Multimodality Treatment in Adult Patients with High-risk Soft-tissue Sarcomas

Objective： We enrolled retrospective data to determine the efficacy of combined chemotherapy and surgery for local tumour control and survival in patients with high-risk soft-tissue sarcomas. Methods： We collected data from 25 patients with high-risk soft-tissue sarcomas treated with four cycles of etoposide, ifosfamide and doxorubicin （EIA） followed by definitive surgery with or without postoperative radiotherapy and adjuvant chemotherapy. 21 patients received chemotherapy in a neoadjuvant/adjuvant clinical setting; eighteen of them completed adjuvant chemotherapy. Four patients received chemotherapy in an adjuvant setting only. Results： The objective response rate of neoadjuvant chemotherapy assessable in 21 patients was 43%. Including NED （n=7） and partial remissions （n=3）, the radiographic response rate was 47.6% with additional 42.9% stable diseases （n=9）. Surgery was performed in two patients before completing four neoadjuvant chemotherapy cycles because of disease progression. Median overall survival for all patients was 21.6＋ months. After completion of chemotherapy, in 62% of patients R0-resection could be performed. Conclusion： High proportion of R0-resections supports the idea of tumour down-staging after neoadjuvant treatment. Response to neoadjuvant chemotherapy is predictive for improved local tumour control resulting in long-term survival benefit.

The Importance of the Multidisciplinary Approach to Surgical Treatment of Extremity Soft-Tissue Sarcomas

Soft tissue sarcomas (STS) are rare neoplasm with frequencies around 1% of all neoplasms. Although it consists of a high heterogeneous group of tumors, surgery is the mean treatment. The STS surgery is still challenging and complex procedure is usually required: this is because STS requires different types of resection and reconstruction due to various tissue-commitments (nerve, arteries, skin and muscle). So, a multidisciplinary team must be prepared for STS approach to obtain the maximum local control and a limited extremity functional impairment. We, here, showing our experience, wish to introduce some technical contrivances in STS surgery, with special reference to tissue reconstruction. This may illustrate the necessity of a multidisciplinary team approach in this surgery.

Clinical utility of circulating tumor DNA sequencing with a large panel in patients with advanced soft-tissue sarcomas

To the editor Soft-tissue sarcomas(STS)represent a very heterogeneous group of rare tumors including more than 100 different subtypes[1].Surgery and neo/adjuvant radiation therapy represent the cornerstone of treatment for STS.However,despite an optimal resection of the tumor,up to 40%of patients will develop metastatic relapse and will die from the disease[1].Doxorubicin represents the first-line standard of care for patients with advanced disease since the 1970s,despite several attempts to identify better regimens.The median overall survival(OS)of patients with metastatic disease is<18 months and has only modestly improved over the past 20 years[2].

Indication and surgical approach for reconstruction with endoprosthesis in bone-associated soft tissue sarcomas:Appropriate case management is vital

It is important for surgeons performing sarcoma surgery to know that bone resection and tumor prosthesis applications in soft tissue sarcomas(STS)have unique features in terms of indication,surgical approach and follow-up,in terms of the management of these cases.Some STS are associated with bone and major neurovascular structures.Bone-associated STS are generally relatively large and relatively deep-seated.Additionally,the tendency for metastasis is high.In some cases,the decision about which structures to resect is difficult.These cases are often accompanied by poor oncological and surgical outcomes.Management of cases should be done by a multidisciplinary team in advanced centers specialized in this field.The surgical team must have sufficient knowledge and experience in the field of limb-sparing surgery.Preoperative evaluation and especially good planning of bone and soft tissue reconstruction are vital.

Gene expression profiling improves prognostication by nomogram in patients with soft-tissue sarcomas

Dear Editor,Surgery remains the cornerstone of treatment for softtissue sarcomas(STS)[1].However,despite adequate locoregional treatment,30%-40%of the patients eventually develop metastases[2,3].Accurate risk assessment is crucial to tailor therapeutic strategies and to identify high-risk patients who may benefit from perioperative chemotherapy.Hence,several efforts have been made to develop prognostic nomograms that enable individual prognosis prediction;representing an important decision-making aid for oncologists and surgeons involved in sarcoma care[4-6].

Metastatic clear cell sarcoma of the pancreas:A rare case report

BACKGROUND Clear cell sarcoma(CCS)is a rare soft-tissue sarcoma.The most common metastatic sites for CCS are the lungs,bones and brain.CCS is highly invasive and mainly metastasizes to the lung,followed by the bone and brain;however,pancreatic metastasis is relatively rare.CASE SUMMARY We report on a rare case of CCS with pancreatic metastasis in a 47-year-old man.The patient had a relevant medical history 3 years ago,with abdominal pain as the main clinical manifestation.No abnormalities were observed on physical examination and the tumor was found on abdominal computed tomography.Based on the medical history and postoperative pathology,the patient was diagnosed with CCS with pancreatic metastasis.The patient was successfully treated with surgical interventions,including distal pancreatectomy and sple-nectomy.CONCLUSION This report summarizes the available treatment modalities for CCS and the importance of regular postoperative follow-up for patients with CCS.

Soft Tissue Sarcomas: Descriptive Study of 232 Cases Collected, over a Period of 10 Years, at the Hospital of Oncology, Department of the Hassan II Hospital FEZ

Soft-tissue sarcomas are uncommon tumors that have traditionally been managed by wide excisional surgery and radiotherapy;the use of chemotherapy has been reserved for advanced disease. Advances in multidisciplinary care have improved the evaluation and care of patients with this disease. Limb-conserving surgical paradigms, superior radiotherapy delivery, and novel adjuvant agents for specific tumors are now available. The objective of this study is to report the epidemiological, clinical, histological, therapeutic and evolutionary characteristics of soft tissue sarcomas at the oncology hospital: Chu Hassan II in FEZ, and to define the factors influencing patient survival. This is a retrospective study of 232 cases of soft tissue sarcoma, collected between January 2010 and June 2020. The eligibility criteria were an age greater than 16 years, and histological evidence of a soft tissue sarcoma excluding gastrointestinal stromal tumors (GIST). Items collected were: epidemiological, clinical, histological, radiological, and therapeutic. These are 232 cases, 120 Men and 112 Women, the mean age was 48.89 years (Extreme = 18 - 76 years). The tumor was localized to the extremities at (58.72%). The predominant histological type was Leiomyosarcoma in 61 cases (26.29%). The tumor stage was localized in (17.67%) of cases, locally advanced in 34.05% and metastatic in 44.08% of patients, all localized cases were treated surgically including (84%) conservative surgery and (16%) radical surgery. Radiation therapy was performed in 32.75% of patients. Chemotherapy was performed in 74.14% of patients. Age and tumor stage are prognostic factors influencing the survival of soft tissue sarcomas.

Emerging role of liquid biopsy in rat sarcoma virus mutated metastatic colorectal cancer:A case report

BACKGROUND In patients with metastatic colorectal cancer(mCRC),the treatment options are limited and have been proved to be affected by rat sarcoma virus(RAS)mutational status.In RAS wild-type(wt)patients,the combination of antiepidermal growth factor receptor(EGFR)monoclonal antibodies with chemotherapy(CT)is more effective than CT alone.On the other hand,RAS-mutated patients are not eligible for treatment with anti-EGFR antibodies.CASE SUMMARY Eleven patients with initially RAS-mutated mCRC were followed from diagnosis to May 2022.At the time of cell-free DNA determination,five patients had undergone one CT line,five patients had undergone two CT lines,and one patient had undergone three CT lines(all in combination with bevacizumab).At the second and third treatment lines[second line(2L),third line(3L)],patients with neo-RAS wt received a combination of CT and cetuximab.In neo-RAS wt patients treated with anti-EGFR,our findings indicated an increase in progression-free survival for both 2L and 3L(14.5 mo,P=0.119 and 3.9 mo,P=0.882,respectively).Regarding 2L overall survival,we registered a slight increase in neo-RAS wt patients treated with anti-EGFR(33.6 mo vs 32.4 mo,P=0.385).At data cut-off,two patients were still alive:A RAS-mutated patient undergoing 3L treatment and a neo-RAS wt patient who received 2L treatment with anti-EGFR(ongoing).CONCLUSION Our case series demonstrated that monitoring RAS mutations in mCRC by liquid biopsy may provide an additional treatment line for neo-RAS wt patients.

Clear cell sarcoma metastasizing to the pancreas

In this editorial based on a case report,we delve into a seldom-seen occurrence of clear cell sarcoma featuring pancreatic metastasis in a 47-year-old male patient.Recognized for its typical tendency to metastasize to the lungs,bones,and brain,clear cell sarcoma rarely extends its reach to the pancreas.Despite the initial absence of discernible abnormalities during the patient's physical examination,the manifestation of abdominal pain prompted further investigation.Subsequent abdominal computed tomography brought to light the presence of a pancreatic tumor,culminating in the definitive diagnosis of clear cell sarcoma with pancreatic metastasis.The successful management of this atypical presentation involved a series of surgical interventions,including distal pancreatectomy and splenectomy.This report not only sheds light on the infrequent manifestation of clear cell sarcoma within the pancreas but also underscores the pivotal role of vigilant postoperative follow-up in addressing this rare sarcoma.The emphasis on postoperative care serves as a crucial aspect of the broader narrative,acknowledging the need for ongoing monitoring and management to ensure a comprehensive and successful treatment trajectory for patients with this unique presentation of clear cell sarcoma.

Clear cell sarcoma of the pancreas,an unusual cancer with unusual metastatic site or unusual primary site?

Clear cell sarcoma(CCS)is a type of malignant tumor that can arise from tendons and aponeuroses.This malignant proliferation of cells with melanocytic lineage normally occurs in young patients,and it is normally identified in extremities.However,different sites including gastrointestinal organs are also described.Due difficulties in the molecular and histopathology evaluation,the diagnosis is often confused with malignant melanoma.Most cases are treated with surgical resection,but overall,the prognosis is poor.In this editorial,we will discuss a very interesting case of CCS identified in the pancreas.We will discuss the literature and controversies in the management of this type of cancer.Furthermore,we will address molecular strategies to be incorporated in those cases to better understand the primary location of the tumor.Finally,future perspectives of the field and new strategies of treatment will be described.

Undifferentiated high-grade pleomorphic sarcoma of the common bile duct:A case report and review of literature

BACKGROUND Undifferentiated pleomorphic sarcoma(UPS)is a rare malignant mesenchymal tumor with a poor prognosis.It mainly occurs in the extremities,trunk,head and neck,and retroperitoneum regions.Owing to the lack of specific clinical manifestations and imaging features,UPS diagnosis mainly depends on pathological and immunohistochemical examinations for exclusive diagnosis.Here we report an extremely rare case of high-grade UPS in the common bile duct(CBD).There are limited available data on such cases.CASE SUMMARY A 70-year-old woman was admitted to our department with yellow eyes and urine accompanied by upper abdominal distending pain for 2 wk.Her laboratory data suggested significantly elevated hepatorenal function levels.The imaging data revealed calculous cholecystitis,intrahepatic and extrahepatic bile duct dilation with extrahepatic bile duct calculi,and a space-occupying lesion at the distal CBD.After endoscopic biliary stenting and symptomatic support therapy,CBD exploration and biopsy were performed.The frozen section indicated malignant spindle cell tumor of the CBD mass,and further radical pancreaticoduodenectomy was performed.Finally,the neoplasm was diagnosed as a high-grade UPS combined with the light-microscopic morphology and immunohistochemical results.CONCLUSION This extremely rare case highlighted the need for increasing physicians'vigilance,reducing the odds of misdiagnosis,and providing appropriate treatment strategies.

Eyelid Kaposi Sarcoma in HIV-Negative Patient

Introduction: Kaposi sarcoma disease is a proliferative and multifocal disorder with dual components, vascular and fibroblastic cellular, cutaneous and visceral expression. Kaposi Sarcoma can affect the ocular surface and adnexa and can masquerade as other entities, delaying prompt diagnosis can lead to diagnostic wandering delaying treatment. Our aim is to describe a case of KS of the eyelid in an HIV seronegative patient. Case Presentation: A seventy-year-old man developed a bilateral growing tumoral reddish purple vascular mass on both the lower and upper eyelid involving rapidly for 6 months. Both feet and the two shanks show the presence of a brown-violet tumor-shaped formation. The patient was negative for HIV. Histology examination showed a nodular tumor-like mass with a fibro hemangioma-epitheliomatous. Polymerase chain reaction was positive for human herpes virus 8. Initial chemotherapy followed by surgery was proposed to the patient. Unfortunately, the patient rejected treatment and was lost to follow-up. Conclusion: This case reports the difficulty of managing KS in developing countries.

Bioinformatics Analysis of the Biological Properties of Ewing Sarcoma

Purpose: Bioinformatics-based approach to screen and analyze differentially expressed genes associated with the biological characteristics of Ewing sarcoma. Means: The GSE17674 dataset was selected for analysis, obtained by data retrieval based on the GEO public database. The R language limma toolkit was used to screen DEmRNAs. After the data were normalized, the Metascape online analysis software and the R language clusterProfiler package were used to analyze the GO function and KEGG pathway enrichment of DEmRNAs lines, respectively. The string database was selected for PPI analysis, and the results were imported into Cytoscape software to derive the core modules and predicted core genes. The genes selected above were analyzed for tissue localization specificity. Results: Through the analysis of GSE17674, differentially expressed genes were screened out, and GO and KEGG analyses were performed on the differentially expressed genes. The GO functional enrichment analysis was mainly enriched in the process of muscle system, muscle contraction, myocyte development, contractile fibers, myogenic fibers, myofibers, myofibrillar segments, actin binding, structural composition of muscle, and actin filament binding. KEGG pathway analysis showed that the core pathways associated with the development of ES were the core genes for myocardial contraction, congestive cardiomyopathy, and hypertrophic cardiomyopathy. Five Hub genes were obtained based on Cytoscape prediction. Tissue localization specificity analysis of Hub genes was performed, and a total of 2 Hub genes with tissue specificity were screened;MYH6 was specifically expressed in cardiac cells and MYL1 was specifically expressed in skeletal muscle cells. Conclusions: The differential genes screened will help to understand the molecular mechanisms underlying the highly invasive and metastasis-prone biological characteristics of ES, as well as provide new ideas for clinical drug-targeted treatment of ES.

Primary Ewing sarcoma of the kidney mimicking cystic papillary renal cell carcinoma in an older patient:A case repor

BACKGROUND Ewing’s sarcoma(ES)is a neuroectodermal tumor that typically occurs in the bones and soft tissues of children and young adults.Primary renal ES is rare;only a few cases and a small case series have been documented,and only four cases involved primary renal ES in older people(>65 years old).CASE SUMMARY Herein,we describe the radiological and pathological features of primary renal ES in an older person.A 76-year-old man complained of poor oral intake and was found to have a large cystic renal mass with indistinct margins on computed tomography.Ultrasound-guided biopsy revealed that the tumor contained small round blue cells.The patient underwent a right radical nephrectomy.The tumor cells showed diffuse membranous CD99,and nuclear friend leukemia integration 1 transcription factor and NK2 Homeobox 2.Fluorescence in situ hybridization revealed EWSR1 translocation.Postoperatively,18F-fluorodeoxyglucose positron emission tomography revealed no evidence of metastasis.The patient was diagnosed with primary renal ES.Six months following the surgery,local recurrence and distant metastasis were observed.Primary renal ES is rare and often lethal in older individuals.The specific imaging findings are unknown,and treatment protocols have not been standardized.CONCLUSION This case report describes the radiological and pathological features of primary renal ES in an older person.

Shedding light on pancreatic metastasis of clear cell sarcoma:An exceptional journey

This editorial comments on the study by Liu et al investigating pancreatic metastasis of clear cell sarcoma(CCS)published in the World Journal of Clinical Cases.CCS is a rare and aggressive melanocytic tumor,that typically arises from tendons and aponeuroses of the limbs,and metastasizes to the lungs,bones,and brain.However,pancreatic metastasis has rarely been reported,presenting unique diagnostic and therapeutic challenges.Elucidating the clinical characteristics,imaging features,prognostic factors,and treatment outcomes of patients with pancreatic CCS metastasis is crucial.Surgery remains an effective management strategy for CCS.However,the high recurrence rate and low effectiveness of traditional adjuvant treatments necessitate a shift towards more personalized and targeted treatment plans.Research is needed to investigate and validate novel therapeutic approaches specifically tailored to the distinct genetic and molecular characteristics of rare malignancies like CCS.Additionally,the development of late metastases after a long disease-free interval is common in CCS patients.Therefore,routine postoperative surveillance for metastasis using computed tomography,magnetic resonance imaging,bone scans,and positron emission tomography scans is crucial.Moving forward,enhanced collaboration,investigation,and creative thinking among scientists,medical professionals,and legislators are essential to gain a deeper understanding of these rare presentations.

Unlocking epigenetics for precision treatment of Ewing’s sarcoma

Ewing’s sarcoma(EWS)is a highly aggressive malignant bone tumor primarily affecting adolescents and young adults.Despite the efficacy of chemoradiotherapy in some cases,the cure rate for patients with metastatic and recurrent disease remains low.Therefore,there is an urgent need for innovative therapeutic approaches to address the challenges associated with EWS treatment.Epigenetic regulation,a crucial factor in physiological processes,plays a significant role in controlling cell proliferation,maintaining gene integrity,and regulating transcription.Recent studies highlight the importance of abnormal epigenetic regulation in the initiation and progression of EWS.A comprehensive understanding of the intricate interactions between EWS and aberrant epigenetic regulation is essential for advancing clinical drug development.This review aims to provide a comprehensive overview of both epigenetic targets implicated in EWS,integrating various therapeutic modalities to offer innovative perspectives for the clinical diagnosis and treatment of EWS.

Clinicopathological analysis of EWSR1/FUS::NFATC2 rearranged sarcoma in the left forearm:A case report

BACKGROUND We present a case of an EWSR1/FUS::NFATC2 rearranged sarcoma in the left forearm and analyze its clinicopathological and molecular features.CASE SUMMARY The patient is a 23-year-old woman.Microscopically,the tumor cells were medium-sized round cells arranged in small nests.The cytoplasm was clear,nuclei were relatively uniform,chromatin was dense,nucleoli were visible,and mitotic figures were rare.Immunohistochemically,the tumor cells were positive for Vimentin,INI-1,CD99,NKX2.2,CyclinD1,friend leukaemia virus integration 1,and NKX3.1.Next-generation sequencing revealed the presence of the EWSR1-NFATC2 fusion gene.EWSR1/FUS::NFATC2 rearranged sarcomas are rare and can easily be misdiagnosed.CONCLUSION Clinical imaging,immunohistochemistry,and molecular pathology should be considered to confirm the diagnosis.

Low-grade myofibrosarcoma of the maxillary sinus:Two case reports

BACKGROUND Low-grade myofibroblastic sarcoma(LGMS)is an extremely rare tumor characterized by the malignant proliferation of myofibroblasts.LGMS most commonly develops in adults,predominantly in males,in the head and neck region,oral cavity,especially on the tongue,mandible,and larynx.This article presents 2 cases of LGMS localized to the maxillary sinus and provides an overview of the available literature.CASE SUMMARY Two patients with LGMS located in the maxillary sinus underwent surgery at the Department of Head and Neck Surgery.Case 1:A 46-year-old patient was admitted to the clinic with suspected LGMS recurrence in the right maxillary sinus(rT4aN0M0),with symptoms of pain in the suborbital area,watering of the right eye,thick discharge from the right nostril,and augmented facial asymmetry.After open biopsy-confirmed LGMS,the patient underwent expanded maxillectomy of the right side with immediate palate reconstruction using a microvascular skin flap harvested surgically from the middle arm.The patient qualified for adjuvant radiotherapy for the postoperative bed,with an additional margin.Currently,the patient is under 1.5 years of observation with no evidence of disease.Case 2:A 45-year-old man was admitted to our clinic with facial asymmetry,strabismus,exophthalmos,and visual impairment in the right eye.Six months earlier,the patient had undergone partial jaw resection at another hospital for fibromatosis.A contrast-enhanced computed tomography scan revealed a tumor mass in the postoperative log after an earlier procedure.An open biopsy confirmed lowgrade fibrosarcoma(rT4aN0M0).The patient qualified for an extended total right maxillectomy with orbital excision and right hemimandibulectomy with immediate microvascular reconstruction using an anterolateral thigh flap.The patient subsequently underwent adjuvant radiotherapy to the postoperative area.After 9 months,recurrence occurred in the right mandibular arch below the irradiated area.The lesion infiltrated the base of the skull,which warranted the withdrawal of radiotherapy and salvage surgery.The patient qualified for palliative chemotherapy with a regimen of doxorubicin+dacarbazine+cyclophosphamide and palliative radiotherapy for bone metastases.The patient died 26 months after surgical treatment.The cases have been assessed and compared with cases in the literature.CONCLUSION No specific diagnostic criteria or treatment strategies have been developed for LGMS.The treatment used for LGMS is the same as that used for sinonasal cancer radical tumor excision;adjuvant radiotherapy or chemoradiotherapy should also be considered.They have low malignant potential but are highly invasive,tend to recur,and metastasize to distant sites.Patients should undergo regular follow-up examinations to detect recurrence or metastasis at an early stage.Patients should be treated and observed at the highest referral centers.

Metastatic clear cell sarcoma of the pancreas:From diagnosis to treatment

This article presents a comprehensive case report on an uncommon instance of metastatic clear cell sarcoma(CCS)originating from the pancreas.The high mortality rate of pancreatic carcinoma underscores the importance of precise diagnosis and early detection.The authors report a novel case of CCS with pancreatic metastasis,detailing successful surgical intervention through distal pancreatectomy and splenectomy,resulting in favourable outcomes.This study highlights the standard role of surgery in treating advanced CCS and emphasizes preoperative imaging and thorough patient history assessment.This article also underscores the necessity for long-term surveillance due to the potential for recurrence or metastasis.Despite the favourable recovery postsurgery,the absence of subsequent follow-up evaluation prompts consideration of the need for extended monitoring.This article raises questions about the nature of the pancreatic lesion and suggests the possibility of a primary lesion.Further evidence is crucial to establish the correlation between the features related to the development of the patient's primary and metastatic tumours.In conclusion,this study offers valuable insights into metastatic CCS of the pancreas,highlighting the importance of regular postoperative follow-up for improved outcomes through early detection and intervention.

Metastatic clear cell sarcoma of the pancreas:A sporadic cancer

Primary or secondary clear cell sarcoma of the pancreas is an exceedingly rare and aggressive disease.In addition to pathology,molecular analysis is pivotal in differential diagnosis,especially with malignant melanoma.A key aspect in identifying clear cell sarcoma is specific genetic alterations,notably the translocation of t(12;22)(q13;q13),a diagnostic hallmark of this sarcoma subtype,which is absent in malignant melanoma.Treatment of primary clear cell sarcoma of the pancreas is the same as that for adenocarcinoma.