BACKGROUND Low grade fibromyxoid sarcoma(LGFMS)is a rare and benign mesenchymal tumor with indolent course,most commonly found in young or middle-aged men.The majority of the LGFMSs are located in the trunk and deep s...BACKGROUND Low grade fibromyxoid sarcoma(LGFMS)is a rare and benign mesenchymal tumor with indolent course,most commonly found in young or middle-aged men.The majority of the LGFMSs are located in the trunk and deep soft tissue of the lower extremities.They appear as well circumscribed,although not encapsulated,which often leads to incomplete surgical resection.Despite their seemingly benign appearance,these tumors have aggressive behavior with high metastatic and recurrence rates.Accurate histopathologic examination of the specimen and its immunohistochemical analysis are mandatory for a precise diagnosis.CASE SUMMARY We report a case of a 38 year-old-man who presented with jaundice and upper abdominal discomfort.Multi-detector computed tomography and magnetic resonance imaging showed a large left liver tumor mass,extending to the hepatoduodenal ligament.Left hepatectomy was performed with resection and reconstruction of hepatic artery and preservation of middle hepatic vein.Histopathologic examination confirmed the tumor being a low-grade fibromyxoid sarcoma.Three and a half years after surgery,the patient died after being diagnosed with spine metastasis.CONCLUSION Due to poor response to all modalities of adjuvant treatment,we consider that the focus of treatment should be on surgery as the only option for curing the disease.展开更多
Low-grade fibromyxoid sarcoma is a slowly growing soft tissue neoplasm, mostly affecting young individuals. It usually arises in a deep soft tissue of the lower limbs and trunk, but few cases of low-grade fibromyxoid ...Low-grade fibromyxoid sarcoma is a slowly growing soft tissue neoplasm, mostly affecting young individuals. It usually arises in a deep soft tissue of the lower limbs and trunk, but few cases of low-grade fibromyxoid sarcoma that presents sciatic nerve pain have been reported. We report a 34-year-old woman with a low-grade fibromyxoid sarcoma originating in the left gluteal region that initially presented as sciatic nerve pain;she had experienced this type of pain for 5 years before visiting our hospital. Magnetic resonance imaging revealed a tumor in the left gluteal region. After needle biopsy revealed it to be a low-grade fibromyxoid sarcoma, we performed the wide resection. Intra-operative findings revealed no tumor invasion into the sciatic nerve. No recurrence or metastasis has been detected 6 months post-surgery. Oncologists who encounter patients with sciatic nerve pain should consider the possibility of less common causes such as the low-grade fibromyxoid sarcoma found in our patient.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 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
Low-grade myofibroblastic sarcoma (LGMS) is a rare, malignant tumor with myofibroblastic differentiation. Despite it being classified as a distinct entity by the World Health Organization, a few cases were reported ...Low-grade myofibroblastic sarcoma (LGMS) is a rare, malignant tumor with myofibroblastic differentiation. Despite it being classified as a distinct entity by the World Health Organization, a few cases were reported in the oral and maxillofacial region. Here, a LGMS developed on the palate of a 73-year-old man who presented with a 1-cm tumor on the posterior border of the palate. Based on the histological and immunohistochemical features, a diagnosis of LGMS was established. The tumor was resected, and no recurrence was observed over 2 years. Although the tongue is the most preferred site for LGMS, it may occur in any region of the oral cavity.展开更多
BACKGROUND Low-grade endometrial stromal sarcoma(LGESS)classically exhibits a proliferative morphology.However,morphological variation of extrauterine tumors presents a diagnostic challenge.CASE SUMMARY We report the ...BACKGROUND Low-grade endometrial stromal sarcoma(LGESS)classically exhibits a proliferative morphology.However,morphological variation of extrauterine tumors presents a diagnostic challenge.CASE SUMMARY We report the case of a 76-year-old female patient with extensive extrauterine and abdominal neoplastic lesions.Computed tomography showed massive pleural and ascitic fluid,and there was an increase in serum cancer antigen 125.She underwent bilateral adnexectomy and tumor resection.The right ovary had been replaced by a multinodular mass that was 8.5 cm×4.5 cm×3.5 cm in size.In addition,there was a 24 cm×15 cm×13 cm mesenteric mass,which was also multinodular,with local invasion of the intestinal serosa and underlying muscle.Under the microscope,the tumors in different places exhibited two different patterns,thus presenting great challenges to diagnosis and treatment.Thorough pathological assessment eliminated all differential diagnoses in favor of metastatic LGESS derived from a 20-year-old primary tumor initially misdiagnosed as leiomyosarcoma.CONCLUSION LGESS morphology varies according to tumor location.Accurate diagnosis is critical for appropriate treatment and improved prognosis and patient care.展开更多
IntroductionMultiple myeloma (MM) is a neoplastic plasma cell dyscrasia char-acterized by anemia; a monoclonal protein(M-protein) in the serum and/or urine; abnormal bone radiographs and bone pain;hypercal-cemia; ...IntroductionMultiple myeloma (MM) is a neoplastic plasma cell dyscrasia char-acterized by anemia; a monoclonal protein(M-protein) in the serum and/or urine; abnormal bone radiographs and bone pain;hypercal-cemia; and renal insuf.ciency or failure.According to the results of immunoelectrophoresis, patients are separated to Ig type (IgG, IgA, IgD, IgE and IgM); light chain; nonsecretory.展开更多
Background:Low-grade endometrial stromal sarcoma(LG-ESS)is a rare tumor that lacks a prognostic prediction model.Our study aimed to develop a nomogram to predict overall survival of LG-ESS patients.Methods:A total of ...Background:Low-grade endometrial stromal sarcoma(LG-ESS)is a rare tumor that lacks a prognostic prediction model.Our study aimed to develop a nomogram to predict overall survival of LG-ESS patients.Methods:A total of 1172 patients confirmed to have LG-ESS between 1988 and 2015 were selected from the Surveillance,Epidemiology and End Results(SEER)database.They were further divided into a training cohort and a validation cohort.The Akaike information criterion was used to select variables for the nomogram.The discrimination and calibration of the nomogram were evaluated using concordance index(C-index),area under time-dependent receiver operating characteristic curve(time-dependent AUC),and calibration plots.The net benefits of the nomogram at different threshold probabilities were quantified and compared with those of the International Federation of Gynecology and Obstetrics(FIGO)criteria-based tumor staging using decision curve analysis(DCA).Net reclassification index(NRI)and integrated discrimination improvement(IDI)were also used to compare the nomogram’s clinical utilitywith that of the FIGO criteria-based tumor staging.The risk stratifications of the nomogram and the FIGO criteria-based tumor staging were compared.Results:Seven variables were selected to establish the nomogram for LG-ESS.The C-index(0.814 for the training cohort and 0.837 for the validation cohort)and the time-dependent AUC(>0.7)indicated satisfactory discriminative ability of the nomogram.The calibration plots showed favorable consistency between the prediction of the nomogram and actual observations in both the training and validation cohorts.The NRI values(training cohort:0.271 for 5-year and 0.433 for 10-year OS prediction;validation cohort:0.310 for 5-year and 0.383 for 10-year OS prediction)and IDI(training cohort:0.146 for 5-year and 0.185 for 10-year OS prediction;validation cohort:0.177 for 5-year and 0.191 for 10-year OS prediction)indicated that the established nomogram performed significantly better than the FIGO criteria-based tumor staging alone(P<0.05).Furthermore,DCA showed that the nomogram was clinically useful and had better discriminative ability to recognize patients at high risk than the FIGO criteria-based tumor staging.Conclusions:A prognostic nomogram was developed and validated to assist clinicians in evaluating prognosis of LG-ESS patients.展开更多
文摘BACKGROUND Low grade fibromyxoid sarcoma(LGFMS)is a rare and benign mesenchymal tumor with indolent course,most commonly found in young or middle-aged men.The majority of the LGFMSs are located in the trunk and deep soft tissue of the lower extremities.They appear as well circumscribed,although not encapsulated,which often leads to incomplete surgical resection.Despite their seemingly benign appearance,these tumors have aggressive behavior with high metastatic and recurrence rates.Accurate histopathologic examination of the specimen and its immunohistochemical analysis are mandatory for a precise diagnosis.CASE SUMMARY We report a case of a 38 year-old-man who presented with jaundice and upper abdominal discomfort.Multi-detector computed tomography and magnetic resonance imaging showed a large left liver tumor mass,extending to the hepatoduodenal ligament.Left hepatectomy was performed with resection and reconstruction of hepatic artery and preservation of middle hepatic vein.Histopathologic examination confirmed the tumor being a low-grade fibromyxoid sarcoma.Three and a half years after surgery,the patient died after being diagnosed with spine metastasis.CONCLUSION Due to poor response to all modalities of adjuvant treatment,we consider that the focus of treatment should be on surgery as the only option for curing the disease.
文摘Low-grade fibromyxoid sarcoma is a slowly growing soft tissue neoplasm, mostly affecting young individuals. It usually arises in a deep soft tissue of the lower limbs and trunk, but few cases of low-grade fibromyxoid sarcoma that presents sciatic nerve pain have been reported. We report a 34-year-old woman with a low-grade fibromyxoid sarcoma originating in the left gluteal region that initially presented as sciatic nerve pain;she had experienced this type of pain for 5 years before visiting our hospital. Magnetic resonance imaging revealed a tumor in the left gluteal region. After needle biopsy revealed it to be a low-grade fibromyxoid sarcoma, we performed the wide resection. Intra-operative findings revealed no tumor invasion into the sciatic nerve. No recurrence or metastasis has been detected 6 months post-surgery. Oncologists who encounter patients with sciatic nerve pain should consider the possibility of less common causes such as the low-grade fibromyxoid sarcoma found in our patient.
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 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.
文摘Low-grade myofibroblastic sarcoma (LGMS) is a rare, malignant tumor with myofibroblastic differentiation. Despite it being classified as a distinct entity by the World Health Organization, a few cases were reported in the oral and maxillofacial region. Here, a LGMS developed on the palate of a 73-year-old man who presented with a 1-cm tumor on the posterior border of the palate. Based on the histological and immunohistochemical features, a diagnosis of LGMS was established. The tumor was resected, and no recurrence was observed over 2 years. Although the tongue is the most preferred site for LGMS, it may occur in any region of the oral cavity.
文摘BACKGROUND Low-grade endometrial stromal sarcoma(LGESS)classically exhibits a proliferative morphology.However,morphological variation of extrauterine tumors presents a diagnostic challenge.CASE SUMMARY We report the case of a 76-year-old female patient with extensive extrauterine and abdominal neoplastic lesions.Computed tomography showed massive pleural and ascitic fluid,and there was an increase in serum cancer antigen 125.She underwent bilateral adnexectomy and tumor resection.The right ovary had been replaced by a multinodular mass that was 8.5 cm×4.5 cm×3.5 cm in size.In addition,there was a 24 cm×15 cm×13 cm mesenteric mass,which was also multinodular,with local invasion of the intestinal serosa and underlying muscle.Under the microscope,the tumors in different places exhibited two different patterns,thus presenting great challenges to diagnosis and treatment.Thorough pathological assessment eliminated all differential diagnoses in favor of metastatic LGESS derived from a 20-year-old primary tumor initially misdiagnosed as leiomyosarcoma.CONCLUSION LGESS morphology varies according to tumor location.Accurate diagnosis is critical for appropriate treatment and improved prognosis and patient care.
文摘IntroductionMultiple myeloma (MM) is a neoplastic plasma cell dyscrasia char-acterized by anemia; a monoclonal protein(M-protein) in the serum and/or urine; abnormal bone radiographs and bone pain;hypercal-cemia; and renal insuf.ciency or failure.According to the results of immunoelectrophoresis, patients are separated to Ig type (IgG, IgA, IgD, IgE and IgM); light chain; nonsecretory.
基金supported by grants no.81670123 and no.81670144 from the National Natural Science Foundation of China(NSFC).
文摘Background:Low-grade endometrial stromal sarcoma(LG-ESS)is a rare tumor that lacks a prognostic prediction model.Our study aimed to develop a nomogram to predict overall survival of LG-ESS patients.Methods:A total of 1172 patients confirmed to have LG-ESS between 1988 and 2015 were selected from the Surveillance,Epidemiology and End Results(SEER)database.They were further divided into a training cohort and a validation cohort.The Akaike information criterion was used to select variables for the nomogram.The discrimination and calibration of the nomogram were evaluated using concordance index(C-index),area under time-dependent receiver operating characteristic curve(time-dependent AUC),and calibration plots.The net benefits of the nomogram at different threshold probabilities were quantified and compared with those of the International Federation of Gynecology and Obstetrics(FIGO)criteria-based tumor staging using decision curve analysis(DCA).Net reclassification index(NRI)and integrated discrimination improvement(IDI)were also used to compare the nomogram’s clinical utilitywith that of the FIGO criteria-based tumor staging.The risk stratifications of the nomogram and the FIGO criteria-based tumor staging were compared.Results:Seven variables were selected to establish the nomogram for LG-ESS.The C-index(0.814 for the training cohort and 0.837 for the validation cohort)and the time-dependent AUC(>0.7)indicated satisfactory discriminative ability of the nomogram.The calibration plots showed favorable consistency between the prediction of the nomogram and actual observations in both the training and validation cohorts.The NRI values(training cohort:0.271 for 5-year and 0.433 for 10-year OS prediction;validation cohort:0.310 for 5-year and 0.383 for 10-year OS prediction)and IDI(training cohort:0.146 for 5-year and 0.185 for 10-year OS prediction;validation cohort:0.177 for 5-year and 0.191 for 10-year OS prediction)indicated that the established nomogram performed significantly better than the FIGO criteria-based tumor staging alone(P<0.05).Furthermore,DCA showed that the nomogram was clinically useful and had better discriminative ability to recognize patients at high risk than the FIGO criteria-based tumor staging.Conclusions:A prognostic nomogram was developed and validated to assist clinicians in evaluating prognosis of LG-ESS patients.