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.

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.

Gemcitabine and Docetaxel as a Second-Line Treatment in Advanced Soft Tissue Sarcomas

OBJECTIVE Promising anti-tumor activity in patients with metastatic or unresectable soft tissue sarcomas has been reported with gemcitabine and/or docetaxel. METHODS Forty patients with advanced soft tissue sarcomas refractory to first-line chemotherapy treatment were enrolled. They received combination of gemcitabine at dose of 900 mg/m2 on days 1 and 8 and docetaxel at dose of 100 mg/m2 on day 8, and had this regimen repeated every 3 weeks. If the patients had received the pelvic irradiation in advance, gemcitabine dose was reduced to 675 mg/m2 on days 1 and 8 and docetaxel to 75 mg/m2 on day 8, and had it repeated every 3 weeks. RESULTS Gemcitabine/docetaxel combination was well tolerated by the patients with an overall response of 20%. After median follow-up of 15 months, a median overall survival time was 12 months （95% CI 7.042-16.958） and a median progression free survival time was 6 months （95% CI 5.445-6.545）. The most common hematologic toxicity was neutropenia （47.5%） while mucositis was the most common nonhematologic toxicity （45%）. The 1- and 2- year survival rates were 50% and 15%, respectively. CONCLUSION This regimen of gemcitabine/docetaxel combination as second-line treatment for the patients with advanced soft tissue sarcomas is effective with acceptable toxicities. These results should be evaluated in a large phase III trial.

Mutational analysis of the PTEN gene in soft tissue sarcomas

Objective： This study was to investigate whether PTEN mutations play a role in the carcinogenesis of soft tissue sarcomas （STS）. Methods： Polymerase chain reaction-single strand conformation polymorphism （PCR-SSCP） was used to amplify 4 exons of PTEN and to analyze the conformation polymorphism, then DNA sequencing methods was used to detect point mutation of PTEN gene four exons of abnormal single strand conformation in soft tissue sarcomas. Results： Two of 86 cases showed 130th condon G→A missense mutation in the exon 8 of PTEN gene, and this mutation made Arg to change to Gin in PTEN protein structure 334th condon A→T missense mutation in the exon 8 of PTEN gene, and this mutation made Asn to change to Lys in PI-EN protein structure. Conclusion： These data indicated the existence of PTEN mutation in soft tissue sarcomas, but PTEN gene mutation rate is very low. PTEN mutation may prays an less important role in the development and malignant transformation of soft tissue sarcomas.

Clinical analysis of 251 cases of soft tissue sarcomas

Objective To explore the therapeutic principles and prognostic factors of soft tissue sarcoma. Methods Two hundred and fifty-one patients with soft tissue sarcoma (STS) treated at Shanghai Cancer Hospital during 1986 -1990 were reviewed retrospectively. Results The 1-, 3 - , 5 - , 10 - year tumor-free survival rates were 67. 74% , 57. 16% , 52, 41% , 38. 60% , respectively.The overall survival rates for 1,3,5 and 10 years were 81. 01 % ,67.75% ,60.79% ,and 49.23% respectively. Log-rank test showed that the patients with different pathological findings, histological grades,mass location and size, anatomical depth, and surgical margin showed different outcomes. Whether the sarcomas invaded the vessels or metastasized would influence the survival rates. The patients who underwent different interventions or operations also had different outcomes. The prognosis of STS was associated with age,histological type,histological grade, tumor size, surgical margin and metastasis according to the Cox regression

Unusual Anterolateral Fascio Cutaneous Tight Flap for Extremity Soft Tissue Reconstruction

Soft tissue sarcoma is rare neoplasms that affect mainly the extremities. Surgery is the mean treatment and the resection results in extensive muscle and skin loss. The anterolateral tight flap is a good option for sarcomas arising in knees, superior third of legs and the medial face of thigh. The anatomy knowledge and the multidisciplinary approach is very important for a successful reconstruction. The authors report a successful case treated with the anterolateral tight flap and a literature review.

Management of oligometastatic disease in soft tissue sarcomas

Oligometastatic soft tissue sarcoma represents an intermediate state between localized and disseminated disease.Combination A combination of surgery,radiotherapy and systemic treatment significantly improves prognosis,with a 5-year overall survival as high as 50%.Due to the high prevalence of lung metastases,most of the surgical evidence is centered around lung metastasectomy.The decision to perform surgical metastasectomy remains dependent on optimal patient selection.Adequate post-surgical lung function,absence of extrapulmonary metastases,control of the primary tumor,and feasibility of achieving negative margins are major criteria for patients to undergo successful surgery.Adequate margins,longer disease-free interval,unilateral,limited number(≤2),metachronous and small(<2 cm)pulmonary metastasis are some factors associated with improved survival.Radiotherapy,especially SBRT,is an effective treatment for disease control,and its use as(neo)-adjuvant therapy has shown promising results.However,studies comparing radiotherapy against surgery are missing and the efficacy of radiotherapy independent of surgery is not yet clear.Interventional radiology techniques such as percutaneous thermal ablation(PTA)or arterial embolization have also been described as potential treatment alternatives in candidates deemed not fit for surgery.Systemic treatment has traditionally consisted of an anthracycline(doxorubicin)-based regimen with the addition of ifosfamide in certain cases.Recent advances in systemic treatment include the use of targeted therapy and immunotherapy in(oligo)-metastatic STS.However,except for certain histologies,most STS subtypes are chemoresistant,and the response to systemic treatment is poor.

INVESTIGATION OF RELATIONSHIPS BETWEEN KI－67SCORE，DNA INDEX，AND HISTOLOGIC GRADEIN SOFT TISSUE SARCOMAS

Proliferative activity of soft tissue sarcomas(STS)in 31 cases was estimated by histologic grading, mitotic count, DNA analysis by flow cytometry, and immunohistochemical procedures with monoclonal antibody Ki-67.Aneuploid was found in 12 of 16 cases(75.0%) with Grade 3, and in 4 of 15 cases(26.7%) with Grade 1, 2(P=0.0121).Tumors with more than 100 Ki-67 positive tumor cells per 10 high power fields(HPF) had a higher rate of aneuploid(81.3%) than those with less than 100 Ki-67 per 10 HPF (26.7%)(P=0.0038). There were significant correlations between Grade and DI (r=0.4901, P=0.0051), Grade and Ki-67(r=0.4636, P=0.0086), Ki-67 and DI(r=0-6368, P=0.0001). The results indicate that DI and reactivity of tumor cells to Ki-67 may reflect Proliferative activity and be helpful for clinicians to judge the biological behaviour of tumors more accurately and objectively. Supplementary to the grading of STS, DI and Ki-67 score could be useful as Prognostic parameters for clinical investigation of multimodality therapy for individual Patients.

Analysis of soft tissue sarcomas in 1118 cases

Background It is important to analyze and compare soft tissue sarcomas periodically so as to update the incidence, the clinical diagnosis, the treatment, and the ongoing research. The present study was conducted to determine the relative frequency of each type of soft tissue sarcoma.Methods A total of 1118 cases of primary soft tissue sarcomas treated between January 1993 and December 2006 were evaluated in a retrospective analysis.Results According to the pathologic grouping, the diseases with the highest proportion were malignant fibrous histiocytomas （35.24%）, synovial sarcomas （17.08%）, liposarcomas （16.28%）, and rhabdomyosarcomas （12.61%）. Soft tissue sarcomas were detected in every age group and occurred in all parts of the body. The number of cases increased gradually over the years.Conclusions Malignant fibrous histiocytomas had the highest frequency among the soft tissue sarcomas. The number of cases increased gradually over the years.

Adjuvant therapy for orbital non-rhabdomyosarcoma soft tissue sarcoma:comparison of long-term outcome between radiotherapy and chemotherapy

AIM:To illustrate clinicopathological features of orbital non-rhabdomyosarcoma soft tissue sarcoma(NRSTS),and to compare the treatment outcome between postoperative radiotherapy(RT) and chemotherapy in a retrospective analysis nearly 20y.METHODS:A retrospective cohort study of 56 patients with orbital NRSTS were reviewed,34 of whom received postoperative RT,and 22 received postoperative chemotherapy.The clinicopathological features,local recurrence,metastases,and survival data were recorded.Survival analysis was performed using the Kaplan-Meier method.RESULTS:During follow-up(111.8mo,ranged 8-233mo) for 56 patients,19 patients of them developed local recurrence,and 7 patients developed distant metastases.Fifteen patients died during follow-up period.Overall survival rates considering the whole study group was 78.57% at 5y,and 72.16% at 10y after the initial diagnosis.Compared with chemotherapy,RT was associated with lower risk of local recurrence [hazard ratio for RT vs chemotherapy,0.263,95% confidence interval(CI),0.095-0.728,P=0.0015];with lower risk of distant metastasis(hazard ratio for RT vs chemotherapy,0.073,95%CI,0.015-0.364,P=0.0014);and with lower risk of death from disease(hazard ratio for RT vs chemotherapy,0.066,95%CI,0.022-0.200,P<0.0001).The 5-year survival rate in RT group was 97.06% compared to 50% in chemotherapy group.CONCLUSION:In patients with orbital NRSTS,postoperative RT provides better control of local recurrence,distant metastasis,and death from disease than chemotherapy.RT is the more preferrable adjuvant therapy compared to chemotherapy possibly.

New trends in the surgical management of soft tissue sarcoma: The role of preoperative biopsy

Soft tissue sarcoma(STS)accounts for 1%of all malignant neoplasms in adults.Their diagnosis and management constitute a challenging target.They originate from the mesenchyme,and 50 subtypes with various cytogenetic profiles concerning soft tissue and bones have been recognized.These tumors mainly affect middle-aged adults but may be present at any age.Half of the patients have metastatic disease at the time of diagnosis and require systemic therapy.Tumors above 3-5 cm in size must be suspected of potential malignancy.A thorough history,clinical examination and imaging that must precede biopsy are necessary.Modern imaging techniques include ultrasound,computed tomography(CT),new magnetic resonance imaging(MRI),and positron emission tomography/CT.MRI findings may distinguish low-grade from high-grade STS based on a diagnostic score(tumor heterogeneity,intratumoral and peritumoral enhancement).A score≥2 indicates a high-grade lesion,and a score≤1 indicates a lowgrade lesion.For disease staging,abdominal imaging is recommended to detect early abdominal or retroperitoneal metastases.Liquid biopsy by detecting genomic material in serum is a novel diagnostic tool.A preoperative biopsy is necessary for diagnosis,prognosis and optimal planning of surgical intervention.Core needle biopsy is the most indicative and effective.Its correct performance influences surgical management.An unsuccessful biopsy means the dissemination of cancer cells into healthy anatomical structures that ultimately affect resectability and survival.Complete therapeutic excision(R0)with an acceptable resection margin of 1 cm is the method of choice.However,near significant structures,i.e.,vessels,nerves,an R2 resection(macroscopic margin involvement)preserving functionality but having a risk of local recurrence can be an acceptable choice,after informing the patient,to prevent an unavoidable amputation.For borderline resectability of the tumor,neoadjuvant chemo/radiotherapy has a place.Likewise,after surgical excision,adjuvant therapy is indicated,but chemotherapy in nonmetastatic disease is still debatable.The five-year survival rate reaches up to 55%.Reresection is considered after positive or uncertain resection margins.Current strategies are based on novel chemotherapeutic agents,improved radiotherapy applications to limit local side effects and targeted biological therapy or immunotherapy,including vaccines.Young age is a risk factor for distant metastasis within 6 mo following primary tumor resection.Neoadjuvant radiotherapy lasting 5-6 wk and surgical resection are indicated for highgrade STS(grade 2 or 3).Wide surgical excision alone may be acceptable for patients older than 70 years.However,locally advanced disease requires a multidisciplinary task of decision-making for amputation or limb salvage.

HIGH DOSE IFOSFAMIDE, DOXORUBICIN, DACARBAZINE AND G-CSF FOR PATIENTS WITH METASTATIC OR LOCALLYADVANCED SOFT TISSUE SARCOMA

Objective: A pilot study to test the feasibility and efficacy of high dose IFO and standard dose ADR and DTIC with G-CSF support in treatment of advanced soft tissue sarcoma (STS). Methods: 35 patients of no prior chemotherapy with metastatic or locally advanced unresectable STS were treated by this regimen, including 18 rhabdomyosarcomas, 7 malignant fibrous histiocytomas, 2 neurofibrosarcomas, 2 fibrosarcomas, 2 leiomyosarcomas, 2 synoviosarcomas, and 2 malignant hemangiopericytomas. IFO dose was 2 g/m2 on day 1–5 (with mesna uroprotection), ADR 50mg/m2 on day 1 and DTIC 250 mg/m2 on day 1–5. G-CSF (2 μg/kg/d) was administered on day 6 to 15 or until recovery of leukocytes account. The cycles were repeated every 3 weeks. Result: There were five complete responses (CR including pathologic CR) and eleven partial responses for overall 46% objective response rate. Most responses were observed within two cycles. The median survival was 15 months. Following CR, two patients remain disease free at 45 and 28 months, respectively. 6/120 (5%) cycles were complicated by grade IV neutropenia, 46/120 (38%) cycles had grade III neutropenia. No patients had treatment-related deaths. Nonhematologic toxicity consisted predominantly of anorexia and vomiting. No other severe toxicities were seen, especially no severe cardiotoxicity. Conclusion: This regimen is well tolerated and has substantial benefits for patients with advanced soft tissue sarcomas.

THE CLINIC－PATHOLOGIC ANALYSIS OF 66 CASES WITH SOFT TISSUE SARCOMA OF ABDOMEN

We accepted 66 cases with soft tissue sarcoma of abdomen testified by the Pathologists. The ratio of male to female was 1.5 to 1, but the aged-groups in female were more. The male incidences increased as the ages did. The mean age was 46.79 ±17. 04. The neoplasms arises more often in the abdominal wall below 30 years old, and it does mainly in the abdominal viscera and in the retroperitoneum over 30 years old. Fibrosarcoma Protuberance was the main type in the abdominal wall,and leiomyosarcoma was the main type in the abdominal viscera, and in the retroperitoneum, the most was liposarcoma, the following was leiomyosarcoma. Among the soft tissue sarcoma of the abdomen. the most was in retroperitoneum, and the following was in abdominal viscera. Therefore, it was difficult to diagnose early. The keys to the question were B-mode ultrasonic examining,computed tomography (CT) scanning and magnetic resonance imaging (MRI). Surgery was the primary therapeutic modality. The resectability was the major prognostic factor Radiotherapy and chemotherapy indicated a clear beneficial effect on soft tissue sarcoma of the abdomen and they can slightly decrease the recurrences and the metastases, but they had no obvious influence on the survival. In 5-year survival, the lesion of abdomen was the best, and those both of abdominal viscera and of retroperitoneum were the bad. To increase the 5-year survival, what counts are early diagnosis and the resectability.

Clinical analysis of 45 cases of surgical treatment for inner thigh primary soft tissue sarcoma

Objective: We aimed to study the relationship between clinical effect and surgical methods of inner thigh primary soft tissue sarcomas. Methods: Wide or radical resection were performed in 45 cases of soft tissue sarcomas, including 20 cases of postoperative recurrence after radiation therapy, 7 cases of first treatment. Thirty-six cases received 4–6 cycles of postoperative chemotherapy. Results: Thirty-eight of 45 cases were followed up for 1–5 years, with 5 case of recurrence and 6 cases of distant metastasis. Conclusion: The inner thigh primary soft tissue sarcoma can be effectively treated with wide or radical resection.

Fascia Lata Donor Site Foreign Body Granuloma: An Unusual Presentation of a Rare Mimic of Soft Tissue Sarcoma

Background: Foreign body granuloma is a rare late complication of fascia lata graft donor site with few reported cases in the literature. It can mimic soft tissue sarcoma. Clinical and radiological findings may not be enough to solve the puzzle and histology remains the mainstay of diagnosis. Aim: Our aim is to highlight the unusual long interval between initial surgery and presentation of foreign body granuloma. Case Presentation: A 65-year-old man who presented 48 years after initial surgery with progressive painless fascia lata donor site soft tissue swelling. The management and histology findings were highlighted. Conclusion: The occurrence of foreign body granuloma remains an important differential following a history of past surgical procedure regardless of the time interval between the surgical procedure and the clinical presentation.

Abdominal metastases of primary extremity soft tissue sarcoma: A systematic review

BACKGROUND Despite the fact that about one third of patients with primary localized extremity soft tissue sarcoma(e STS)will develop metastatic disease,abdominal metastases(AM)and retroperitoneal metastases(RM)constitute rare events.There is no clear consensus on how to achieve follow-up on patients with primary localized e STS following curative resection,especially regarding the surveillance of potential AM/RM.AIM To systematically analyse incidence,diagnosis,treatment and outcome of AM/RM in e STS patients.METHODS In this systematic review,899 studies available in Pub Med and published between 2000 and 2018 were screened,identifying 17 original articles focused on AM or RM in e STS.Article selection was based on the PRISMA guidelines,using the search terms(abdominal metastasis AND soft tissue sarcoma)and(soft tissue sarcoma metastasis abdomen).All studies published between January 1,2000 and December 31,2018 were screened.Further articles were identified by crosssearching article references,with the final search date being February 18,2019.Due to limited data and the different reporting techniques used,the present review focused on descriptive analysis of the included studies.RESULTS Of the 17 studies included,six original articles reported on incidence±diagnosis,therapy and outcome in AM and RM,whilst three original and eight case reports focused on diagnostic pathway,therapeutic procedures or outcomes without allowing conclusions regarding incidence of AM and RM.According to the former six studies,incidence of AM ranged from 0.9%-5.6%in patients with miscellaneous histological subtypes,and up to 12.1%in patients with myxoid liposarcoma.The most common histological subtypes that developed AM or RM were(myxoid)liposarcoma and leiomyosarcoma,but also rare subtypes such asepithelioid sarcoma,myxofibrosarcoma,synovial sarcoma,and malignant peripheral nerve sheath tumour had been reported to develop AM/RM.Surgery for AM/RM was performed in five of eight case-reports(62.5%)and in 20.8%-100.0%of original articles.In particular,patients with hepatic metastases undergoing metastasectomy had a survival benefit compared to patients treated with chemotherapy or best supportive care(>3 years vs<6 mo).CONCLUSION Patients with e STS should undergo surveillance with abdominal ultrasonography/computed tomography,or even whole-body-magnetic resonance imaging to detect AM/RM at an early stage.

Potential Mechanisms of Yanghe Decoction in the Treatment of Soft Tissue Sarcoma and Arteriosclerosis Obliterans Based on Network Pharmacology

Objective The objective of this study was to investigate potential mechanisms of Yanghe Decoction(YHD)in treating soft tissue sarcoma(STS)and arteriosclerosis obliterans(ASO)based on the use of network pharmacology.Methods Candidate compounds and potential targets were identifed through the TCM Systems Pharmacology database and a comprehensive literature search.Related targets of STS and ASO were collected in the GeneCards database,DisGeNET database,and Drugbank database.Furthermore,The STRING 11.0 database was used to determine protein-protein interaction(PPI)networks;common targets were obtained and imported into Cytoscape 3.7.2.Then,a PPI network comprising common targets was drawn,and network topology analysis was performed to screen for key shared targets.Gene ontology functional enrichment analysis and Kyoto Encyclopedia of Genes and Genomes pathway enrichment analysis of key shared targets were performed by using Metascape software.Subsequently.a compound-target-pathway network was constructed via Cytoscape 3.7.2.Results The following signaling pathways were found to be associated with the mechanisms of YHD in treating STS and ASO:AGE-RAGE signaling pathway,IL-17 signaling pathway;HIF-1 signaling pathway,TNF signaling pathway,interactions between cytokines and cytokine receptors,Th17 cell differentiation,and NOD-like receptor signaling pathway.Among the compounds and targets involved in these pathways,quercetin,luteolin,and kaempferol were found to be core compounds,and TNF,IL-6,and MAPK1 were found to be core targets.Conclusion Taken together,our findings elucidated that potential mechanisms of YHD in treating STS and ASO involved cellular proliferation/differentiation,angiogen-esis,inflammation,immune responses,oxidative stress,and other related signaling pathways.

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.

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.

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.