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
BACKGROUND Low-grade myofibroblastic sarcoma(LGMS)is an extremely rare tumor characterized by the malignant proliferation of myofibroblasts.LGMS most commonly develops in adults,predominantly in males,in the head and ...BACKGROUND Low-grade myofibroblastic sarcoma(LGMS)is an extremely rare tumor characterized by the malignant proliferation of myofibroblasts.LGMS most commonly develops in adults,predominantly in males,in the head and neck region,oral cavity,especially on the tongue,mandible,and larynx.This article presents 2 cases of LGMS localized to the maxillary sinus and provides an overview of the available literature.CASE SUMMARY Two patients with LGMS located in the maxillary sinus underwent surgery at the Department of Head and Neck Surgery.Case 1:A 46-year-old patient was admitted to the clinic with suspected LGMS recurrence in the right maxillary sinus(rT4aN0M0),with symptoms of pain in the suborbital area,watering of the right eye,thick discharge from the right nostril,and augmented facial asymmetry.After open biopsy-confirmed LGMS,the patient underwent expanded maxillectomy of the right side with immediate palate reconstruction using a microvascular skin flap harvested surgically from the middle arm.The patient qualified for adjuvant radiotherapy for the postoperative bed,with an additional margin.Currently,the patient is under 1.5 years of observation with no evidence of disease.Case 2:A 45-year-old man was admitted to our clinic with facial asymmetry,strabismus,exophthalmos,and visual impairment in the right eye.Six months earlier,the patient had undergone partial jaw resection at another hospital for fibromatosis.A contrast-enhanced computed tomography scan revealed a tumor mass in the postoperative log after an earlier procedure.An open biopsy confirmed lowgrade fibrosarcoma(rT4aN0M0).The patient qualified for an extended total right maxillectomy with orbital excision and right hemimandibulectomy with immediate microvascular reconstruction using an anterolateral thigh flap.The patient subsequently underwent adjuvant radiotherapy to the postoperative area.After 9 months,recurrence occurred in the right mandibular arch below the irradiated area.The lesion infiltrated the base of the skull,which warranted the withdrawal of radiotherapy and salvage surgery.The patient qualified for palliative chemotherapy with a regimen of doxorubicin+dacarbazine+cyclophosphamide and palliative radiotherapy for bone metastases.The patient died 26 months after surgical treatment.The cases have been assessed and compared with cases in the literature.CONCLUSION No specific diagnostic criteria or treatment strategies have been developed for LGMS.The treatment used for LGMS is the same as that used for sinonasal cancer radical tumor excision;adjuvant radiotherapy or chemoradiotherapy should also be considered.They have low malignant potential but are highly invasive,tend to recur,and metastasize to distant sites.Patients should undergo regular follow-up examinations to detect recurrence or metastasis at an early stage.Patients should be treated and observed at the highest referral centers.展开更多
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 an...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.展开更多
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 follo...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.展开更多
Introduction: Kaposi sarcoma disease is a proliferative and multifocal disorder with dual components, vascular and fibroblastic cellular, cutaneous and visceral expression. Kaposi Sarcoma can affect the ocular surface...Introduction: Kaposi sarcoma disease is a proliferative and multifocal disorder with dual components, vascular and fibroblastic cellular, cutaneous and visceral expression. Kaposi Sarcoma can affect the ocular surface and adnexa and can masquerade as other entities, delaying prompt diagnosis can lead to diagnostic wandering delaying treatment. Our aim is to describe a case of KS of the eyelid in an HIV seronegative patient. Case Presentation: A seventy-year-old man developed a bilateral growing tumoral reddish purple vascular mass on both the lower and upper eyelid involving rapidly for 6 months. Both feet and the two shanks show the presence of a brown-violet tumor-shaped formation. The patient was negative for HIV. Histology examination showed a nodular tumor-like mass with a fibro hemangioma-epitheliomatous. Polymerase chain reaction was positive for human herpes virus 8. Initial chemotherapy followed by surgery was proposed to the patient. Unfortunately, the patient rejected treatment and was lost to follow-up. Conclusion: This case reports the difficulty of managing KS in developing countries.展开更多
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 diagnos...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.展开更多
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
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 pr...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.展开更多
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 c...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.展开更多
Purpose: Development of sarcoma is a known late rare negative side effect of radiotherapy. We add two cases to emphasize the need for open-end follow-up and critical evaluation to avoid misinterpretation. Patients, Me...Purpose: Development of sarcoma is a known late rare negative side effect of radiotherapy. We add two cases to emphasize the need for open-end follow-up and critical evaluation to avoid misinterpretation. Patients, Methods, and Results: Two patients developed osteosarcoma as a second malignancy in the humerus after adjuvant radiotherapy of a primary tumor not directly involving the later affected bone. The first patient had a Ewing sarcoma of the scapula at age 13 years. Though after neoadjuvant chemotherapy the resected specimen showed only fibrotic necrotic areas within clear resection margins, the study group indicated adjuvant radiotherapy in a field including the shoulder joint. At age 24 years she developed an osteosarcoma of the humeral head, which was resected and reconstructed with a proximal humerus endoprosthesis. She is alive without disease at age 32 years. The second patient presented with an osteosarcoma of the proximal humerus 29 years after irradiation for breast cancer including the shoulder joint. The sarcoma was misinterpreted as radiation-induced necrosis and the patient was treated with a reverse shoulder endoprosthesis. Pathologic examination of the resected humeral head then showed a typical osteosarcoma. Two years later the humeral reverse shoulder implant was resected and a proximal humerus tumor prosthesis implanted leaving the original glenosphere. Conclusions: In both cases radiation-induced osteosarcoma developed in bone not affected by the primary cancer. Protecting uninvolved structures must be warranted in the planning of radiotherapy. The long latency between the primary and second cancer mandates long-term—best indefinite—follow-up, as with appropriate treatment of a radiation-induced osteosarcoma good healing rates comparable to those of primary osteosarcoma can still be achieved.展开更多
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...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.展开更多
BACKGROUND Low-grade myofibroblastic sarcoma(LGMS)is a rare spindle cell sarcoma espe-cially in the pancreas,with myofibroblastic differentiation.Hitherto,only a few cases have been reported.CASE SUMMARY Herein,we rep...BACKGROUND Low-grade myofibroblastic sarcoma(LGMS)is a rare spindle cell sarcoma espe-cially in the pancreas,with myofibroblastic differentiation.Hitherto,only a few cases have been reported.CASE SUMMARY Herein,we report a case involving the discovery of a pancreatic mass detected during a routine physical examination.Subsequent imaging and pathological tests of the patient led to the diagnosis of LGMS of the pancreas.Following surgical intervention,the patient experienced recurrence and metastasis.Conventional treatment is not effective for postoperative recurrent pancreatic LGMS with multiple metastases.After communicating with the patients and their families,informed consent was obtained for the treatment of anlotinib combined with pembrolizumab.Evaluation of imaging and clinical symptoms post-treatment revealed a relatively favorable response to the combination of anlotinib and pembrolizumab.CONCLUSION Based on the comprehensive literature review,our report aimed to provide evidence for a better understanding of the disease characteristics,diagnostic criteria,imaging findings,and identification of LGMS.And explore novel treatment strategies for this disease.展开更多
BACKGROUND Extraskeletal Ewing sarcoma(EES)is a rare and highly malignant small round cell tumor associated with a poor clinical outcome.Ewing sarcoma(ES)involving the stomach is an uncommon presentation and can be ea...BACKGROUND Extraskeletal Ewing sarcoma(EES)is a rare and highly malignant small round cell tumor associated with a poor clinical outcome.Ewing sarcoma(ES)involving the stomach is an uncommon presentation and can be easily confused with other small round cell tumors.We herein present a rare case of ES involving the gastric area.CASE SUMMARY We report a case of gastric ES in a 19-year-old female patient who initially presented with a complaint of a tender epigastric mass for 5 d.Contrast-enhanced abdominal computed tomography revealed a soft-tissue-density mass with a diameter of 8.5 cm between the liver and stomach;the mass was connected to the gastric antrum.Then,the mass was surgically excised completely.Upon histopathological,immunophenotype and molecular analysis,the mass was identified to be a primary gastric ES.CONCLUSION EES is an aggressive tumor with poor prognosis.Therefore,early diagnosis and timely intervention are essential for a good prognosis.It is imperative for us to raise awareness about this rare tumor.Surgical resection is still the best treatment option.展开更多
Undifferentiated pleomorphic sarcoma is an extremely rare malignant thyroid tumor.Thyroid sarcoma differs from common malignant thyroid tumors,such as thyroid follicular cell carcinoma.It is usually highly malignant,p...Undifferentiated pleomorphic sarcoma is an extremely rare malignant thyroid tumor.Thyroid sarcoma differs from common malignant thyroid tumors,such as thyroid follicular cell carcinoma.It is usually highly malignant,progresses rapidly,and is prone to remote metastasis.Currently,there is no standard protocol for the treatment of thyroid sarcomas,and most treatment effects are unsatisfactory.Argon-helium cryoablation is an important method of local treatment that is widely used in patients with unresectable advanced tumors.However,owing to the low incidence of thyroid sarcomas,there are no relevant literature reports on the treatment of thyroid sarcomas using cryoablation in China.This study reports the case of a patient with undifferentiated pleomorphic sarcoma of the thyroid gland who was treated with argon-helium cryoablation,and the immediate outcome was good.Based on a review of relevant literature,we discussed the effectiveness and safety of argon-helium cryoablation treatment,to provide clinical guidance and references for the treatment of patients with thyroid sarcoma.展开更多
BACKGROUND The mixed lineage leukemia(MLL)-eleven-nineteen lysine-rich leukemia(ELL)fusion gene is a rare occurrence among the various MLL fusion genes.We present the first case in which myeloid sarcoma(MS)was the onl...BACKGROUND The mixed lineage leukemia(MLL)-eleven-nineteen lysine-rich leukemia(ELL)fusion gene is a rare occurrence among the various MLL fusion genes.We present the first case in which myeloid sarcoma(MS)was the only manifestation of adult MLL-ELL-positive acute myeloid leukemia(AML).CASE SUMMARY We report a case of a 33-year-old male patient who was admitted in June 2022 with a right occipital area mass measuring approximately 7 cm×8 cm.Blood work was normal.The patient underwent right occipital giant subscalp mass excision and incisional flap grafting.Immunohistochemistry was positive for myeloperoxidase,CD43 and CD45 and negative for CD3,CD20,CD34,and CD56.The bone marrow aspirate showed hypercellularity with 20%myeloblasts.Flow cytometry showed that myeloblasts accounted for 27.21%of the nucleated cells,which expressed CD33,CD38,and CD117.The karyotype was 46,XY,t(11,19)(q23;p13.1),-12,+mar/46,XY.Next-generation sequencing showed a fusion of MLL exon 7 to exon 2 of ELL.A diagnosis of MLL-ELL-positive AML(M2 subtype)with subcutaneous MS was made.CONCLUSION MLL-ELL-positive AML with MS is a rare clinical entity.Additional research is needed to elucidate the molecular mechanisms of the pathogenesis of MS.展开更多
BACKGROUND Ewing sarcoma(ES)is a malignant neoplasm of neuroectodermal origin and is commonly observed in children and young adults.The musculoskeletal system is the main body system impacted and ES is rarely seen in ...BACKGROUND Ewing sarcoma(ES)is a malignant neoplasm of neuroectodermal origin and is commonly observed in children and young adults.The musculoskeletal system is the main body system impacted and ES is rarely seen in the visceral organs particularly the adrenal gland.AIM To present a comprehensive review of primary adrenal ES,with emphasis on diagnosis,therapy and oncological outcomes.METHODS A systematic review of the literature was performed according to the Preferred Reporting Items for Systematic reviews and Meta-Analyses 2020.PubMed/MEDLINE,EMBASE and Google Scholar bibliographic databases were searched to identify articles from 1989 to 2022 and included patients with ES/primitive neuroectodermal tumor(PNET)of the adrenal gland.PubMed,Google Scholar and EMBASE medical databases were searched,combining the terms“adrenal”,“ES”and“PNET”.Demographic,clinical,pathological and oncological data of patients were analyzed by SPSS version 29.0.RESULTS A total of 52 studies were included for review(47 case reports and 5 case series)with 66 patients reported to have primary adrenal ES.Mean age at diagnosis was 26.4±15.4 years(37.9%males,57.6%females,sex not reported in 3 cases).The most frequent complaint was abdominal/flank pain or discomfort(46.4%)followed by a palpable mass(25.0%),and the average duration of symptoms was 2.6±3.1 mo.The imaging modality of choice was computed tomography scan(81.5%),followed by magnetic resonance imaging(20.4%).Preoperative staging revealed that 17 tumors(27.9%)were metastatic and 14 patients had inferior vena cava or renal vein neoplastic thrombus at initial diagnosis.Open adrenalectomy was performed in the majority of cases(80.0%),of which 27.9%required more extensive resection.Minimally invasive surgery was attempted in 8.2%of tumors.Complete surgical resection was achieved in 89.4%of the patients.Adjuvant therapy was administered to 32 patients,in the form of chemotherapy(62.5%),radiotherapy(3.1%)or combination(34.4%).Median overall survival was 15 mo and 24-mo overall survival was 40.5%.Median disease-free survival was 10 mo and 24-mo disease-free survival was 33.3%.CONCLUSION The significant progress in molecular biology and genetics of ES does not reflect on patient outcomes.ES remains an aggressive tumor with a poor prognosis and high mortality.展开更多
BACKGROUND Undifferentiated pleomorphic sarcomas,also known as spindle cell sarcomas,are a relatively uncommon subtype of soft tissue sarcomas in clinical practice.CASE SUMMARY We present a case report of a 69-year-ol...BACKGROUND Undifferentiated pleomorphic sarcomas,also known as spindle cell sarcomas,are a relatively uncommon subtype of soft tissue sarcomas in clinical practice.CASE SUMMARY We present a case report of a 69-year-old female patient who was diagnosed with undifferentiated spindle cell soft tissue sarcoma on her left thigh.Surgical excision was initially performed,but the patient experienced a local recurrence following multiple surgeries and radioactive particle implantations.High-intensity focused ultrasound(HIFU)was subsequently administered,resulting in complete ablation of the sarcoma without any significant complications other than bone damage at the treated site.However,approximately four months later,the patient experienced a broken lesion at the original location.After further diagnostic workup,the patient underwent additional surgery and is currently stable with a good quality of life.CONCLUSION HIFU has shown positive outcomes in achieving local control of limb spindle cell sarcoma,making it an effective non-invasive treatment option.展开更多
Using data from the Surveillance, Epidemiology, and End Results (SEER) Program based at the National Cancer Institute in the US, conditional survival rates are reported for 1,988 Ewing Sarcoma patients diagnosed durin...Using data from the Surveillance, Epidemiology, and End Results (SEER) Program based at the National Cancer Institute in the US, conditional survival rates are reported for 1,988 Ewing Sarcoma patients diagnosed during the period 2000-2015. These patients represent the experience of 26.5% of the US population. Specifically, 5-year conditional relative survival rates are calculated for these patients for the first eight years subsequent to diagnosis of their cancer by Extent of Disease (EOD) (Localized, Regional, and Distant as coded by the SEER Program), gender, and age (<18, 18 - 34, and 35+). Findings include showing how the conditional survival rate patterns improve over time and that there are differences by gender, age, and EOD.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
A 66-year-old female with a 1-month history of increasing fatigue, dyspnea on exertion, and palpitations presented with clinical signs of heart failure. Chest computed tomography (CT) revealed a large, mobile left atr...A 66-year-old female with a 1-month history of increasing fatigue, dyspnea on exertion, and palpitations presented with clinical signs of heart failure. Chest computed tomography (CT) revealed a large, mobile left atrial (LA) mass attached to the mitral valve causing severe mitral stenosis and mitral regurgitation. The mass was surgically debulked and the mitral valve was replaced. Pathology revealed a poorly differentiated malignant spindle cell neoplasm with diffuse nuclear positivity of MDM2 and multifocal positivity of CDK4, consistent with intimal sarcoma. This case seeks to describe an uncommon presentation of a rare malignancy, and the surgical and medical management of the disease.展开更多
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BACKGROUND Low-grade myofibroblastic sarcoma(LGMS)is an extremely rare tumor characterized by the malignant proliferation of myofibroblasts.LGMS most commonly develops in adults,predominantly in males,in the head and neck region,oral cavity,especially on the tongue,mandible,and larynx.This article presents 2 cases of LGMS localized to the maxillary sinus and provides an overview of the available literature.CASE SUMMARY Two patients with LGMS located in the maxillary sinus underwent surgery at the Department of Head and Neck Surgery.Case 1:A 46-year-old patient was admitted to the clinic with suspected LGMS recurrence in the right maxillary sinus(rT4aN0M0),with symptoms of pain in the suborbital area,watering of the right eye,thick discharge from the right nostril,and augmented facial asymmetry.After open biopsy-confirmed LGMS,the patient underwent expanded maxillectomy of the right side with immediate palate reconstruction using a microvascular skin flap harvested surgically from the middle arm.The patient qualified for adjuvant radiotherapy for the postoperative bed,with an additional margin.Currently,the patient is under 1.5 years of observation with no evidence of disease.Case 2:A 45-year-old man was admitted to our clinic with facial asymmetry,strabismus,exophthalmos,and visual impairment in the right eye.Six months earlier,the patient had undergone partial jaw resection at another hospital for fibromatosis.A contrast-enhanced computed tomography scan revealed a tumor mass in the postoperative log after an earlier procedure.An open biopsy confirmed lowgrade fibrosarcoma(rT4aN0M0).The patient qualified for an extended total right maxillectomy with orbital excision and right hemimandibulectomy with immediate microvascular reconstruction using an anterolateral thigh flap.The patient subsequently underwent adjuvant radiotherapy to the postoperative area.After 9 months,recurrence occurred in the right mandibular arch below the irradiated area.The lesion infiltrated the base of the skull,which warranted the withdrawal of radiotherapy and salvage surgery.The patient qualified for palliative chemotherapy with a regimen of doxorubicin+dacarbazine+cyclophosphamide and palliative radiotherapy for bone metastases.The patient died 26 months after surgical treatment.The cases have been assessed and compared with cases in the literature.CONCLUSION No specific diagnostic criteria or treatment strategies have been developed for LGMS.The treatment used for LGMS is the same as that used for sinonasal cancer radical tumor excision;adjuvant radiotherapy or chemoradiotherapy should also be considered.They have low malignant potential but are highly invasive,tend to recur,and metastasize to distant sites.Patients should undergo regular follow-up examinations to detect recurrence or metastasis at an early stage.Patients should be treated and observed at the highest referral centers.
文摘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.
文摘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.
文摘Introduction: Kaposi sarcoma disease is a proliferative and multifocal disorder with dual components, vascular and fibroblastic cellular, cutaneous and visceral expression. Kaposi Sarcoma can affect the ocular surface and adnexa and can masquerade as other entities, delaying prompt diagnosis can lead to diagnostic wandering delaying treatment. Our aim is to describe a case of KS of the eyelid in an HIV seronegative patient. Case Presentation: A seventy-year-old man developed a bilateral growing tumoral reddish purple vascular mass on both the lower and upper eyelid involving rapidly for 6 months. Both feet and the two shanks show the presence of a brown-violet tumor-shaped formation. The patient was negative for HIV. Histology examination showed a nodular tumor-like mass with a fibro hemangioma-epitheliomatous. Polymerase chain reaction was positive for human herpes virus 8. Initial chemotherapy followed by surgery was proposed to the patient. Unfortunately, the patient rejected treatment and was lost to follow-up. Conclusion: This case reports the difficulty of managing KS in developing countries.
文摘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.
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.
文摘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.
文摘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.
文摘Purpose: Development of sarcoma is a known late rare negative side effect of radiotherapy. We add two cases to emphasize the need for open-end follow-up and critical evaluation to avoid misinterpretation. Patients, Methods, and Results: Two patients developed osteosarcoma as a second malignancy in the humerus after adjuvant radiotherapy of a primary tumor not directly involving the later affected bone. The first patient had a Ewing sarcoma of the scapula at age 13 years. Though after neoadjuvant chemotherapy the resected specimen showed only fibrotic necrotic areas within clear resection margins, the study group indicated adjuvant radiotherapy in a field including the shoulder joint. At age 24 years she developed an osteosarcoma of the humeral head, which was resected and reconstructed with a proximal humerus endoprosthesis. She is alive without disease at age 32 years. The second patient presented with an osteosarcoma of the proximal humerus 29 years after irradiation for breast cancer including the shoulder joint. The sarcoma was misinterpreted as radiation-induced necrosis and the patient was treated with a reverse shoulder endoprosthesis. Pathologic examination of the resected humeral head then showed a typical osteosarcoma. Two years later the humeral reverse shoulder implant was resected and a proximal humerus tumor prosthesis implanted leaving the original glenosphere. Conclusions: In both cases radiation-induced osteosarcoma developed in bone not affected by the primary cancer. Protecting uninvolved structures must be warranted in the planning of radiotherapy. The long latency between the primary and second cancer mandates long-term—best indefinite—follow-up, as with appropriate treatment of a radiation-induced osteosarcoma good healing rates comparable to those of primary osteosarcoma can still be achieved.
基金Supported by the National Natural Science Foundation of China (No.82171099,No.82000940,No.81970835,No.81800867)the Natural Science Foundation of Shanghai (No.20ZR1409500)。
文摘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.
文摘BACKGROUND Low-grade myofibroblastic sarcoma(LGMS)is a rare spindle cell sarcoma espe-cially in the pancreas,with myofibroblastic differentiation.Hitherto,only a few cases have been reported.CASE SUMMARY Herein,we report a case involving the discovery of a pancreatic mass detected during a routine physical examination.Subsequent imaging and pathological tests of the patient led to the diagnosis of LGMS of the pancreas.Following surgical intervention,the patient experienced recurrence and metastasis.Conventional treatment is not effective for postoperative recurrent pancreatic LGMS with multiple metastases.After communicating with the patients and their families,informed consent was obtained for the treatment of anlotinib combined with pembrolizumab.Evaluation of imaging and clinical symptoms post-treatment revealed a relatively favorable response to the combination of anlotinib and pembrolizumab.CONCLUSION Based on the comprehensive literature review,our report aimed to provide evidence for a better understanding of the disease characteristics,diagnostic criteria,imaging findings,and identification of LGMS.And explore novel treatment strategies for this disease.
基金Supported by the Science and Education Project of Neijiang First People’s Hospital。
文摘BACKGROUND Extraskeletal Ewing sarcoma(EES)is a rare and highly malignant small round cell tumor associated with a poor clinical outcome.Ewing sarcoma(ES)involving the stomach is an uncommon presentation and can be easily confused with other small round cell tumors.We herein present a rare case of ES involving the gastric area.CASE SUMMARY We report a case of gastric ES in a 19-year-old female patient who initially presented with a complaint of a tender epigastric mass for 5 d.Contrast-enhanced abdominal computed tomography revealed a soft-tissue-density mass with a diameter of 8.5 cm between the liver and stomach;the mass was connected to the gastric antrum.Then,the mass was surgically excised completely.Upon histopathological,immunophenotype and molecular analysis,the mass was identified to be a primary gastric ES.CONCLUSION EES is an aggressive tumor with poor prognosis.Therefore,early diagnosis and timely intervention are essential for a good prognosis.It is imperative for us to raise awareness about this rare tumor.Surgical resection is still the best treatment option.
文摘Undifferentiated pleomorphic sarcoma is an extremely rare malignant thyroid tumor.Thyroid sarcoma differs from common malignant thyroid tumors,such as thyroid follicular cell carcinoma.It is usually highly malignant,progresses rapidly,and is prone to remote metastasis.Currently,there is no standard protocol for the treatment of thyroid sarcomas,and most treatment effects are unsatisfactory.Argon-helium cryoablation is an important method of local treatment that is widely used in patients with unresectable advanced tumors.However,owing to the low incidence of thyroid sarcomas,there are no relevant literature reports on the treatment of thyroid sarcomas using cryoablation in China.This study reports the case of a patient with undifferentiated pleomorphic sarcoma of the thyroid gland who was treated with argon-helium cryoablation,and the immediate outcome was good.Based on a review of relevant literature,we discussed the effectiveness and safety of argon-helium cryoablation treatment,to provide clinical guidance and references for the treatment of patients with thyroid sarcoma.
基金Supported by Scientific Research Project of Anhui Provincial Health Commission,No.AHWJ2021b005.
文摘BACKGROUND The mixed lineage leukemia(MLL)-eleven-nineteen lysine-rich leukemia(ELL)fusion gene is a rare occurrence among the various MLL fusion genes.We present the first case in which myeloid sarcoma(MS)was the only manifestation of adult MLL-ELL-positive acute myeloid leukemia(AML).CASE SUMMARY We report a case of a 33-year-old male patient who was admitted in June 2022 with a right occipital area mass measuring approximately 7 cm×8 cm.Blood work was normal.The patient underwent right occipital giant subscalp mass excision and incisional flap grafting.Immunohistochemistry was positive for myeloperoxidase,CD43 and CD45 and negative for CD3,CD20,CD34,and CD56.The bone marrow aspirate showed hypercellularity with 20%myeloblasts.Flow cytometry showed that myeloblasts accounted for 27.21%of the nucleated cells,which expressed CD33,CD38,and CD117.The karyotype was 46,XY,t(11,19)(q23;p13.1),-12,+mar/46,XY.Next-generation sequencing showed a fusion of MLL exon 7 to exon 2 of ELL.A diagnosis of MLL-ELL-positive AML(M2 subtype)with subcutaneous MS was made.CONCLUSION MLL-ELL-positive AML with MS is a rare clinical entity.Additional research is needed to elucidate the molecular mechanisms of the pathogenesis of MS.
文摘BACKGROUND Ewing sarcoma(ES)is a malignant neoplasm of neuroectodermal origin and is commonly observed in children and young adults.The musculoskeletal system is the main body system impacted and ES is rarely seen in the visceral organs particularly the adrenal gland.AIM To present a comprehensive review of primary adrenal ES,with emphasis on diagnosis,therapy and oncological outcomes.METHODS A systematic review of the literature was performed according to the Preferred Reporting Items for Systematic reviews and Meta-Analyses 2020.PubMed/MEDLINE,EMBASE and Google Scholar bibliographic databases were searched to identify articles from 1989 to 2022 and included patients with ES/primitive neuroectodermal tumor(PNET)of the adrenal gland.PubMed,Google Scholar and EMBASE medical databases were searched,combining the terms“adrenal”,“ES”and“PNET”.Demographic,clinical,pathological and oncological data of patients were analyzed by SPSS version 29.0.RESULTS A total of 52 studies were included for review(47 case reports and 5 case series)with 66 patients reported to have primary adrenal ES.Mean age at diagnosis was 26.4±15.4 years(37.9%males,57.6%females,sex not reported in 3 cases).The most frequent complaint was abdominal/flank pain or discomfort(46.4%)followed by a palpable mass(25.0%),and the average duration of symptoms was 2.6±3.1 mo.The imaging modality of choice was computed tomography scan(81.5%),followed by magnetic resonance imaging(20.4%).Preoperative staging revealed that 17 tumors(27.9%)were metastatic and 14 patients had inferior vena cava or renal vein neoplastic thrombus at initial diagnosis.Open adrenalectomy was performed in the majority of cases(80.0%),of which 27.9%required more extensive resection.Minimally invasive surgery was attempted in 8.2%of tumors.Complete surgical resection was achieved in 89.4%of the patients.Adjuvant therapy was administered to 32 patients,in the form of chemotherapy(62.5%),radiotherapy(3.1%)or combination(34.4%).Median overall survival was 15 mo and 24-mo overall survival was 40.5%.Median disease-free survival was 10 mo and 24-mo disease-free survival was 33.3%.CONCLUSION The significant progress in molecular biology and genetics of ES does not reflect on patient outcomes.ES remains an aggressive tumor with a poor prognosis and high mortality.
文摘BACKGROUND Undifferentiated pleomorphic sarcomas,also known as spindle cell sarcomas,are a relatively uncommon subtype of soft tissue sarcomas in clinical practice.CASE SUMMARY We present a case report of a 69-year-old female patient who was diagnosed with undifferentiated spindle cell soft tissue sarcoma on her left thigh.Surgical excision was initially performed,but the patient experienced a local recurrence following multiple surgeries and radioactive particle implantations.High-intensity focused ultrasound(HIFU)was subsequently administered,resulting in complete ablation of the sarcoma without any significant complications other than bone damage at the treated site.However,approximately four months later,the patient experienced a broken lesion at the original location.After further diagnostic workup,the patient underwent additional surgery and is currently stable with a good quality of life.CONCLUSION HIFU has shown positive outcomes in achieving local control of limb spindle cell sarcoma,making it an effective non-invasive treatment option.
文摘Using data from the Surveillance, Epidemiology, and End Results (SEER) Program based at the National Cancer Institute in the US, conditional survival rates are reported for 1,988 Ewing Sarcoma patients diagnosed during the period 2000-2015. These patients represent the experience of 26.5% of the US population. Specifically, 5-year conditional relative survival rates are calculated for these patients for the first eight years subsequent to diagnosis of their cancer by Extent of Disease (EOD) (Localized, Regional, and Distant as coded by the SEER Program), gender, and age (<18, 18 - 34, and 35+). Findings include showing how the conditional survival rate patterns improve over time and that there are differences by gender, age, and EOD.
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.
文摘A 66-year-old female with a 1-month history of increasing fatigue, dyspnea on exertion, and palpitations presented with clinical signs of heart failure. Chest computed tomography (CT) revealed a large, mobile left atrial (LA) mass attached to the mitral valve causing severe mitral stenosis and mitral regurgitation. The mass was surgically debulked and the mitral valve was replaced. Pathology revealed a poorly differentiated malignant spindle cell neoplasm with diffuse nuclear positivity of MDM2 and multifocal positivity of CDK4, consistent with intimal sarcoma. This case seeks to describe an uncommon presentation of a rare malignancy, and the surgical and medical management of the disease.
