High Local Recurrence Rate Research Articles

Is Programmed Death Ligand 1(PD-L1) Expression in Vulvar Cancer Prognostic for Locoregional Control?

Vulvar cancer is a rare female genital neoplasm in which surgery and radiotherapy play an integral role in the treatment paradigm; however, locoregional recurrence remains the predominant pattern of failure. Little is known about the impact of PD-L1 status in vulvar cancer and its value for clinical outcomes and response prediction to immunotherapy. We sought to explore clinical outcomes of patients with positive PD-L1 expression in vulvar cancer. Single-institution retrospective analysis of patients with surgically resectable invasive vulvar carcinoma from 2001-2021 was performed. Patients with locally advanced disease not amendable to upfront surgery or de novo metastatic disease were excluded. Immunohistochemical PD-L1 expression was assessed using the Combined Positive Score (CPS) with positive expression defined as ≥1, and Tumor Proportion Score (TPS) with positive expression defined as ≥1%. Survival and disease control outcomes were calculated using the Kaplan-Meier Method with log-rank t-test. Multivariable analysis was conducted using a parsimonious cox regression analysis using forward conditional selection. A total of 85 patients were identified with a median age of 69 years old (IQR: 59-78), 54% (n = 46) FIGO stage I-II, 97% (n = 82) squamous cell carcinoma histology, 41% (n = 35) p16 positive status, 74% (n = 63) without a history of lichen sclerosis, 40% (n = 34) without co-existing vulvar intraepithelial neoplasm (VIN), and 49% (n = 42) treated with surgery alone. There were 72% (n = 61) with positive PD-L1 TPS (≥1%), and 81% (n = 69) with positive PD-L1 CPS (≥1) expression. The median follow up was 49 months (IQR: 21-75 months). The 5-year OS was 79% (95% CI, 70%-89%), DFS 55% (95% CI, 43%-67%), local control (LC) 59% (95% CI, 47%-72%), regional control (RC) 86% (95% CI, 78%-94%), and distant metastasis (DM) 96% (95% CI, 92%-100%). PD-L1 expression was associated with lower LC and DFS by TPS ≥1%. The 5-year LC of 82% (95% CI, 65%-98%) for PD-L1 negative versus 50% (95% CI, 34%-65%) positive disease (p = 0.03). The 5-year DFS was 77% (95% CI, 59%-95%) for PD-L1 negative versus 46% (95% CI, 31%-61%) positive disease (p = 0.03). No significant DFS or LC difference was noted by CPS levels ≥1. No significant difference was observed for RC, DM, or OS. On multivariable analysis, PD-L1 TPS remained a significant predictor for LC (HR = 3.01, 95% CI, 1.07-8.95, p = 0.04). No significant difference in DFS was observed for PD-L1 TPS on multivariable analysis. PD-L1 expression is associated with higher rates of local recurrence and may represents a potentially important actionable target independent of p16 status to improve the predominant pattern of relapse in this uncommon malignancy.

Spiradenoma With Adenoid Cystic Carcinoma-like Changes: A Case Series of This Rare Variant With a Potential Diagnostic Pitfall.

Spiradenomas are benign cutaneous adnexal neoplasms with sweat gland differentiation that can manifest a broad spectrum of histomorphologic appearances. While they show a characteristic histopathologic phenotype and clinical management involves surgical excision with a low risk of recurrence, there have been unusual histopathologic variants of spiradenoma reported, including cases with adenoid cystic carcinoma (ACC)-like changes. Primary cutaneous ACC is a low-grade malignancy presenting as a subcutaneous mass with the potential for local invasion, perineural invasion, and high rates of local recurrence after excision. The diagnosis of spiradenomas with ACC-like features can be challenging, especially when only the ACC-like component is present for evaluation. A retrospective analysis of 21 cases of spiradenoma with ACC-like changes were obtained from large academic institutions, was performed, and summarized below. All cases showed background of conventional spiradenoma, and the ACC-like areas represented a component in all lesions. The percentage of ACC-like component ranged from 5% to 40% in all cases. The ACC-like component was multifocal and without pleomorphism, perineural and/or vascular invasion, necrosis, or increased mitotic activity. MYB translocation and protein expression was studied in 16 cases by fluorescence in situ hybridization, polymerase chain reaction, and immunohistochemistry. All studied cases were negative for MYB / NFIB , MYB L1, and MYB F by fluorescence in situ hybridization and polymerase chain reaction and 3 cases were positive for MYB expression by immunohistochemistry. Our study expands on spiradenomas with ACC-like features that ought to be considered in the differential diagnosis of cutaneous neoplasms such as primary cutaneous ACC. Our results indicate that a thorough histopathologic inspection and strict application of well-defined histologic criteria are necessary to support the diagnosis of this unusual histopathologic variant. These tumors can be difficult to diagnose, and awareness of their histomorphologic spectrum will facilitate definitive diagnosis and avoid misdiagnosis with other conditions.

Angiosarcoma in the breast: a population-based cohort from Sweden.

Breast angiosarcoma is a rare disease mostly observed in breast cancer (BC) patients who have previously received radiotherapy (RT). Little is known about angiosarcoma aetiology, management, and outcome. The study aim was to estimate risk and to characterize breast angiosarcoma in a Swedish population-based cohort. The Swedish Cancer Registry was searched for breast angiosarcoma between 1992 and 2018 in three Swedish healthcare regions (population 5.5 million). Information on previous BC, RT, management, and outcome were retrieved from medical records. Overall, 49 angiosarcomas located in the breast, chest wall, or axilla were identified, 8 primary and 41 secondary to BC treatment. Median age was 51 and 73 years, respectively. The minimum latency period of secondary angiosarcoma after a BC diagnosis was 4 years (range 4-21 years). The cumulative incidence of angiosarcoma after breast RT increased continuously, reaching 1.4‰ after 20 years. Among 44 women with angiosarcoma treated by surgery, 29 developed subsequent local recurrence. Median recurrence-free survival was 3.4 and 1.8 years for primary and secondary angiosarcoma, respectively. The 5-year overall survival probability for the whole cohort was 50 per cent (95 per cent c.i., 21 per cent-100 per cent) for primary breast angiosarcoma and 35 per cent (95 per cent c.i., 23 per cent-54 per cent) for secondary angiosarcoma. Breast angiosarcoma is a rare disease strongly associated with a history of previous BC RT. Overall survival is poor with high rates of local recurrences and distant metastasis.

Orbital Liposarcoma: A Surveillance, Epidemiology and End Results Database Study.

Orbital liposarcoma is a challenging tumor to treat due to its rarity and high rate of local recurrence, and the role of radiotherapy and chemotherapy remain unclear. Analysis of big data may improve our overall understanding of orbital disease and role of adjuvant therapies. Data were extracted from the Surveillance, Epidemiology and End Results (SEER) Research Plus database from 1975 to 2017. All patients with a diagnosis of liposarcoma (ICD-O3 codes 8850-8858, 8869-8862, 8870, 8880, 8881) were included. Cases were divided into 4 groups by primary site: orbit, retroperitoneum, soft tissue, and other. A total of 16,958 patients were included. Patients with orbital involvement were younger and more likely to be female ( p < 0.05). Among orbital lesions, myxoid liposarcoma was the most common histologic subtype (6/19; 31.6%) followed by well differentiated (5/19; 26.3%). This differed from the distribution of histologic subtypes encountered elsewhere, for which well-differentiated liposarcoma was the most common (retroperitoneum 979/3,136; 31%, soft tissue 3,493/11,671; 30%, and other sites 497/2,132; 23%, p < 0.05). Dedifferentiated histologic subtype was the second most common subtype found in the retroperitoneum (946/3,136; 30%), whereas it was less common in the orbit (2/19; 11%) and soft tissue (1,396/11,671; 12%) ( p < 0.001). Patients with orbital liposarcoma had similar disease-specific mortality compared with soft-tissue location ( p = 0.825) and lower disease-specific mortality compared with retroperitoneal location ( p < 0.001). When all locations were combined, patients with well-differentiated liposarcoma had the lowest disease-specific mortality. Patients with orbital liposarcoma tend to be younger, female, and have a better prognosis than those with retroperitoneal disease, likely due to the lower incidence of dedifferentiated histologic subtype.

Myxofibrosarcoma of Head and Neck Region - A Rare Case Report.

Myxofibrosarcoma (MFS) is a rare subtype of soft tissue sarcoma that usually occurs in the extremities of the body. Its location in the head and neck region is unique. Surgery is the primary treatment for all non-metastatic MFS. It has high rates of local recurrence and metastasis. Like other soft tissue sarcomas, the aim of adjuvant treatment is to decrease the chances of local recurrence or metastasis in MFS. Due to its rarity, there is a lack of data showing the benefit of adjuvant treatment in MFS of the head and neck region. We are presenting a case report and literature review on MFS in the head and neck.

What Is the Comparative Efficacy of Surgical, Endoscopic, Transanal Resection, and Radiotherapy Modalities in the Treatment of Rectal Cancer?

Rectal cancer is a significant healthcare burden, and effective treatment is crucial. This research aims to compare the effectiveness of surgical and endoscopic resection, transanal resection, and radiotherapy. A literature analysis was conducted in order to identify relevant studies, by comparing the different surgical approaches and variables affecting treatment decisions. The findings were analyzed and synthesized to provide a comprehensive overview. Surgical treatment, particularly TME (total mesorectal excision), proved consistent efficacy in achieving complete tumor resection and improving long-term survival. Endoscopic treatment and transanal resection techniques were promising for early-stage tumors but were associated with higher local recurrence rates. Radiotherapy, especially in combination with chemotherapy, played a crucial role in locally advanced cases, improving local control and reducing recurrence risk. Patient data, tumor characteristics, and healthcare system factors were identified as important factors in treatment modality selection. Surgical treatment, specifically TME, remains the recommended standard approach for rectal cancer, providing excellent oncological outcomes. Endoscopic treatment and transanal resection techniques can be considered for selected early-stage cases, while radiotherapy is beneficial for locally advanced tumors. Treatment decisions should be personalized based on patient and tumor characteristics, considering the available resources and expertise within the healthcare system.

Comprehensive molecular characterization of adenoid cystic carcinoma reveals tumor suppressors as novel drivers and prognostic biomarkers.

Adenoid cystic carcinoma (ACC) is a MYB-driven head and neck malignancy with high rates of local recurrence and distant metastasis and poor long-term survival. New effective targeted therapies and clinically useful biomarkers for patient stratification are needed to improve ACC patient survival. Here, we present an integrated copy number and transcriptomic analysis of ACC to identify novel driver genes and prognostic biomarkers. A total of 598 ACCs were studied. Clinical follow-up was available from 366 patients, the largest cohort analyzed to date. Copy number losses of 1p36 (70/492; 14%) and of the tumor suppressor gene PARK2 (6q26) (85/343; 25%) were prognostic biomarkers; patients with concurrent losses (n = 20) had significantly shorter overall survival (OS) than those with one or no deletions (p < 0.0001). Deletion of 1p36 independently predicted short OS in multivariate analysis (p = 0.02). Two pro-apoptotic genes, TP73 and KIF1B, were identified as putative 1p36 tumor suppressor genes whose reduced expression was associated with poor survival and increased resistance to apoptosis. PARK2 expression was markedly reduced in tumors with 6q deletions, and PARK2 knockdown increased spherogenesis and decreased apoptosis, indicating that PARK2 is a tumor suppressor in ACC. Moreover, analysis of the global gene expression pattern in 30 ACCs revealed a transcriptomic signature associated with short OS, multiple copy number alterations including 1p36 deletions, and reduced expression of TP73. Taken together, the results indicate that TP73 and PARK2 are novel putative tumor suppressor genes and potential prognostic biomarkers in ACC. Our studies provide new important insights into the pathogenesis of ACC. The results have important implications for biomarker-driven stratification of patients in clinical trials. © 2023 The Authors. The Journal of Pathology published by John Wiley & Sons Ltd on behalf of The Pathological Society of Great Britain and Ireland.

Establishment and characterization of NCC-DFSP4-C1: a novel cell line from a patient with dermatofibrosarcoma protuberans having the fibrosarcomatous transformation.

Dermatofibrosarcoma protuberans (DFSP) is a superficial low-grade sarcoma, genetically characterized by a fusion gene in collagen type I α (COL1A1) gene and platelet-derived growth factor subunit β (PDGFB). DFSP is locally aggressive and does not typically metastasize. However, DFSP with fibrosarcomatous transformation, which occurs in 7-16% of DFSP cases, demonstrates a poor prognosis than classic DFSP with a higher local recurrence rate and metastatic potential. Although imatinib, a PDGF receptor inhibitor, is a potent therapeutic agent for classic DFSP, it is less effective for DFSP with fibrosarcomatous transformation. The development of definitive chemotherapies for DFSP with fibrosarcomatous transformation is required. Patient-derived tumor cell lines are indispensable tools for preclinical research to discover novel therapeutic agents. However, only seven cell lines were derived from DFSP, out of which only two were established from DFSP with fibrosarcomatous transformation. Hence, in the present study, we established a novel DFSP cell line, NCC-DFSP4-C1, from a surgically resected DFSP tumor specimen with fibrosarcomatous transformation. NCC-DFSP4-C1 harbored an identical COL1A1-PDGFB fusion gene as its donor tumor. NCC-DFSP4-C1 cells retained the morphology of their donor tumor and demonstrated constant proliferation, spheroid formation, and invasion capability in vitro. By screening a drug library, we found that bortezomib and romidepsin demonstrated the strongest suppressive effects on the proliferation of NCC-DFSP4-C1 cells. In conclusion, we report a novel cell line of DFSP with fibrosarcomatous transformation, and demonstrate its utility in the development of novel therapeutic agents for DFSP.

Whole genome sequencing for metastatic mutational burden in extraskeletal myxoid chondrosarcoma.

Extraskeletal myxoid chondrosarcoma (EMC) is an ultra-rare cancer that makes up less than 3% of all soft tissue sarcomas. It most often arises in the soft tissues of the proximal limbs and has a higher incidence in males. Though EMC has a good prognosis, it has an indolent course with high rates of local recurrence as well as metastasis to the lungs. EMC is characterized in 70% of cases by an EWS1-NR4A3 translocation, leading to constitutive expression of NR4A3. Structural variants (SVs) in EMC, especially large-scale genomic alterations, have not been well studied and studies are severely limited by sample size. In this study, we describe Whole Genome Sequencing (WGS) of a rare case of matched EMC primary tumor, lung metastasis, and pelvic metastasis to identify genomic alterations. We examined somatic variants, copy number variants (CNVs), and larger scale SVs such as translocations and breakend points. While the primary tumor and lung metastasis had similar somatic variations and CNVs, the pelvic metastasis had more unique SVs with especially increased mutational burden of SVs in chromosome 2. This suggests that different molecular drivers appear in more advanced, relapsing EMC compared with the primary tumor and early lung metastasis. Genomic studies such as ours may identify novel molecular complexities in rare cancers that may be leveraged for therapeutic strategies and precision medicine.

Nectin-4 is frequently expressed in primary salivary gland cancer and corresponding lymph node metastases and represents an important treatment-related biomarker

Many locally advanced and metastatic salivary gland carcinomas (SGC) lack therapeutic targets. Enfortumab vedotin, an antibody–drug conjugate binding to Nectin-4, recently gained FDA approval for third-line urothelial carcinoma. Therefore, the aim of this study was to assess the expression of Nectin-4 in primary SGC and corresponding lymph node metastases and to correlate it with clinicopathological data. Immunohistochemical staining for Nectin-4 was performed for patients who had undergone surgery with curative intent for primary SGC of the parotid or submandibular gland in a tertiary referral center between 1990 and 2019. One hundred twenty-two primary SGC and twenty corresponding lymph node metastases were included. Nectin-4 was expressed in 80.3% of primary SGC with a mean Histo(H-)score of 61.2 and in 90.0% of lymph node metastases with a mean H-score of 75.6. A moderate or high Nectin-4 expression was found in 25.9% of salivary duct carcinomas (SaDu) and in 30.7% of adenoid cystic carcinomas (ACC). SaDu patients with a lower T-stage (p = 0.04), no loco-regional lymph node metastases (p = 0.049), no vascular invasion (p = 0.04), and no perineural spread (p = 0.03) showed a significantly higher mean Nectin-4 H-score. There was a statistical tendency towards a more favorable disease-free survival among SaDu patients with a higher Nectin-4 expression (p = 0.09). Nectin-4 is expressed in SGC and therefore represents a potential therapeutic target, especially in entities with a high rate of local recurrence and metastatic spread such as SaDu and ACC.

Similar additive effects of doxorubicin in combination with photon or proton irradiation in soft tissue sarcoma models.

High-precision radiotherapy with proton beams is frequently used in the management of aggressive soft tissue sarcoma (STS) and is often combined with doxorubicin (Dox), the first-line chemotherapy for STS. However, current treatment approaches continue to result in high local recurrence rates often occurring within the treatment field. This strongly indicates the need of optimized treatment protocols taking the vast heterogeneity of STS into account, thereby fostering personalized treatment approaches. Here, we used preclinical STS models to investigate the radiation response following photon (X) or proton (H) irradiation alone and in combination with different treatment schedules of Dox. As preclinical models, fibrosarcoma (HT-1080), undifferentiated pleiomorphic sarcoma (GCT), and embryonal rhabdomyosarcoma (RD) cell lines were used; the latter two are mutated for TP53. The cellular response regarding clonogenic survival, apoptosis, cell-cycle distribution, proliferation, viability, morphology, and motility was investigated. The different STS cell types revealed a dose-dependent radiation response with reduced survival, proliferation, viability, and motility whereas G2/M phase arrest as well as apoptosis were induced. RD cells showed the most radiosensitive phenotype; the linear quadratic model fit could not be applied. In combined treatment schedules, Dox showed the highest efficiency when applied after or before and after radiation; Dox treatment only before radiation was less efficient. GCT cells were the most chemoresistant cell line in this study most probably due to their TP53 mutation status. Interestingly, similar additive effects could be observed for X or H irradiation in combination with Dox treatment. However, the additive effects were determined more frequently for X than for H irradiation. Thus, further investigations are needed to specify alternative drug therapies that display superior efficacy when combined with H therapy.

Clinical Outcomes of Total En Bloc Spondylectomy for Previously Irradiated Spinal Metastases: A Retrospective Propensity Score-Matched Comparative Study.

This study aimed to investigate the clinical outcomes of total en bloc spondylectomy (TES) for spinal metastases previously treated with radiotherapy (RT). This study enrolled 142 patients who were divided into two groups: those with and those without an RT history. Forty-two patients were selected from each group through propensity score matching, and postoperative complications, local recurrence, and overall survival rates were compared. The incidence of postoperative complications was significantly higher in the group with an RT history than in the group without an RT history (57.1% vs. 35.7%, respectively). The group with an RT history had a higher local recurrence rate than the group without an RT history (1-year rate: 17.5% vs. 0%; 2-year rate: 20.8% vs. 2.9%; 5-year rate: 24.4% vs. 6.9%). The overall postoperative survival tended to be lower in the group with an RT history; however, there was no significant difference between the two groups (2-year survival: 64.3% vs. 66.7%; 5-year survival: 47.3% vs. 57.1%). When planning a TES for irradiated spinal metastases, the risk of postoperative complications and local recurrence should be fully considered.

Pure tubular carcinoma of the breast: a case series

BackgroundPure tubular breast carcinoma is a rare and well-differentiated tumor with high survival and low local recurrence rate. Our study aims to determine the clinical, radiological, appropriate management, and prognosis of this carcinoma.Materials and methodsA review of Salah Azaiez institute registry from 2004 to 2019 was performed including seven cases of PTC of the breast.ResultsClinical-pathologic features and outcomes were analyzed. The median follow-up was 3 years. In our study, we found that the cohort presented more frequently with pT1 disease and pN0 disease. Conservative surgery was more frequently indicated (five cases). All patients had hormone-receptor positivity and Human Epidermal growth factor Receptor 2 (HER2) negativity. The majority of tumors had a molecular profile luminal A and a low-grade SBR. In one case we found axillary lymph node metastasis. Adjuvant radiotherapy was indicated in all cases of breast conservation and in only one case of radical surgery. One patient received chemotherapy. The mean follow-up was 4 years. We did not find any local or distant recurrence in our study.ConclusionPTC showed an excellent prognosis with a low SBR grade, a molecular profile luminal A, and a low incidence of recurrence.

Machine learning-based radiomics analysis for predicting local recurrence of primary dermatofibrosarcoma protuberans after surgical treatment

Background and purposeDermatofibrosarcoma protuberans (DFSP) is characterized by locally invasive growth patterns and high local recurrence rates. Accurately identifying patients with high local recurrence risk may benefit patients during follow-up and has potential value for making treatment decisions. This study aimed to investigate whether machine learning-based radiomics models could accurately predict the local recurrence of primary DFSP after surgical treatment. Materials and methodsThis retrospective study included a total of 146 patients with DFSP who underwent MRI scans between 2010 and 2016 from two different institutions: institution 1 (n = 104) for the training set and institution 2 (n = 42) for the external test set. Three radiomics random survival forest (RSF) models were developed using MRI images. Additionally, the performance of the Ki67 index was compared with the three RSF models in the external validation set. ResultsThe average concordance index (C-index) scores of the RSF models based on fat-saturation T2W (FS-T2W) images, fat-saturation T1W with gadolinium contrast (FS-T1W + C) images, and both FS-T2W and FS-T1W + C images from 10-fold cross-validation in the training set were 0.855 (95% CI: 0.629, 1.00), 0.873 (95% CI: 0.711, 1.00), and 0.875 (95% CI: 0.688, 1.00), respectively. In the external validation set, the C-indexes of the three trained RSF models were higher than that of the Ki67 index (0.838, 0.754, and 0.866 vs. 0.601, respectively). ConclusionRandom survival forest models developed using radiomics features derived from MRI images were proven helpful for accurate prediction of local recurrence of primary DFSP after surgical treatment and showed better predicting performance than the Ki67 index.

Necrotic related-lncRNAs: Prediction of prognosis and differentiation between cold and hot tumors in head and neck squamous cell carcinoma.

Treatment of head and neck squamous cell carcinoma (HNSCC) is a substantial clinical challenge due to the high local recurrence rate and chemotherapeutic resistance. This project seeks to identify new potential biomarkers of prognosis prediction and precision medicine to improve this condition. A synthetic data matrix for RNA transcriptome datasets and relevant clinical information on HNSCC and normal tissues was downloaded from the Genotypic Tissue Expression Project and The Cancer Genome Atlas (TCGA). The necrosis-associated long-chain noncoding RNAs (lncRNAs) were identified by Pearson correlation analysis. Then 8-necrotic-lncRNA models in the training, testing and entire sets were established through univariate Cox (uni-Cox) regression and Lasso-Cox regression. Finally, the prognostic ability of the 8-necrotic-lncRNA model was evaluated via survival analysis, nomogram, Cox regression, clinicopathological correlation analysis, and receiver operating characteristic (ROC) curve. Gene enrichment analysis, principal component analysis, immune analysis and prediction of risk group semi-maximum inhibitory concentration (IC50) were also conducted. Correlations between characteristic risk score and immune cell infiltration, immune checkpoint molecules, somatic gene mutations, and anti-cancer drug sensitivity were analyzed. Eight necrosis-associated lncRNAs (AC099850.3, AC243829.2, AL139095.4, SAP30L-AS1, C5orf66-AS1, LIN02084, LIN00996, MIR4435-2HG) were developed to improve the prognosis prediction of HNSCC patients. The risk score distribution, survival status, survival time, and relevant expression standards of these lncRNAs were compared between low- and high-risk groups in the training, testing and entire sets. Kaplan-Meier analysis showed the low-risk patients had significantly better prognosis. The ROC curves revealed the model had an acceptable predictive value in the TCGA training and testing sets. Cox regression and stratified survival analysis indicated that the 8 necrosis-associated lncRNAs were risk factors independent of various clinical parameters. We recombined the patients into 2 clusters through Consensus ClusterPlus R package according to the expressions of necrotic lncRNAs. Significant differences were found in immune cell infiltration, immune checkpoint molecules, and IC50 between clusters, suggesting these characteristics can be used to evaluate the clinical efficacy of chemotherapy and immunotherapy. This risk model may serve as a prognostic signature and provide clues for individualized immunotherapy for HNSCC patients.

Giant cell tumor of clivus: a rare case report and review of literature

Clival giant cell tumors (GCTs) are an extremely rare type of tumor, with only 13 cases reported to date. Despite being histologically considered benign, these tumors can be locally aggressive and have a high rate of local recurrence, as well as the potential for distant metastasis. Due to their rarity and the difficulty of treating them due to their critical location, the ideal treatment protocol for managing clival GCTs remains controversial. The present report describes a 22-year-old female who experienced ptosis on her right eye and visual disturbance for one month. Magnetic resonance imaging (MRI) revealed a large, heterogenous, lobulated mass lesion arising from the clivus, extending anteriorly and compressing the optic chiasm and optic tract, with both parasellar extension and extension into the sphenoid sinus. An endoscopic endonasal trans-sphenoidal procedure was performed to excise the lesion. Post-operative MRI revealed residual disease, so a revision surgery was done using a combined microscopic and endoscopic procedure. Following surgery, the patient was given Denosumab for one year as well as has been symptom-free for the past 18 months of follow-up. This report contributes to the limited literature on the GCTs involving the clivus.

Needle tract seeding of a sclerosing epithelioid fibrosarcoma in a biopsy tract: a case report

BackgroundA sclerosing epithelioid fibrosarcoma (SEF) is an uncommon tumor of the deep soft tissue. An SEF has been described as a low-grade tumor with high local recurrence and metastatic rates. Generally, in bone and soft tissue tumors, a resection of the biopsy route is recommended; however, there is limited evidence with respect to the dissemination of the tumor tissue during a needle biopsy.Case presentationA mass in the right pelvic cavity, with no symptoms, was observed in a 45-year-old woman during a gynecological examination. Computed tomography (CT) revealed a multilocular mass with calcification in the pelvic cavity. The magnetic resonance imaging (MRI) showed an iso-signal intensity on T1 weighted images and hypo- and iso-signal intensity on T2 weighted images. The CT-guided core needle biopsy was performed using a dorsal approach, and the biopsy diagnosis was a low-grade spindle cell tumor. The tumor was excised using an anterior approach. The tumor tissue comprised spindle cells and epithelioid cells with irregular nuclei, and the immunohistological analysis was positive for vimentin and epithelial membrane antigen, which was consistent with a diagnosis of sclerosing epithelioid fibrosarcoma. Five years after the surgery, the MRI showed a tumor recurrence in the subcutaneous tissue of the right buttock, which was consistent with the needle biopsy tract. The patient underwent a tumor excision, and the resected tumor was similar to the primary tumor.ConclusionsThe recurrent tumor was excised with a surgical margin, and the tumor specimen had the histological features of a sclerosing epithelioid fibrosarcoma. It was difficult to investigate the association of the core needle biopsy with the tumor recurrence because the approach of the biopsy tract is usually same as that used in a tumor excision. However, the present case indicated the tumor may recur in the biopsy tract of a soft tissue sarcoma. Surgeons should be aware of the possibility of disseminating tumor tissues in a needle biopsy.

Pelvic Ewing sarcoma in children: Multimodal treatment, long term outcome and prognostic factors.

e22010 Background: The pelvis is one of the primary sites of Ewing sarcoma (ES) and is associated with a higher rate of local recurrence and poorer prognosis, compared with nonpelvic sites. Due to the rarity of this disease and limited data available, the prognostic factors of pelvic ES remain controversial. This study aims to assess the demographic, clinical features, treatment approach, outcome and prognostic factors in children with pelvic Ewing Sarcoma. Methods: Files of 264 children and adolescents with Ewing sarcoma diagnosed and treated between 1990-2022 in our center were retrospectively reviewed, of these 42 (15.9 %) were located in the pelvis. All recieved a COG modified chemotherapy regimen of ifosfamide-etoposide, alternating with vincristin, adriamisin/actinomycinD and cyclophosphamide (IE/VAC) every three weeks (every two weeks since 2012). Local treatment was discussed in each patient in the tumor board. Variables including age, gender, tumor stage, local and systemic treatment, outcome were analyzed using the Kaplan-Meier method and log rank test. Results: The median age of the 42 (23 male, 19 female) patients was 12.5 (2-16) years. The median time to diagnosis was 4 (0.25-12) months. The primary site was skeletal in 37 and extraskeletal in 5 patients. Seventeen (%40) had metastasis at diagnosis (lung, bone, bone marrow). Radiotherapy (RT) was given to 36 patients (10 preoperatively, 5 postoperatively, 21 definitive RT), 19 underwent surgery (4 at diagnosis, 14 had additional RT ). Surgical margins were positive in 6 and negative in 13. The histopathological necrosis was &lt;90% in 6 and ≥ 90% in 9. The median follow-up was 56 (10-370) months. At a median of 13 months (3-115), 22 patients had recurrence(9) /progression (13). The 3-year event free survival (EFS) and overall survival (OS) were 50.1% and 62.6%; in all; 62.2 and 75.3% in nonmetastatic; 31.9% ve %42.9% in metastatic patients ( p=0.048, p= 0.028) respectively. The 3-year EFS and OS were significantly higher in patients with negative surgical margins compared to ones with positive margins [(%83.9 vs 0 % (p=0.0001) and %91.7 vs %16.7 (p=0.001) respectively] . The 3-year EFS and overall survival OS were higher in patients with ≥90% necrosis [76.2 vs 33.3 % ( p=0.029); 87.5 vs 50% (p=0.13)respectively]. Age, gender, the mode of local therapy (surgery/RT/RT+surgery) was not prognostic. One patient who had recieved oral etoposide and RT at recurrence and who achieved a complete response, developed acute myeloid leukemia (AML), M5 myelomonocytic subtype, 20 months after relapse, and died. Conclusions: In our cohort of patients with pelvic Ewing sarcoma, 40% had metastasis at diagnosis. Metastasis, positive surgical margins, poor necrosois rate &lt;90% after neoadjuvant chemotherapy were found to be poor prognostic factors for outcome. Patients with localized pelvic Ewing sarcoma had a successful outcome with multidisciplinary treatment approach.

Is the Thickness of the Margin Associated With Local Recurrence and Survival in Patients With Myxofibrosarcoma?

Myxofibrosarcoma (MFS) is a spectrum of aggressive soft tissue fibroblastic neoplasms characterized by variable myxoid stroma, pleomorphism, and a distinctive curved vascular pattern; these tumors are associated with a high likelihood of recurrence. Better local tumor control (a tumor-free margin) is believed to be important to minimize the risk of recurrence, but the effect of surgical resection margin status on local recurrence and survival in MFS is not as well-characterized as it might be. (1) Is margin width associated with local recurrence? (2) Is there a relationship between greater margin thickness and improved overall and disease-free survival (DFS)? (3) Is worsening French Federation of Cancer Centers grade associated with local recurrence and poorer overall survival? Using a database of patients with bone and soft tissue tumors at a tertiary university hospital, we retrospectively reviewed the medical records of 282 patients who had soft tissue sarcomas and who had been surgically treated by a multidisciplinary bone and soft tissue tumor care team between January 2010 and December 2021. Of these 282 patients, 38 were identified as having MFS. Patients who received surgical care for MFS outside our institution (unplanned resection) (four patients) and whose surgical margins were not reported as microscopic numerical data (10) were excluded from the analysis. We estimated survival and local recurrence and examined factors potentially influencing these outcomes. Patient demographics, tumor characteristics, surgical margin distance (in mm), and disease-related outcomes were recorded. The minimum follow-up was 3 months (median 41.5 months, range 3 to 128 months). Overall 1-year local recurrence-free rates were 66.7% (95% CI 50% to 88%). Patients with positive margins were more likely to have local recurrence than patients with negative margins (HR 10.91 [95% CI 2.61 to 45.66]; p = 0.001). Patients with an inadequate margin (positive margin or a negative margin of 1 mm or less) had a greater risk of local recurrence (HR 9.96 [95% CI 1.22 to 81.44]; p = 0.032). Patients with positive margins or margins less than or equal to 1 mm had worse 2-year local recurrence-free survival than did those with margins of greater than 1 mm (46.9% [95% CI 16% to 76%] versus 91.7% [95% CI 75% to 100%]; p = 0.005). The mean overall survival was 98 months (95% CI 77.2 to 118.8). The Kaplan-Meier overall 1-, 2- and 5-year estimated rates of survival were 88% (95% CI 75% to 100%), 79.2% (95% CI 64.5% to 97.2%), and 73.5% (95% CI 57.2 % to 94.5%), respectively. Positive surgical margins were associated with decreased overall survival (HR 6.96 [95% CI 1.39 to 34.89]; p = 0.018). There was a mean DFS time of 4.25 months (95% CI 0.92 to 7.59) in microscopically positive patients, 75.5 months (95% CI 37.47 to 113.53) in patients with margins 1 mm or less, and 118 months (95% CI 99.23 to 136.77) in patients with margins over 1 mm. There was a statistical difference between DFS times according to surgical margin classification (p < 0.001). With the numbers we had, we could not detect any difference between the histologic grades determined by the French Federation of Cancer Centers grading system in terms of local recurrence (HR 3.80 [95% CI 0.76 to 18.94]; p = 0.103) and overall survival (HR 6.91 [95% CI 0.79 to 60.13]; p = 0.080). Tumor size was the prognostic factor associated with a higher local recurrence rate among all factors analyzed as univariate (HR 1.18 [95% CI 1.05 to 1.32]; p = 0.004). A surgical procedure with a sufficient negative surgical margin distance appears to be associated with a lower proportion of patients who experience a local recurrence and is associated with overall patient survival. It is difficult to define what a sufficient margin is, but in our patients, it appears to be greater than 1 mm.Level of Evidence Level III, therapeutic study.

The efficacy and safety of cold snare versus hot snare polypectomy for endoscopic removal of small colorectal polyps: a systematic review and meta-analysis of randomized controlled trials.

The optimal technique for removing small colorectal polyps ranging from 5 to 10mm in size remains uncertain. In order to compare the efficacy and adverse events between cold snare polypectomy (CSP) and hot snare polypectomy (HSP) for removing small polyps, we conducted a systematic review and meta-analysis of available randomized controlled trials. We conducted a comprehensive search of MEDLINE, EMBASE, and the Cochrane Library databases from 1998 to May 2023 to identify randomized controlled trials that evaluated the efficacy and safety of cold snare polypectomy (CSP) and hot snare polypectomy (HSP) for resecting small colorectal polyps. The primary outcome measure was the incomplete resection rate (IRR). Seven studies that met our study criteria, with a total of 3178 included polyps, were included in our analysis. The incomplete resection rate (IRR) was found to be significantly higher in the CSP group compared to the HSP group (risk ratio [RR] 1.57 [1.17-2.11], P = 0.003). Although the CSP group had a higher local recurrence rate than the HSP group, the difference was not statistically significant (RR: 3.98 [0.66-23.84], P = 0.13). The polyp retrieval rates were not significantly different between the two groups (RR: 1.00 [0.99-1], P = 0.22). Perforation was not reported or mentioned in any of the seven studies. The overall immediate bleeding rate was significantly higher in the CSP group than the HSP group (RR: 2.26 [1.63-3.14], P < 0.001), but immediate postpolypectomy bleeding requiring additional intervention was similar between the groups (RR: 1.08 [0.54-2.17], P = 0.82). The delayed bleeding rate (RR: 0.83 [0.45-1.55], P = 0.56) and specific polypectomy time (RR: -0.46 [-1.05-0.12], P = 0.12) were also similar between the groups. The meta-analysis shows a significantly higher IRR for CSP compared with HSP when removing small polyps.