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.

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.

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.

Metastatic clear cell sarcoma of the pancreas:An overview

Clear cell sarcoma(CCS)is a rare soft-tissue sarcoma that accounts for less than 1%of all cases and was originally reported in 1965.The incidence of CCS is estimated to be approximately 0.014/100000 depending on the surveillance,epidemiology and end results databases.CCS is a highly invasive type that mainly metastasizes to the lungs,followed by the bones and brain;however,pancreatic metastasis is relatively rare.It has a high probability of recurrence or metastasis and has a poor prognosis with a high mortality rate.Finally,even after recovery,it is fundamental to keep regular postoperative follow-up for CCS patients.

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.

Medical imaging for the diagnosis,recurrence and metastasis evaluation of clear cell sarcoma

Clear cell sarcoma(CCS)of soft tissue is extremely rare,accounting for approximately 1%of all soft tissue tumours.It is very difficult to diagnose CCS based on clinical manifestations.Magnetic resonance imaging(MRI)provides highresolution images of soft tissues and pathological features such as mucus,necrosis,bleeding,and fat through high and low signals on T1 weighted image(T1WI)and T2 weighted image(T2WI).On the other hand,the paramagnetism of melanin in CCS shortens the relaxation time of T1 and T2,and high signal intensity on T1WI and low signal intensity on T2WI can be found.This is different from most other soft tissue sarcomas.At present,the treatment method for CCS is surgical resection.MRI can effectively display the tumour edge,extent of surrounding oedema,and extent of fat involvement,which is highly important for guiding surgical resection and predicting postoperative recurrence.As an invasive sarcoma,CCS has a high risk of metastasis.Regardless of the pathological condition of the resected tumour,MRI or computed tomography(CT)should be performed every 1-2 years to assess recurrence at the primary site and to screen for metastasis in the lungs,liver,and bones.If necessary,PET-CT can be performed to evaluate the overall condition of the patient.

Malignant melanoma:An important differential diagnosis for clear cell sarcoma of the gastrointestinal tract

A case report by Liu et al describes the characteristics of metastatic clear cell sarcoma(CCS)of the pancreas and provides valuable therapeutic insights for this rare malignancy.This case is interesting because of its rarity,suggesting that the pancreas may be a potential target organ for CCS,either primary or metastatic.At the same time,the authors also emphasize the importance of regular postoperative follow-up for timely detection of recurrent lesions,as CCS is characterized by a high degree of malignancy and a high rate of recurrent metastases.Considering that CCS of the gastrointestinal tract is easily confused with malignant melanoma(MM)of the gastrointestinal tract,here we compare the clinical features,histopathological and immunohistochemical characteristics,diagnosis,treatment,and prognosis of CCS and MM of the gastrointestinal tract,hoping to provide a reference for clinical work.

Essential role of postoperative follow-up in the management of clear cell sarcoma

Clear cell sarcoma(CCS)is a rare melanocytic soft tissue sarcoma known for itspropensity to metastasize to the lymph nodes and typically has an unfavorableprognosis.Currently,surgical resection is the primary treatment for localizedCCS,while radiotherapy and chemotherapy are preferred for metastatic cases.The roles of adjuvant chemotherapy,radiotherapy,and lymph node dissection arecontroversial.Although immunotherapy has emerged as a promising avenue inCCS treatment research,there are no established clinical standards for postoperativefollow-up.This editorial discusses a recent article by Liu et al,with afocus on current diagnostic modalities,treatment approaches,and the challengingprognosis associated with CCS.Our aim is to underscore the importance of longtermpatient follow-up in CCS management.

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.

Clear Cell Pleomorphic Dermal Sarcoma: A Case Report and Literature Review

Introduction: Atypical fibroxanthoma (AFX) and pleomorphic dermal sarcoma (PDS) are one spectrum of rare cutaneous neoplasms that typically arise in sun-exposed skin of older population. AFX/PDS is essentially diagnosis of exclusion requiring Immunohistochemical work-up to exclude other types of tumors. Case Report: We present a case involving an ulcerated solitary lesion on the scalp of an elderly man. Histological examination revealed that the dermal tumor was composed of large pleomorphic, epithelioid, and spindle cells with clear cytoplasm. These cells were negative for cytokeratins, melanocytes and smooth muscle markers, but positive for CD10. These findings are consistent with a diagnosis of clear cell (CC) PDS. Conclusion: PDS is a low-grade malignancy that can recur locally and metastasize, which is distinguished from AFX by its larger size and the presence of aggressive histopathologic features including deeper invasion into the subcutaneous tissue, tumor necrosis, and lymphovascular and/or perineural involvement. Among several histopathologic variants, the CC variant is extremely rare with only two cases of PDS reported in the literature to date.

Analysis of Sentinel Lymph Node Biopsy on Clear Cell Sarcoma Treatment

Clear Cell Sarcoma (CCS) is a rare soft tissue sarcoma that corresponds to a less than 1% of all sarcomas and is a high degree neoplasm with propensity to disseminate to regional lymph nodes. Regional lymphadenectomies have a controversial impact on sarcomas treatment and survival, even though those patients have higher local recurrence and lower survival. So, sentinel lymph node biopsy must be one option for better regional staging to STS with higher propensity to lymph node commitment. The authors demonstrate two cases of CCS submitted to surgery through compartmental resection associated with the sentinel lymph node biopsy. A literature review about soft tissue sarcoma and lymph node sentinel biopsy is shown as an initial experience of two cases.

Paraplegia after transcatheter artery chemoembolization in a child with clear cell sarcoma of the kidney:A case report

BACKGROUND Transcatheter arterial chemoembolization(TACE)is a common treatment for inoperable malignant renal tumors.However,a series of complications may follow the TACE treatment.Spinal cord injury caused by the embolization of intercostal or lumbar arteries is extremely rare.CASE SUMMARY We describe a case with quite uncommon spinal cord injury after TACE in a 3-year-old child with clear cell sarcoma of the kidney.Sensory impairment beneath the T10 dermatomes and paraplegia on the day after TACE were found in this patient.Unfortunately,sustained paraplegia still existed for more than 2 mo after TACE despite the large dose of steroids and supportive therapy.CONCLUSION We should draw attention to an uncommon complication of paraplegia after TACE treatment in malignant renal tumors.Although it is rare,the result is disastrous.

Primary clear cell sarcoma of soft tissue in the posterior cervical spine invading the medulla oblongata:A case report

BACKGROUND Clear cell sarcoma(CCS)is a rare and highly malignant soft tissue tumor,usually occurring in the deep soft tissues of the distal tendons and aponeurosis of the extremities,especially the feet and knees.CCS originating in the head and neck is extremely rare.The clinical manifestations of CCS in the head and neck are not typical,and the imaging manifestations have certain characteristics,but the diagnosis still depends on pathological examination and genetic testing.CASE SUMMARY A 33-year-old male patient had paroxysmal headache for more than 4 years,accompanied by nausea and vomiting,which could be relieved after rest.Computed tomography angiography showed a left paraspinal soft tissue mass.Contrast-enhanced imaging showed obvious uneven enhancement with adjacent bone lytic destruction.Magnetic resonance imaging examination showed isosignal on T_(1)-weighted images,slightly high signal on T_(2)-weighted images(T_(2)WI),high signal on Tirm fat suppression sequence,significantly high signal on diffusion weighted imaging,and obvious and uneven enhancement.The lesion invaded the anterior medulla oblongata through the left atlantoaxial foramen and compressed the cervical spinal cord on T_(2)WI.Primary CCS of soft tissue was diagnosed by pathology and genetic examination.CONCLUSION CCS should be considered in the differential diagnosis of soft tissue tumors of the head and neck,and their diagnosis depends on pathological examination and genetic testing.

Postoperative multiple metastasis of clear cell sarcoma-like tumor of the gastrointestinal tract in adolescent:A case report

BACKGROUND Clear cell sarcoma-like tumor of the gastrointestinal tract(CCSLGT)is a rare malignant gastrointestinal mesenchymal soft tissue tumor.Its genetic feature is EWSR1 gene rearrangement.Histologically,it is often accompanied by a varying number of CD68-positive osteoclast-like giant cells.CCSLGT mostly occurs in the small intestinal wall of young people and children.In terms of clinical manifestations,there is no significant difference between it and other gastrointestinal tumors,and the diagnosis depends on immunohistochemistry and gene detection.CASE SUMMARY A 16-year-old man developed dizziness and fatigue 2 mo ago,and 10 d ago showed progressive exacerbation of paroxysmal epigastric pain and stopped flatulence and defecation.Computed tomography showed a soft tissue mass in the distal ileum.After complete resection of the lesion,it was diagnosed by combined immunohistochemical and genetic examination as CCSLGT.After surgery,the patient gradually developed lymph node,liver,lung,bone,left thigh,pleura and adrenal metastasis.The survival time was 4 years and 8 mo.CONCLUSION Whole abdominal computed tomography enhancement is recommended for patients with gastrointestinal symptoms.There is no effective treatment for CCSLGT with multiple metastases via the lymphatic system and bloodstream after surgical resection.

Low expression of fatty acid oxidation related gene ACADM indicates poor prognosis of renal clear cell carcinoma and is related to tumor immune infltration

This research aims to identify the key fatty acid beta-oxidation(FAO)genes that are altered in kidney renal clear cell carcinoma(KIRC)and to analyze the role of these genes in KIRC The Gene Expression Omnibus(GEO)and FAO datasets were used to identify these key genes.Wilcoxon rank sum test was used to assess the levels of acyl-CoA dehydrogenase medium chain(ACADM)between KIRC and non cancer samples.The logistic regression and Wilcoxon rank sum test were used to explore the association between ACADM and clinical features.The diagnostic performance of ACADM for KIRC was asessed using a diagnostic receiver operating ch aracteristic(ROC)curve.The co-expressed genes of ACADM were identifed in LinkedOmics database,and their function and pathway enrichment were analyzed.The correlation between ACADM expression level and immune infitration was analyzed by Gene Set Variation Analysis(GSVA)method Additionally,the proliferation,migration,and invasion abilities of KIRC cells were assessed after overexpressing ACADM.Following differential analysis and intersection,we identifed six hub genes,induding ACADM.We found that the expression level of ACADM was decreased in KIRC tissues and had a better diagnostic efect(AUC=0.916).Survival analysis suggested that patients with decreased ACADM expression had a worse prognosis.According to correlation analysis,a variety of dinical features were associated with the expression level of ACADML By analyzing the infiltration level of immune cells,we found that ACADM may be related to the enrichment of immune cells.Finally,ACADM overexpression inhibited proliferation,migration,and invasion of KIRC cells.In conclusion,our findings suggest that reduced ACADM expression in KIRC patients is indicative of poor prognosis.These results imply that ACADM may be a diagnostic and prognostic marker for individuals with KIRC,offering a reference for dinicians in diagnosis and treatment.

Endobronchial metastasis secondary to renal clear cell carcinoma:A case report

BACKGROUND Endobronchial metastases(EBMs)are tumours that metastasise from a malignant tumour outside the lungs to the central and subsegmental bronchi,and are visible under a bronchofibrescope.Most EBMs are formed by direct invasion or metastasis of intrathoracic malignant tumours,such as lung cancer,oesophageal cancer or mediastinum tumours.Renal cell carcinoma(RCC),accounting for 2%to 3%of all tumours,is a common malignant tumour of the urinary system.Renal clear cell carcinoma(RCCC)constitutes the predominant pathological subtype of RCC,comprising approximately 70%to 80%of all RCC cases.RCCC can spread and metastasise through arterial,venous and lymphatic circulation to almost all organs of the body.Moreover,lung,bone,liver,brain and local recurrence are the most common metastatic neoplasms of RCCC.However,EBM from RCCC has a low complication rate and is often misdiagnosed as primary lung cancer.CASE SUMMARY A 71-year-old male patient who had undergone radical left nephrectomy 7 years prior due to RCCC was referred to our hospital due to a 1-mo history of productive cough.The results of an enhanced chest CT scan indicated the presence of a soft tissue nodule in the upper lobe of the left lung,and flexible bronchoscopy revealed a hypervascular lesion in the bronchus of the left lung's superior lobe.Therefore,the patient underwent thoracoscopic left superior lobe wedge resection,and pathology confirmed EBM from the RCCC.CONCLUSION EBM from RCCC has a low incidence and no characteristic clinical manifestations in the early stage.If a bronchial tumour is found in a patient with RCCC,the possibility of bronchial metastatic cancer should be considered.

Left lower lobe sleeve resection for the clear cell variant of pulmonary mucoepidermoid carcinoma:A case report

BACKGROUND Pulmonary mucoepidermoid carcinoma(PMEC)is a rare malignancy that arises from minor salivary glands within the tracheobronchial tree.The clear cell variant of PMEC is exceptionally uncommon and presents notable diagnostic challenges,primarily attributable to its morphological similarity to other tumors containing clear cells.CASE SUMMARY A 22-year-old male,formerly in good health,came in with a two-month duration of persistent cough and production of sputum.Subsequent imaging and bronchoscopy examinations revealed a 2 cm tumor in the distal left main bronchus,which resulted in complete atelectasis of the left lung.Further assessment via positron emission tomography/computed tomography scans and endoscopic biopsy confirmed the primary malignant nature of the tumor,charac-terized by clear cell morphology in most of the tumor cells.The patient underwent a left lower lobe sleeve resection accompanied by systematic mediastinal lymph node dissection.Molecular pathology analysis subsequently revealed a CRTC3-MAML2 gene fusion,leading to a definitive pathological diagnosis of the clear cell variant of PMEC,staged as T2N0M0.After surgery,the patient experienced a smooth recovery and exhibited no signs of recurrence during the one-and-a-half-year follow-up period.CONCLUSION This article describes an unusual case of a clear cell variant of PMEC characterized by the presence of a CRTC3-MAML2 gene fusion in a 22-year-old male.The patient underwent successful left lower lobe sleeve resection.This case underscores the distinctive challenges associated with diagnosing and treating this uncommon malignancy,underscoring the importance of precise diagnosis and personalized treatment strategies.

Vaginal clear cell adenocarcinoma in Herlyn-Werner-Wunderlich syndrome:A case report

BACKGROUND Herlyn-Werner-Wunderlich(HWW)syndrome is a rare Müllerian duct anomaly,characterized by a combination of urogenital abnormalities.The occurrence of primary cervico-vaginal carcinomas in patients with HWW syndrome is excep-tionally rare,posing significant challenges for screening,early diagnosis,and effective management.CASE SUMMARY We report a rare case of primary clear cell carcinoma of the vagina complicated in a 40-year-old woman with HWW syndrome.The patient presented with irregular vaginal bleeding for 4 years.On gynecological examination,an oblique vaginal septum was suspected.Surgical resection of the vaginal septum revealed a com-municating fistula and a tumor on the left vagina and the left side of the septum,which was confirmed as clear cell carcinoma.One month later,she underwent a radical hysterectomy,vaginectomy,bilateral salpingo-oophorectomy,and pelvic lymph node dissection.Due to significant side effects,she completed only one course of chemotherapy.A year later,lung metastasis was detected and continued to grow.A thoracoscopic wedge resection of the right upper lobe was performed 4 years after the initial surgery.We also conducted a systemic review of the lite-rature on primary cervical or vaginal carcinoma in HWW syndrome to explore this rare entity.CONCLUSION Cervico-vaginal adenocarcinomas in patients with HWW syndrome are occult,and require early surgical intervention and regular imaging surveillance.

Network pharmacology and molecular docking analysis reveal insights into the molecular mechanism of Gualou Qumai Wan in clear cell renal cell carcinoma

Background:To initially clarify the potential therapeutic targets and pharmacological mechanism regarding Gualou Qumai Wan(GQW),a kind of traditional Chinese medicine(TCM),in clear cell renal cell carcinoma(ccRCC)by virtue of the network pharmacology analysis and molecular docking analysis.Methods:The screening of bioactive components and targets of GQW was based on the Traditional Chinese Medicine System Pharmacology(TCMSP)and the UniProt platform served for standardizing their targets.Online Mendelian Inheritance in Man(OMIM),PharmGkb,TTD,DrugBank and GeneCards databases were searched to collect the disease targets of ccRCC.Cytoscape assisted in constructing herb-compound-target(H-C-T)networks.The STRING database was searched for constructing the target protein-protein interaction(PPI)networks,while the R programming language served for analyzing GO functional terms and the KEGG pathways related to potential targets.Analyses of core genes related to survival and tumor microenvironment(TME)were conducted respectively based on the GEPIA2 database and TIMER 2.0 database.Human Protein Atlas(HPA)and The Cancer Genome Atlas(TCGA)helped to obtain core genes’protein expression as well as transcriptome expression level.Autodock Vina software validated the molecular docking regarding GQW components and pivotal targets.Results:The constructed H-C-T networks mainly had 33 compounds and 65 targets.A topological analysis of the PPI network identified that ESR1,AKT1,HIF1A,PTGS2,TP53 and VEGFA serve as core targets in the way GQW affects ccRCC.According to the GO and KEGG pathway enrichment analyses,the effects of GQW are mediated by genes related to hypoxia and oxidative stress as well as the Chemical carcinogenesis-receptor activation and PI3K-Akt signaling pathways.AKT1 shows a close relation to the recruitment of various immune cells and can remarkably affect disease prognosis according to reports.Molecular docking and molecular dynamics simulations showed that diosgenin has higher affinity with core targets.Conclusion:The study makes a comprehensive explanation of the biological activity,potential targets,as well as molecular mechanism regarding GQW against ccRCC,which promisingly assists in revealing the action mechanism of TCM formulae in disease treatment and the respective and scientific basis.