BACKGROUND Retroperitoneal liposarcoma(RLPS)is a rare malignant tumor of the connective tissue and usually grows to a large size,undetected.Diagnosis is currently based on collective findings from clinical examination...BACKGROUND Retroperitoneal liposarcoma(RLPS)is a rare malignant tumor of the connective tissue and usually grows to a large size,undetected.Diagnosis is currently based on collective findings from clinical examinations and computed tomography(CT)and magnetic resonance imaging,the latter of which show a fat density mass and possible surrounding organ involvement.Surgical resection is the main therapeutic strategy.The efficacy and safety of further therapeutic choices,such as chemotherapy and radiotherapy,are still controversial.CASE SUMMARY A 61-year-old man presented with complaint of a large left inguinal mass that had appeared suddenly,after a slight exertion.Ultrasonography revealed an omental inguinal hernia.During further clinical examination,an enormous palpable abdominal mass,continuing from the left inguinal location,was observed.CT revealed a giant RLPS,with remarkable mass effect and wide visceral dislocation.After multidisciplinary consultation,surgical intervention was performed.Subsequent neoadjuvant chemotherapy and radiotherapy were precluded by the mass’large size and retroperitoneal localization,features typically associated with non-response to these types of treatment.Instead,the patient underwent conservative treatment via radical surgical excision.After 1 year,his clinical condition remained good,with no radiological signs of recurrence.CONCLUSION Conservative treatment via surgery resulted in a successful outcome for a large RLPS.展开更多
BACKGROUND The classification of uterine sarcomas is based on distinctive morphological and immunophenotypic characteristics,increasingly supported by molecular genetic diagnostics.Data on neurotrophic tyrosine recept...BACKGROUND The classification of uterine sarcomas is based on distinctive morphological and immunophenotypic characteristics,increasingly supported by molecular genetic diagnostics.Data on neurotrophic tyrosine receptor kinase(NTRK)gene fusionpositive uterine sarcoma,potentially aggressive and morphologically similar to fibrosarcoma,are limited due to its recent recognition.Pan-TRK immunohistochemistry(IHC)analysis serves as an effective screening tool with high sensitivity and specificity for NTRK-fusion malignancies.CASE SUMMARY We report a case of a malignant mesenchymal tumor originating from the uterine cervix,which was pan-TRK IHC-positive but lacked NTRK gene fusions,accompanied by a brief literature review.A 55-year-old woman presented to the emergency department with abdominal pain and distension,exhibiting significant ascites and multiple solid pelvic masses.Pelvic examination revealed a tumor encompassing the uterine cervix,extending to the vagina and uterine corpus.A punch biopsy of the cervix indicated NTRK sarcoma with positive immunochemical pan-TRK stain.However,subsequent next generation sequencing revealed no NTRK gene fusion,leading to a diagnosis of poorly differentiated,advanced-stage sarcoma.CONCLUSION The clinical significance of NTRK gene fusion lies in potential treatment with TRK inhibitors for positive sarcomas.Identifying such rare tumors is crucial due to the potential applicability of tropomyosin receptor kinase inhibitor treatment.展开更多
Background: The low-grade fibromyxoid sarcoma (LGFMS) is an exceptionally uncommon sarcoma that primarily manifests in the extremities or trunk of young adults, presenting as painless lesions. The histological feature...Background: The low-grade fibromyxoid sarcoma (LGFMS) is an exceptionally uncommon sarcoma that primarily manifests in the extremities or trunk of young adults, presenting as painless lesions. The histological features of this tumor are benign, but it exhibits an exceptionally high rate of late recurrence and a significant potential for metastasis. Imaging examinations serve as a crucial method for detecting LGFMS, while the definitive diagnosis relies on histopathological assessment. Currently, the primary treatment modality for this neoplasm is surgical resection. Early aggressive surgery with negative margins is a critical factor in mitigating the risk of tumor recurrence and metastasis. The present study presented a case of LGFMS located in the right thigh. The patient underwent a mass resection procedure following an MRI examination. During the telephone follow-up one year post-surgery, despite the absence of an imaging review, the surgical site demonstrated satisfactory recovery with no reported abnormal symptoms. Case Presentation: The patient, a 31-year-old male, presented to our hospital for evaluation of an asymptomatic mass in his right thigh that was incidentally discovered 13 years ago. The MRI showed a well-defined mass measuring 8.2 cm × 6.8 cm × 9.6 cm in the right thigh. The tumor signals exhibit a mixed pattern, characterized by predominantly isointense and hypointense signals on T1-weighted imaging (T1WI), a central area of hyperintensity on T2-weighted imaging (T2WI), and peripheral circular enhancement observed on contrast-enhanced scans. The patient underwent surgical resection. Microscopically, the mass was composed of intricately interwoven fibrous matrix and a distinct mucoid region. The tumor cells exhibited a distinctive arrangement in a swirling or wheel-like pattern, with minimal variation in their karyotypic characteristics. The immunohistochemical examination revealed diffuse and intense MUC4 positivity in the tumor cells. The diagnosis of LGFMS was confirmed by post-operative histopathological examination. Conclusions: The LGFMS is an exceptionally uncommon mesenchymal tumor renowned for its benign histological manifestations and malignant behavior. It is crucial to provide a comprehensive summary of the research findings and thoroughly review the existing literature pertaining to this rare disease.展开更多
BACKGROUND Enhanced recovery after surgery(ERAS)programs provide recommendations for an optimized management of patients undergoing surgery.An ERAS program tailored on surgery for retroperitoneal sarcomas(RPS)may impr...BACKGROUND Enhanced recovery after surgery(ERAS)programs provide recommendations for an optimized management of patients undergoing surgery.An ERAS program tailored on surgery for retroperitoneal sarcomas(RPS)may improve patient outcomes and it has still not been established.AIM To determine how an ERAS program tailored to RPS surgery can be agreed upon,structured,and implemented.METHODS Twenty-five candidate items from existing ERAS programs,potentially relevant for RPS surgery,were identified via literature review and expert input.These were included in a questionnaire refined through cognitive interviews and pilot testing.Expert sarcoma surgeons rated each item’s relevance and feasibility on a 6-point scale.The survey was recirculated after one year.Intra-observer reproducibility,inter-observer concordance,and agreement with the modal value of the most experienced participants were analyzed.RESULTS Thirteen sarcoma surgeons from 6 centers participated in the survey.Although surgeons agreed on several items,their overall concordance was low.After recirculating the survey,the intraobserver reproducibility was low.Interestingly,the median concordance with the reference increased for relevance and decreased for feasibility.CONCLUSION Despite interest in ERAS for RPS,surgeon concordance on item relevance and feasibility remains low,underscoring the need for collaborative efforts toward a standardized,consensus-based protocol.展开更多
BACKGROUND Inferior vena cava(IVC)leiomyosarcomas are rare and aggressive tumors.Complete cure depends on achieving R0 resection,which often requires circumferential resection and reconstruction.Synthetic grafts have ...BACKGROUND Inferior vena cava(IVC)leiomyosarcomas are rare and aggressive tumors.Complete cure depends on achieving R0 resection,which often requires circumferential resection and reconstruction.Synthetic grafts have traditionally been used when venous continuity must be restored.However,the use of cadaveric IVC grafts for reconstruction has not been widely reported.CASE SUMMARY Herein,we present the case of a 64-year-old woman diagnosed with an intrahepatic IVC leiomyosarcoma with local invasion.The patient responded favorably to chemotherapy and subsequently underwent an en bloc right hepatectomy,retrohepatic IVC resection,and reconstruction with an interpositional cadaveric IVC graft.Serial imaging follow-ups until 2 years after the operation showed persistent patency of the graft and no graft-related complications.CONCLUSION Cadaveric IVC grafts are an alternative to synthetic grafts for reconstruction,with acceptable outcomes.Larger,long-term studies are necessary to validate these findings.展开更多
Metastatic peritoneal sarcomatosis most commonly occurs from primary soft tissue sarcomas arising either within the abdomen or extremities. Metastatic peritoneal sarcomatosis from an osteosarcoma is extremely rare;onl...Metastatic peritoneal sarcomatosis most commonly occurs from primary soft tissue sarcomas arising either within the abdomen or extremities. Metastatic peritoneal sarcomatosis from an osteosarcoma is extremely rare;only six cases have previously been reported. We report the first case of metastatic peritoneal sarcomatosis originating from radiation-induced osteoblastic osteosarcoma in a 22-year-old woman who had previously been treated for pelvic Ewing’s sarcoma. Abdominal computed tomography, bone scintigraphy and FDG PET/CT demonstrated extensive finely nodular disseminated peritoneal lesions. Histopathologic examination of these peritoneal lesions revealed osteosarcomatosis. In summary, we describe an unusual case of metastatic peritoneal sar comatosis from secondary osteosarcoma arising in a previously irradiated pelvicEwing’s sarcoma.展开更多
Splenic histiocytic sarcoma(SHS)is a rare,aggressive hematological malignancy with unclear progression and management.Our case illustrates the progression and pathophysiological processes of SHS and provides key data ...Splenic histiocytic sarcoma(SHS)is a rare,aggressive hematological malignancy with unclear progression and management.Our case illustrates the progression and pathophysiological processes of SHS and provides key data for the diagnosis,treatment and management of SHS.A 60-year-old female with incidentally detected splenic mass(6.0 cm×5.7 cm)underwent splenectomy,confirmed as SHS in 2020.Post-op imatinib therapy was given.In 2022,hepatic metastases(2.4 cm×2.9 cm)with pancytopenia led to supportive care.Lesions enlarged to 4.3 cm×2.7 cm,leading to multi-organ failure and death at 33 months.The case was categorized into three distinct stages based on the pathophysiology of SHS:Early-stage splenic tumor growth,mid-stage liver metastasis with hematological abnormalities,and late-stage tumor infiltration leading to multiorgan failure.For SHS,this case highlights the pivotal role of early intervention and the value of personalized treatment strategies.展开更多
Myofibroblastic sarcoma(MS)is a rare malignant soft tissue tumor characterized by myofibroblasts.It most commonly arises in the head and neck region,especially the tongue,with rare occurrences in the limbs.MS exhibits...Myofibroblastic sarcoma(MS)is a rare malignant soft tissue tumor characterized by myofibroblasts.It most commonly arises in the head and neck region,especially the tongue,with rare occurrences in the limbs.MS exhibits varying histopathology,ranging from low-to high-grade,with diverse subtypes showing different clinical behaviors and prognoses.This article reports the first case of high-grade MS in the hand,adding to the limited documentation of this rare condition.Here,we present the case of a 30-year-old healthy female with a year-long history of progressive shortening,mobility loss,and weakness in the first finger of the left hand.Left-hand imaging revealed a lytic,cottony tumor involving the entire first metacarpal.Following surgical resection,which included metatarsal grafting and joint reconstruction,a diagnosis of high-grade MS was confirmed based on histological manifestations and immunohistochemical staining,which was further classified as grade 2 according to the French Federation of Cancer Centers Sarcoma Group system.Postoperative radiotherapy was administered and the patient experienced a successful recovery without graft osteonecrosis.The patient regained 90%mobility and strength,without shortening,after surgical resection and radiotherapy.Six months post-surgery,the patient reported full hand functionality.MS is a rare tumor that infrequently affects bones and is often misdiagnosed owing to its controversial characteristics.The initial treatment should focus on complete resection with negative margins,followed by reconstructive surgery to preserve function.Further case studies are needed to establish standardized surgical treatment protocols.展开更多
Synovial sarcoma is a high-grade soft tissue malignancy characterized by a unique fusion gene known as SS18-SSX.The SS18-SSX fusion protein acts as an oncogenic driver of synovial sarcoma,and it has thus been commonly...Synovial sarcoma is a high-grade soft tissue malignancy characterized by a unique fusion gene known as SS18-SSX.The SS18-SSX fusion protein acts as an oncogenic driver of synovial sarcoma,and it has thus been commonly accepted that disruption of SS18-SSX function represents a therapeutic means of treating synovial sarcoma,but emerging evidence suggests that upon depletion of SS18-SSX,an anti-apoptotic signal surprisingly arises to protect synovial sarcoma cell survival.In this article,we discuss the controversial roles of SS18-SSX’s transcriptional activity in synovial sarcoma biology and outline a synergistic strategy for overcoming the resistance of synovial sarcoma cells to SS18-SSX targeted therapeutics.展开更多
A 63-year-old man was admitted to the hospital with a>1-year history of repeated acid reflux and belching and a 1-month history of an abdominal mass.On admission,the patient was in good condition,and his vital sign...A 63-year-old man was admitted to the hospital with a>1-year history of repeated acid reflux and belching and a 1-month history of an abdominal mass.On admission,the patient was in good condition,and his vital signs were stable.Laboratory examinations revealed no significant abnormalities.展开更多
BACKGROUND Primary renal synovial sarcoma(PRSS)is extremely rare in clinical practice,and most cases are associated with SYT-SSX gene fusion.The PRSS with specific MDM2 gene amplification has not been reported so far....BACKGROUND Primary renal synovial sarcoma(PRSS)is extremely rare in clinical practice,and most cases are associated with SYT-SSX gene fusion.The PRSS with specific MDM2 gene amplification has not been reported so far.Therefore,there is no practical experience regarding the clinical,pathological features and diagnosis and treatment plans for patients of this type.This article reports a case of PRSS with specific MDM2 gene amplification.CASE SUMMARY The patient was preoperatively diagnosed with a malignant tumor of the left kidney(with a high probability of clear cell carcinoma).During the operation,a radical left nephrectomy was performed.The postoperative pathological examination report confirmed synovial sarcoma,and the gene test suggested PRSS with specific MDM2 gene amplification.Forty-eight days after the operation,the patient presented with"abdominal distension and diarrhea"and was found to have a huge metastatic tumor in the original left renal area.The patient died clinically 17 hours after admission due to"multiple organ failure".CONCLUSION PRSS with MDM2 gene amplification has a poorer prognosis,a higher degree of malignancy,and a faster progression,and clinicians need to be highly vigilant.展开更多
Alveolar soft tissue sarcoma is a rare malignant tumor of soft tissue,more common in young women,with deep soft tissues in the limbs and buttocks being the most prevalent sites.There are few reported cases in clinical...Alveolar soft tissue sarcoma is a rare malignant tumor of soft tissue,more common in young women,with deep soft tissues in the limbs and buttocks being the most prevalent sites.There are few reported cases in clinical practice.The clinical manifestations lack specificity and the imaging signs are diverse.This case presents ultrasound,MRI and PET/CT images of alveolar soft tissue sarcoma of the gluteus maximus muscle to enhance readers’understanding and awareness of the imaging signs of this rare disease in order to raise awareness of its diagnosis.The characteristics of this case are summarized and reported in combination with domestic literature.展开更多
Soft tissue sarcomas(STS)are rare malignant tumors originating from mesoder-mal tissues with a poor prognosis,accounting for approximately 1%of all malig-nancies and comprising around 50 distinct subtypes.Conventional...Soft tissue sarcomas(STS)are rare malignant tumors originating from mesoder-mal tissues with a poor prognosis,accounting for approximately 1%of all malig-nancies and comprising around 50 distinct subtypes.Conventional imaging mo-dalities,such as computed tomography(CT)and magnetic resonance imaging(MRI),primarily provide anatomical information,whereas 18F-fluorodeoxyglucose positron emission tomography/CT(18F-FDG PET/CT)integrates functional metabolic and anatomical imaging,serving as a critical complementary tool in the diagnosis and management of STS.This article reviews recent advances in the application of 18F-FDG PET/CT for STS.The advantages of 18F-FDG PET/CT in STS include:(1)Early detection of metabolic activity changes in tumors,partic-ularly when morphological alterations are insignificant;(2)Effective differen-tiation between benign and malignant soft tissue tumors,as well as aiding in distinguishing high-grade from low-grade sarcomas;(3)Identification of occult metastatic lesions,improving staging accuracy,and facilitating restaging in cases of recurrence or metastasis;(4)Utilization of parameters such as maximum stan-dardized uptake value and metabolic tumor volume to assist in tumor grading and prognostic evaluation;and(5)Monitoring treatment response to guide adjust-ments in personalized therapeutic strategies.However,18F-FDG PET/CT has limitations in diagnosis of certain STS subtypes(e.g.,myxoid liposarcoma),detection and biopsy of metastatic lymph nodes,necessitating integration with clinical evaluation and other imaging techniques.18F-FDG PET/CT is poised to play an increasingly vital role in the precision diagnosis and treatment of STS.展开更多
BACKGROUND Kirsten rat sarcoma viral oncogene homolog(KRAS)is a commonly identified oncogenic driver in solid tumors,especially in non-small cell lung cancer.Until recently,KRAS was believed to be impossible to target...BACKGROUND Kirsten rat sarcoma viral oncogene homolog(KRAS)is a commonly identified oncogenic driver in solid tumors,especially in non-small cell lung cancer.Until recently,KRAS was believed to be impossible to target because it lacks adenosine triphosphate-binding domains or other regions that allow specic small-molecule inhibitors to act.In this report,we described using KRAS at glycine 12 to cysteine(G12C)inhibitors as posterior line therapy in a patient with relapsed metastatic lung adenocarcinoma carrying KRAS G12C mutation.CASE SUMMARY A 53-year-old Chinese man was treated with radical surgical resection for lung cancer in June 2014.Re-examination in June 2015 indicated postoperative rec-urrence with metastasis.The patient completed several courses of antitumor therapy,including pemetrexed and nedaplatin,bevacizumab with docetaxel and cisplatin,bevacizumab and pemetrexed,sintilimab and anlotinib,sintilimab and albumin-bound paclitaxel,and cadonilimab and docetaxel.In early May 2023,the patient developed a cough productive of bloody sputum and shortness of breath after exercise.The main adverse reactions associated with KRAS G12C inhibitor therapy were gastrointestinal reactions,which could be alleviated by daily oral ondansetron tablets.CONCLUSION After multiple-line treatment including chemotherapy,targeted therapy,and immunotherapy,disease control was achieved in a case of advanced pulmonary adenocarcinoma carrying the KRAS G12C mutation by mutation-specific inhibitor therapy,and the adverse reactions to the therapy were tolerable.展开更多
Uterine sarcomas (US) are rare mesenchymal tumours accounting approximately for 3%–7% of all uterine cancers. Histologically, US are classified into mesenchymal tumours or mixed epithelial and mesenchymal tumours. Th...Uterine sarcomas (US) are rare mesenchymal tumours accounting approximately for 3%–7% of all uterine cancers. Histologically, US are classified into mesenchymal tumours or mixed epithelial and mesenchymal tumours. The group of mesenchymal tumours includes uterine leiomyosarcoma (uLMS, 65% of cases), endometrial stromal sarcoma (ESS, 21%)– traditionally divided into low grade (LG-ESS) and high grade–undifferentiated uterine sarcoma (5%) and other rare subtypes such as alveolar or embryonal rhabdomyosarcoma. Despite the fact that several drugs demonstrated clinical activity in advanced or metastatic settings, the role of postoperative therapy in US remains controversial. In this review, we have summarised the current state of the art, including the chief trials on adjuvant treatment modalities in US, especially focusing on uLMS, LG-ESS and other rare histotypes.展开更多
Pediatric soft tissue sarcomas are a group of malignant neoplasms arising within embryonic mesenchymal tissues during the process of differentiation into muscle, fascia and fat. The tumors have a biphasic peak for age...Pediatric soft tissue sarcomas are a group of malignant neoplasms arising within embryonic mesenchymal tissues during the process of differentiation into muscle, fascia and fat. The tumors have a biphasic peak for age of incidence. Rhabdomyosarcoma(RMS) is diagnosed more frequently in younger children, whereas adult-type non-RMS soft tissue sarcoma is predominately observed in adolescents. The latter group comprises a variety of rare tumors for which diagnosis can be difficult and typically requires special studies, including immunohistochemistry and molecular genetic analysis. Current management for the majority of pediatric sarcomas is based on the data from large multi-institutional trials, which has led to great improvements in outcomes over recent decades. Although surgery remains the mainstay of treatment, the curative aim cannot be achieved without adjuvant treatment. Pre-treatment staging and risk classification are of prime importance in selecting an effective treatment protocol. Tumor resectability, the response to induction chemotherapy, and radiation generally determine the risk-group, and these factors are functions of tumor site, size and biology. Surgery provides the best choice of local control of small resectable tumors in a favorable site. Radiation therapy is added when surgery leaves residual disease or there is evidence of regional spread. Chemotherapy aims to reduce the risk of relapse and improve overall survival. In addition, upfront chemotherapy reduces the aggressiveness of the required surgery and helps preserve organ function in a number of cases. Long-term survival in low-risk sarcomas is feasible, and the intensity of treatment can be reduced. In high-risk sarcoma, current research is allowing more effective disease control.展开更多
BACKGROUND Clinical workup and treatment guidelines have been published by the National Comprehensive Cancer Network(NCCN)to ensure patients are treated uniformly and appropriately.This study sought to retrospectively...BACKGROUND Clinical workup and treatment guidelines have been published by the National Comprehensive Cancer Network(NCCN)to ensure patients are treated uniformly and appropriately.This study sought to retrospectively review patients with a new diagnosis of sarcoma who were treated in a National Cancer Institute(NCI)designated center and determine compliance rates with guidelines for sarcoma.AIM To evaluate our compliance of NCCN sarcoma guidelines at a major NCI designated center and to report instances of deviation that could be used for future studies to improve patient care.METHODS Data was collected retrospectively as an internal review and quality assessment of 35 newly diagnosed and treated patients.Demographic data were recorded and information concerning whether patients had appropriate imaging,biopsy and management.Variables of interest were expressed as raw numbers and percentages.RESULTS Primary site imaging was obtained in 100%of cases.Chest and full-body imaging were obtained in 97%and 100%of indicated cases,respectively.Tissue was obtained preoperatively in 97%of cases.Imaging was reviewed at multidisciplinary Treatment Planning Conference(TPC)in 97%of cases.Pathology was reviewed in 94%of cases in TPC.Both tumor,node,metastasis staging and plan of care were reviewed in 100%of cases in TPC.Treatment guidelines were followed in 94%of cases reviewed.CONCLUSION This study evaluated the workup and treatment provided by a single NCI designated sarcoma service to a series of patients with pathologies defined with the NCCN sarcoma treatment guidelines.Although adherence to NCCN was reported to be very high future prospective studies are required to investigate whether NCCN guidelines impact patient outcomes.展开更多
Osteosarcoma (OS), chondrosarcoma (CSA), and Ewings sarcoma (ES) are the most common primary malignancies of bone, and are rare diseases. As with all sarcomas, the prognosis of these diseases ultimately depends on the...Osteosarcoma (OS), chondrosarcoma (CSA), and Ewings sarcoma (ES) are the most common primary malignancies of bone, and are rare diseases. As with all sarcomas, the prognosis of these diseases ultimately depends on the presence of metastatic disease. Survival is therefore closely linked with the biology and metastatic potential of a particular bone tumor’s cells. Here we describe a significant correlation of aldehyde dehydrogenase (ALDH) activity and the presence/absence of distant metastases in ten consecutive cases of human bone sarcomas. Additionally, cultured human CSA cells, which are historically chemo- and radio-resistant, may be sensitive to the ALDH inhibitor, disulfiram. While it is premature to draw broad conclusions from such a small series, the importance of ALDH activity and inhibition in the metastatic potential of primary bone sarcomas should be investigated further.展开更多
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 combinat...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.展开更多
The combination of radiotherapy(RT) and function-preserving surgery is the most usual contemporary approach in the management of soft tissue sarcomas(STS). Pre- and postoperative RT result in similar local control rat...The combination of radiotherapy(RT) and function-preserving surgery is the most usual contemporary approach in the management of soft tissue sarcomas(STS). Pre- and postoperative RT result in similar local control rates, as shown by a landmark trial in extremity STS. In this review, the role of RT in the management of extremity STS will be discussed, but STS in other sites,including retroperitoneal STS, will also be addressed. The focus will consider various aspects of RT including strategies to reduce the volume of tissue being irradiated, dose, scheduling, and the possible of omission of RT in selected cases. Finally, technology advances through the use of intensity-modulated radiotherapy(IMRT), image-guided IMRT, intraoperative radiotherapy(IORT)and particle therapy will also be discussed.展开更多
文摘BACKGROUND Retroperitoneal liposarcoma(RLPS)is a rare malignant tumor of the connective tissue and usually grows to a large size,undetected.Diagnosis is currently based on collective findings from clinical examinations and computed tomography(CT)and magnetic resonance imaging,the latter of which show a fat density mass and possible surrounding organ involvement.Surgical resection is the main therapeutic strategy.The efficacy and safety of further therapeutic choices,such as chemotherapy and radiotherapy,are still controversial.CASE SUMMARY A 61-year-old man presented with complaint of a large left inguinal mass that had appeared suddenly,after a slight exertion.Ultrasonography revealed an omental inguinal hernia.During further clinical examination,an enormous palpable abdominal mass,continuing from the left inguinal location,was observed.CT revealed a giant RLPS,with remarkable mass effect and wide visceral dislocation.After multidisciplinary consultation,surgical intervention was performed.Subsequent neoadjuvant chemotherapy and radiotherapy were precluded by the mass’large size and retroperitoneal localization,features typically associated with non-response to these types of treatment.Instead,the patient underwent conservative treatment via radical surgical excision.After 1 year,his clinical condition remained good,with no radiological signs of recurrence.CONCLUSION Conservative treatment via surgery resulted in a successful outcome for a large RLPS.
基金Supported by Grant of the Korea Health Technology R&D Project through the Korea Health Industry Development Institute,funded by the Ministry of Health&Welfare,Republic of Korea,No.RS-2022-KH129889.
文摘BACKGROUND The classification of uterine sarcomas is based on distinctive morphological and immunophenotypic characteristics,increasingly supported by molecular genetic diagnostics.Data on neurotrophic tyrosine receptor kinase(NTRK)gene fusionpositive uterine sarcoma,potentially aggressive and morphologically similar to fibrosarcoma,are limited due to its recent recognition.Pan-TRK immunohistochemistry(IHC)analysis serves as an effective screening tool with high sensitivity and specificity for NTRK-fusion malignancies.CASE SUMMARY We report a case of a malignant mesenchymal tumor originating from the uterine cervix,which was pan-TRK IHC-positive but lacked NTRK gene fusions,accompanied by a brief literature review.A 55-year-old woman presented to the emergency department with abdominal pain and distension,exhibiting significant ascites and multiple solid pelvic masses.Pelvic examination revealed a tumor encompassing the uterine cervix,extending to the vagina and uterine corpus.A punch biopsy of the cervix indicated NTRK sarcoma with positive immunochemical pan-TRK stain.However,subsequent next generation sequencing revealed no NTRK gene fusion,leading to a diagnosis of poorly differentiated,advanced-stage sarcoma.CONCLUSION The clinical significance of NTRK gene fusion lies in potential treatment with TRK inhibitors for positive sarcomas.Identifying such rare tumors is crucial due to the potential applicability of tropomyosin receptor kinase inhibitor treatment.
文摘Background: The low-grade fibromyxoid sarcoma (LGFMS) is an exceptionally uncommon sarcoma that primarily manifests in the extremities or trunk of young adults, presenting as painless lesions. The histological features of this tumor are benign, but it exhibits an exceptionally high rate of late recurrence and a significant potential for metastasis. Imaging examinations serve as a crucial method for detecting LGFMS, while the definitive diagnosis relies on histopathological assessment. Currently, the primary treatment modality for this neoplasm is surgical resection. Early aggressive surgery with negative margins is a critical factor in mitigating the risk of tumor recurrence and metastasis. The present study presented a case of LGFMS located in the right thigh. The patient underwent a mass resection procedure following an MRI examination. During the telephone follow-up one year post-surgery, despite the absence of an imaging review, the surgical site demonstrated satisfactory recovery with no reported abnormal symptoms. Case Presentation: The patient, a 31-year-old male, presented to our hospital for evaluation of an asymptomatic mass in his right thigh that was incidentally discovered 13 years ago. The MRI showed a well-defined mass measuring 8.2 cm × 6.8 cm × 9.6 cm in the right thigh. The tumor signals exhibit a mixed pattern, characterized by predominantly isointense and hypointense signals on T1-weighted imaging (T1WI), a central area of hyperintensity on T2-weighted imaging (T2WI), and peripheral circular enhancement observed on contrast-enhanced scans. The patient underwent surgical resection. Microscopically, the mass was composed of intricately interwoven fibrous matrix and a distinct mucoid region. The tumor cells exhibited a distinctive arrangement in a swirling or wheel-like pattern, with minimal variation in their karyotypic characteristics. The immunohistochemical examination revealed diffuse and intense MUC4 positivity in the tumor cells. The diagnosis of LGFMS was confirmed by post-operative histopathological examination. Conclusions: The LGFMS is an exceptionally uncommon mesenchymal tumor renowned for its benign histological manifestations and malignant behavior. It is crucial to provide a comprehensive summary of the research findings and thoroughly review the existing literature pertaining to this rare disease.
文摘BACKGROUND Enhanced recovery after surgery(ERAS)programs provide recommendations for an optimized management of patients undergoing surgery.An ERAS program tailored on surgery for retroperitoneal sarcomas(RPS)may improve patient outcomes and it has still not been established.AIM To determine how an ERAS program tailored to RPS surgery can be agreed upon,structured,and implemented.METHODS Twenty-five candidate items from existing ERAS programs,potentially relevant for RPS surgery,were identified via literature review and expert input.These were included in a questionnaire refined through cognitive interviews and pilot testing.Expert sarcoma surgeons rated each item’s relevance and feasibility on a 6-point scale.The survey was recirculated after one year.Intra-observer reproducibility,inter-observer concordance,and agreement with the modal value of the most experienced participants were analyzed.RESULTS Thirteen sarcoma surgeons from 6 centers participated in the survey.Although surgeons agreed on several items,their overall concordance was low.After recirculating the survey,the intraobserver reproducibility was low.Interestingly,the median concordance with the reference increased for relevance and decreased for feasibility.CONCLUSION Despite interest in ERAS for RPS,surgeon concordance on item relevance and feasibility remains low,underscoring the need for collaborative efforts toward a standardized,consensus-based protocol.
文摘BACKGROUND Inferior vena cava(IVC)leiomyosarcomas are rare and aggressive tumors.Complete cure depends on achieving R0 resection,which often requires circumferential resection and reconstruction.Synthetic grafts have traditionally been used when venous continuity must be restored.However,the use of cadaveric IVC grafts for reconstruction has not been widely reported.CASE SUMMARY Herein,we present the case of a 64-year-old woman diagnosed with an intrahepatic IVC leiomyosarcoma with local invasion.The patient responded favorably to chemotherapy and subsequently underwent an en bloc right hepatectomy,retrohepatic IVC resection,and reconstruction with an interpositional cadaveric IVC graft.Serial imaging follow-ups until 2 years after the operation showed persistent patency of the graft and no graft-related complications.CONCLUSION Cadaveric IVC grafts are an alternative to synthetic grafts for reconstruction,with acceptable outcomes.Larger,long-term studies are necessary to validate these findings.
文摘Metastatic peritoneal sarcomatosis most commonly occurs from primary soft tissue sarcomas arising either within the abdomen or extremities. Metastatic peritoneal sarcomatosis from an osteosarcoma is extremely rare;only six cases have previously been reported. We report the first case of metastatic peritoneal sarcomatosis originating from radiation-induced osteoblastic osteosarcoma in a 22-year-old woman who had previously been treated for pelvic Ewing’s sarcoma. Abdominal computed tomography, bone scintigraphy and FDG PET/CT demonstrated extensive finely nodular disseminated peritoneal lesions. Histopathologic examination of these peritoneal lesions revealed osteosarcomatosis. In summary, we describe an unusual case of metastatic peritoneal sar comatosis from secondary osteosarcoma arising in a previously irradiated pelvicEwing’s sarcoma.
基金Supported by the Program of General Hospital of Western Theater,No.2021-XZYG-C33.
文摘Splenic histiocytic sarcoma(SHS)is a rare,aggressive hematological malignancy with unclear progression and management.Our case illustrates the progression and pathophysiological processes of SHS and provides key data for the diagnosis,treatment and management of SHS.A 60-year-old female with incidentally detected splenic mass(6.0 cm×5.7 cm)underwent splenectomy,confirmed as SHS in 2020.Post-op imatinib therapy was given.In 2022,hepatic metastases(2.4 cm×2.9 cm)with pancytopenia led to supportive care.Lesions enlarged to 4.3 cm×2.7 cm,leading to multi-organ failure and death at 33 months.The case was categorized into three distinct stages based on the pathophysiology of SHS:Early-stage splenic tumor growth,mid-stage liver metastasis with hematological abnormalities,and late-stage tumor infiltration leading to multiorgan failure.For SHS,this case highlights the pivotal role of early intervention and the value of personalized treatment strategies.
文摘Myofibroblastic sarcoma(MS)is a rare malignant soft tissue tumor characterized by myofibroblasts.It most commonly arises in the head and neck region,especially the tongue,with rare occurrences in the limbs.MS exhibits varying histopathology,ranging from low-to high-grade,with diverse subtypes showing different clinical behaviors and prognoses.This article reports the first case of high-grade MS in the hand,adding to the limited documentation of this rare condition.Here,we present the case of a 30-year-old healthy female with a year-long history of progressive shortening,mobility loss,and weakness in the first finger of the left hand.Left-hand imaging revealed a lytic,cottony tumor involving the entire first metacarpal.Following surgical resection,which included metatarsal grafting and joint reconstruction,a diagnosis of high-grade MS was confirmed based on histological manifestations and immunohistochemical staining,which was further classified as grade 2 according to the French Federation of Cancer Centers Sarcoma Group system.Postoperative radiotherapy was administered and the patient experienced a successful recovery without graft osteonecrosis.The patient regained 90%mobility and strength,without shortening,after surgical resection and radiotherapy.Six months post-surgery,the patient reported full hand functionality.MS is a rare tumor that infrequently affects bones and is often misdiagnosed owing to its controversial characteristics.The initial treatment should focus on complete resection with negative margins,followed by reconstructive surgery to preserve function.Further case studies are needed to establish standardized surgical treatment protocols.
文摘Synovial sarcoma is a high-grade soft tissue malignancy characterized by a unique fusion gene known as SS18-SSX.The SS18-SSX fusion protein acts as an oncogenic driver of synovial sarcoma,and it has thus been commonly accepted that disruption of SS18-SSX function represents a therapeutic means of treating synovial sarcoma,but emerging evidence suggests that upon depletion of SS18-SSX,an anti-apoptotic signal surprisingly arises to protect synovial sarcoma cell survival.In this article,we discuss the controversial roles of SS18-SSX’s transcriptional activity in synovial sarcoma biology and outline a synergistic strategy for overcoming the resistance of synovial sarcoma cells to SS18-SSX targeted therapeutics.
基金supported by the National Natural Science Foundation of China(Project No.82160348)the Yunnan Province Major Special Plan(No.202302AA310018-D-8)+1 种基金the Youth Talent Project of Yunnan Province's“Xingdian Talent Support Program”(No.XDYC-QNRC-2022-0608)the 2024 Senior Health Technology and Medical Discipline Leader of Yunnan Provincial Health Commission(No.D-2024056).
文摘A 63-year-old man was admitted to the hospital with a>1-year history of repeated acid reflux and belching and a 1-month history of an abdominal mass.On admission,the patient was in good condition,and his vital signs were stable.Laboratory examinations revealed no significant abnormalities.
基金Supported by The Joint Special Project of Universities in Yunnan Province in 2022,No.2022-ES-530101-207The Hospital-level Scientific Research Project of The Fourth Affiliated Hospital of Dali University,No.2024YJ14.
文摘BACKGROUND Primary renal synovial sarcoma(PRSS)is extremely rare in clinical practice,and most cases are associated with SYT-SSX gene fusion.The PRSS with specific MDM2 gene amplification has not been reported so far.Therefore,there is no practical experience regarding the clinical,pathological features and diagnosis and treatment plans for patients of this type.This article reports a case of PRSS with specific MDM2 gene amplification.CASE SUMMARY The patient was preoperatively diagnosed with a malignant tumor of the left kidney(with a high probability of clear cell carcinoma).During the operation,a radical left nephrectomy was performed.The postoperative pathological examination report confirmed synovial sarcoma,and the gene test suggested PRSS with specific MDM2 gene amplification.Forty-eight days after the operation,the patient presented with"abdominal distension and diarrhea"and was found to have a huge metastatic tumor in the original left renal area.The patient died clinically 17 hours after admission due to"multiple organ failure".CONCLUSION PRSS with MDM2 gene amplification has a poorer prognosis,a higher degree of malignancy,and a faster progression,and clinicians need to be highly vigilant.
文摘Alveolar soft tissue sarcoma is a rare malignant tumor of soft tissue,more common in young women,with deep soft tissues in the limbs and buttocks being the most prevalent sites.There are few reported cases in clinical practice.The clinical manifestations lack specificity and the imaging signs are diverse.This case presents ultrasound,MRI and PET/CT images of alveolar soft tissue sarcoma of the gluteus maximus muscle to enhance readers’understanding and awareness of the imaging signs of this rare disease in order to raise awareness of its diagnosis.The characteristics of this case are summarized and reported in combination with domestic literature.
文摘Soft tissue sarcomas(STS)are rare malignant tumors originating from mesoder-mal tissues with a poor prognosis,accounting for approximately 1%of all malig-nancies and comprising around 50 distinct subtypes.Conventional imaging mo-dalities,such as computed tomography(CT)and magnetic resonance imaging(MRI),primarily provide anatomical information,whereas 18F-fluorodeoxyglucose positron emission tomography/CT(18F-FDG PET/CT)integrates functional metabolic and anatomical imaging,serving as a critical complementary tool in the diagnosis and management of STS.This article reviews recent advances in the application of 18F-FDG PET/CT for STS.The advantages of 18F-FDG PET/CT in STS include:(1)Early detection of metabolic activity changes in tumors,partic-ularly when morphological alterations are insignificant;(2)Effective differen-tiation between benign and malignant soft tissue tumors,as well as aiding in distinguishing high-grade from low-grade sarcomas;(3)Identification of occult metastatic lesions,improving staging accuracy,and facilitating restaging in cases of recurrence or metastasis;(4)Utilization of parameters such as maximum stan-dardized uptake value and metabolic tumor volume to assist in tumor grading and prognostic evaluation;and(5)Monitoring treatment response to guide adjust-ments in personalized therapeutic strategies.However,18F-FDG PET/CT has limitations in diagnosis of certain STS subtypes(e.g.,myxoid liposarcoma),detection and biopsy of metastatic lymph nodes,necessitating integration with clinical evaluation and other imaging techniques.18F-FDG PET/CT is poised to play an increasingly vital role in the precision diagnosis and treatment of STS.
基金Supported by National Natural Sciences Foundation of China,No.81803553.
文摘BACKGROUND Kirsten rat sarcoma viral oncogene homolog(KRAS)is a commonly identified oncogenic driver in solid tumors,especially in non-small cell lung cancer.Until recently,KRAS was believed to be impossible to target because it lacks adenosine triphosphate-binding domains or other regions that allow specic small-molecule inhibitors to act.In this report,we described using KRAS at glycine 12 to cysteine(G12C)inhibitors as posterior line therapy in a patient with relapsed metastatic lung adenocarcinoma carrying KRAS G12C mutation.CASE SUMMARY A 53-year-old Chinese man was treated with radical surgical resection for lung cancer in June 2014.Re-examination in June 2015 indicated postoperative rec-urrence with metastasis.The patient completed several courses of antitumor therapy,including pemetrexed and nedaplatin,bevacizumab with docetaxel and cisplatin,bevacizumab and pemetrexed,sintilimab and anlotinib,sintilimab and albumin-bound paclitaxel,and cadonilimab and docetaxel.In early May 2023,the patient developed a cough productive of bloody sputum and shortness of breath after exercise.The main adverse reactions associated with KRAS G12C inhibitor therapy were gastrointestinal reactions,which could be alleviated by daily oral ondansetron tablets.CONCLUSION After multiple-line treatment including chemotherapy,targeted therapy,and immunotherapy,disease control was achieved in a case of advanced pulmonary adenocarcinoma carrying the KRAS G12C mutation by mutation-specific inhibitor therapy,and the adverse reactions to the therapy were tolerable.
文摘Uterine sarcomas (US) are rare mesenchymal tumours accounting approximately for 3%–7% of all uterine cancers. Histologically, US are classified into mesenchymal tumours or mixed epithelial and mesenchymal tumours. The group of mesenchymal tumours includes uterine leiomyosarcoma (uLMS, 65% of cases), endometrial stromal sarcoma (ESS, 21%)– traditionally divided into low grade (LG-ESS) and high grade–undifferentiated uterine sarcoma (5%) and other rare subtypes such as alveolar or embryonal rhabdomyosarcoma. Despite the fact that several drugs demonstrated clinical activity in advanced or metastatic settings, the role of postoperative therapy in US remains controversial. In this review, we have summarised the current state of the art, including the chief trials on adjuvant treatment modalities in US, especially focusing on uLMS, LG-ESS and other rare histotypes.
文摘Pediatric soft tissue sarcomas are a group of malignant neoplasms arising within embryonic mesenchymal tissues during the process of differentiation into muscle, fascia and fat. The tumors have a biphasic peak for age of incidence. Rhabdomyosarcoma(RMS) is diagnosed more frequently in younger children, whereas adult-type non-RMS soft tissue sarcoma is predominately observed in adolescents. The latter group comprises a variety of rare tumors for which diagnosis can be difficult and typically requires special studies, including immunohistochemistry and molecular genetic analysis. Current management for the majority of pediatric sarcomas is based on the data from large multi-institutional trials, which has led to great improvements in outcomes over recent decades. Although surgery remains the mainstay of treatment, the curative aim cannot be achieved without adjuvant treatment. Pre-treatment staging and risk classification are of prime importance in selecting an effective treatment protocol. Tumor resectability, the response to induction chemotherapy, and radiation generally determine the risk-group, and these factors are functions of tumor site, size and biology. Surgery provides the best choice of local control of small resectable tumors in a favorable site. Radiation therapy is added when surgery leaves residual disease or there is evidence of regional spread. Chemotherapy aims to reduce the risk of relapse and improve overall survival. In addition, upfront chemotherapy reduces the aggressiveness of the required surgery and helps preserve organ function in a number of cases. Long-term survival in low-risk sarcomas is feasible, and the intensity of treatment can be reduced. In high-risk sarcoma, current research is allowing more effective disease control.
文摘BACKGROUND Clinical workup and treatment guidelines have been published by the National Comprehensive Cancer Network(NCCN)to ensure patients are treated uniformly and appropriately.This study sought to retrospectively review patients with a new diagnosis of sarcoma who were treated in a National Cancer Institute(NCI)designated center and determine compliance rates with guidelines for sarcoma.AIM To evaluate our compliance of NCCN sarcoma guidelines at a major NCI designated center and to report instances of deviation that could be used for future studies to improve patient care.METHODS Data was collected retrospectively as an internal review and quality assessment of 35 newly diagnosed and treated patients.Demographic data were recorded and information concerning whether patients had appropriate imaging,biopsy and management.Variables of interest were expressed as raw numbers and percentages.RESULTS Primary site imaging was obtained in 100%of cases.Chest and full-body imaging were obtained in 97%and 100%of indicated cases,respectively.Tissue was obtained preoperatively in 97%of cases.Imaging was reviewed at multidisciplinary Treatment Planning Conference(TPC)in 97%of cases.Pathology was reviewed in 94%of cases in TPC.Both tumor,node,metastasis staging and plan of care were reviewed in 100%of cases in TPC.Treatment guidelines were followed in 94%of cases reviewed.CONCLUSION This study evaluated the workup and treatment provided by a single NCI designated sarcoma service to a series of patients with pathologies defined with the NCCN sarcoma treatment guidelines.Although adherence to NCCN was reported to be very high future prospective studies are required to investigate whether NCCN guidelines impact patient outcomes.
文摘Osteosarcoma (OS), chondrosarcoma (CSA), and Ewings sarcoma (ES) are the most common primary malignancies of bone, and are rare diseases. As with all sarcomas, the prognosis of these diseases ultimately depends on the presence of metastatic disease. Survival is therefore closely linked with the biology and metastatic potential of a particular bone tumor’s cells. Here we describe a significant correlation of aldehyde dehydrogenase (ALDH) activity and the presence/absence of distant metastases in ten consecutive cases of human bone sarcomas. Additionally, cultured human CSA cells, which are historically chemo- and radio-resistant, may be sensitive to the ALDH inhibitor, disulfiram. While it is premature to draw broad conclusions from such a small series, the importance of ALDH activity and inhibition in the metastatic potential of primary bone sarcomas should be investigated further.
文摘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.
文摘The combination of radiotherapy(RT) and function-preserving surgery is the most usual contemporary approach in the management of soft tissue sarcomas(STS). Pre- and postoperative RT result in similar local control rates, as shown by a landmark trial in extremity STS. In this review, the role of RT in the management of extremity STS will be discussed, but STS in other sites,including retroperitoneal STS, will also be addressed. The focus will consider various aspects of RT including strategies to reduce the volume of tissue being irradiated, dose, scheduling, and the possible of omission of RT in selected cases. Finally, technology advances through the use of intensity-modulated radiotherapy(IMRT), image-guided IMRT, intraoperative radiotherapy(IORT)and particle therapy will also be discussed.
