and Methods: Twenty patients (age range 16-72 years [mean 44.4 years]) with resectable and STS were included in this prospective study. Pre-operative MRI was carried out in all patients with acquisition of T1W, T2W, and short tau inversion recovery (STIR) sequences in appropriate planes. Contrast enhanced MRI was performed in four patients. Whole body PET-CT was performed in 13 patients. Demographic data, clinical features, pre-operative imaging analysis, operative, and histopathological findings were analyzed using SPSS software version 11.5. Results: The most common histologic type was malignant fibrous histiocytoma (MFH) (30%). Of 18 STSs 20 were high-grade. Agreement existed between MR and operative size. MRI had 100% negative predictive value (NPV) in predicting neurovascular bundle involvement. However, positive predictive value (PPV) was 33%. MRI had PPV of 20% while PET-CT had 50% PPV in detecting lymph node involvement. Overall staging accuracy of MRI was 75% when correlated with surgical and histopathological findings. Combined PET-CT and MRI staging, in 13 patients, was better (92.31%) when compared with staging with MRI (84.62%). Specific diagnosis on image characteristics was correctly suggested in 35% patients. Conclusions: MRI is the robust modality in local staging of STSs and PET-CT adds greater accuracy to overall staging in combination with MRI. [ABSTRACT FROM AUTHOR]

Brain metastasis in soft-tissue sarcoma is an uncommon event. It usually follows lung metastasis, with a lag period between the occurrences of the two events. We present such a case of soft-tissue sarcoma with lung and brain metastases, with the intention of drawing attention toward this rare occurrence and need of anticipation of brain metastasis in patients who are asymptomatic for the same and have already developed lung metastasis. [ABSTRACT FROM AUTHOR]