Introduction: Primary cardiac myxofibrosarcoma (MFS) is a rare and aggressive malignancy, with limited treatment options and a high recurrence rate. Multimodal imaging is critical for diagnosis, and surgery remains th...Introduction: Primary cardiac myxofibrosarcoma (MFS) is a rare and aggressive malignancy, with limited treatment options and a high recurrence rate. Multimodal imaging is critical for diagnosis, and surgery remains the primary treatment, though recurrence is common. Case Presentation: We present the case of a 41-year-old male with history of intermittent chest pain and palpitations. Initial work-up revealed a left atrial mass, which after thorough evaluation with multimodal imaging and histopathological assessment, was diagnosed as a primary cardiac myxofibrosarcoma. Despite complete surgical resection and low-grade histology, recurrence ensued, prompting reintervention and adjuvant radiotherapy. Discussion: Cardiac MFS poses significant diagnostic challenges. Multimodal imaging and immunohistochemistry are key to diagnosis. While surgery is the mainstay of treatment, high recurrence rates necessitate consideration of adjuvant therapies and long-term monitoring. Conclusion: This case highlights the importance of differential diagnosis of intracardiac masses, as well as reaching a consensus regarding the approach for malignant cardiac tumors.展开更多
BACKGROUND Cardiac tumors are rare and complex entities.Surgery represents the cornerstone of therapy,while the role of adjuvant treatment remains unclear and,in case of relapse or metastatic disease,the prognosis is ...BACKGROUND Cardiac tumors are rare and complex entities.Surgery represents the cornerstone of therapy,while the role of adjuvant treatment remains unclear and,in case of relapse or metastatic disease,the prognosis is very poor.Lack of prospective,randomized clinical trials hinders the generation of high level evidence for the optimal diagnostic workup and multimodal treatment of cardiac sarcomas.Herein,we describe the multidisciplinary clinical management and molecular characterization of a rare case of cardiac myxofibrosarcoma in an elderly woman.CASE SUMMARY A 73-year-old woman presented signs and symptoms of acute left-sided heart failure.Imaging examination revealed a large,left atrial mass.With suspicion of a myxoma,she underwent surgery,and symptoms were promptly relieved.Histology showed a cardiac myxofibrosarcoma,a rare histotype of cardiac sarcoma.Eight months later,disease unfortunately relapsed,and after a multidisciplinary discussion,a chemotherapy with doxorubicin and then gemcitabine was started,achieving partial radiologic and complete metabolic response,which was maintained up to 2 years and is still present.This report is focused on the entire clinical path of our patient from diagnosis to follow-up,through surgery and strategies adopted at relapse.Moreover,due to their rarity,very little is known about the molecular landscape of myxofibrosarcomas.Thus,we also performed and described preliminary genome analysis of the tumor tissue to get further insight on mechanisms involved in tumor growth,and to possibly unveil new clinically actionable targets.CONCLUSION We report a case of cardiac myxofibrosarcoma that achieved a very good prognosis due to an integrated surgical,cardiac and oncologic treatment strategy.展开更多
BACKGROUND A myxofibrosarcoma(MFS)is a malignant fibroblastic tumor that tends to occur in the lower and upper extremities.The reported incidence of head and neck MFSs is extremely rare.We report a 46-year-old male w...BACKGROUND A myxofibrosarcoma(MFS)is a malignant fibroblastic tumor that tends to occur in the lower and upper extremities.The reported incidence of head and neck MFSs is extremely rare.We report a 46-year-old male with“a neoplasm in the scalp”who was hospitalized and diagnosed with an MFS(highly malignant with massive necrotic lesions)based on histologic and immunohistochemistry evaluations.The magnetic resonance imaging manifestations did not demonstrate the“tail sign”mentioned in several studies,which resulted in a great challenge to establish an imaging diagnosis.The treatment plan is closely associated with the anatomic location and histologic grade,and more importantly,aggressive surgery and adjuvant radiotherapy may be helpful.Hence,we report the case and share some valuable information about the disease.CASE SUMMARY A 46-year-old male with“a neoplasm in the scalp for 6 mo”was hospitalized.Initially,the tumor was about the size of a soybean,without algesia or ulceration.The patient ignored the growth,did not seek treatment,and thus,did not receive treatment.Recently,the tumor increased to the size of an egg;there was no bleeding or algesia.His family history was unremarkable.No abnormalities were found upon laboratory testing,including routine hematologic,biochemistry,and tumor markers.Computed tomography showed an ovoid mass(6.25 cm×3.29 cm×3.09 cm in size)in the left frontal scalp with low density intermingled with equidense strips in adjacent areas of the scalp.Magnetic resonance imaging revealed a lesion with an irregular surface and an approximate size of 3.55 cm×6.34 cm in the left frontal region,with clear boundaries and visible separation.Adjacent areas of the skull were damaged and the dura mater was involved.Contrast enhancement showed an uneven enhancement pattern.Surgery was performed and postoperative adjuvant radiotherapy was administered to avoid recurrence or metastasis.The post-operative pathologic diagnosis confirmed an MFS.A repeat computed tomography scan showed no local recurrence or distant metastasis 19 mo post-operatively.CONCLUSION The case reported herein of MFS was demonstrated in an extremely rare location on the scalp and had atypical magnetic resonance imaging findings,which serves as a reminder to radiologists of the possibility of this diagnosis to assist in clinical treatment.Given the special anatomic location and the high malignant potential of this rare tumor,combined surgical and adjuvant radiotherapy should be considered to avoid local recurrence and distant metastasis.The significance of regular follow-up is strongly recommended to improve the long-term survival rate.展开更多
BACKGROUND Myxofibrosarcoma(MFS)is a fibroblast-derived sarcoma that mainly occurs in subcutaneous tissue.MFS rarely occurs in the gastrointestinal tract,especially in the esophagus.CASE SUMMARY A 79-year-old male pat...BACKGROUND Myxofibrosarcoma(MFS)is a fibroblast-derived sarcoma that mainly occurs in subcutaneous tissue.MFS rarely occurs in the gastrointestinal tract,especially in the esophagus.CASE SUMMARY A 79-year-old male patient was admitted to our hospital for dysphagia for a week.Computed tomography and electronic gastroscopy showed that a giant mass was located 30 cm from the incisor and extended to the cardia.There was incomplete esophageal stenosis.Endoscopic pathology showed spindle cell lesions,which were considered inflammatory myofibroblast like hyperplasia.Considering the strong demands of the patient and his family,and the fact that most inflammatory myofibroblast tumors are benign,we decided to perform endoscopic submucosal dissection(ESD)even if the tumor size was giant(9.0 cm×3.0 cm).Postoperative pathological examination resulted in a final diagnosis of MFS.MFS rarely occurs in the gastrointestinal tract,especially in the esophagus.Surgical resection and local adjuvant radiotherapy are the first choices to improve the prognosis.This case report firstly described the ESD for esophageal giant MFS.It suggests that ESD may be an alternative treatment for primary esophageal MFS.CONCLUSION This case report for the first time describe the successful treatment of a giant esophageal MFS by ESD,suggesting that ESD may be an alternative treatment for primary esophageal MFS,especially in elderly high-risk patients with obvious dysphagia symptoms.展开更多
Here, we report the case of a 47-year-old male who presented with a painless palpable mass in the right shoulder. This extremity tumor was diagnosed as a high-grade myxofibrosarcoma after a wide excision. Simultaneous...Here, we report the case of a 47-year-old male who presented with a painless palpable mass in the right shoulder. This extremity tumor was diagnosed as a high-grade myxofibrosarcoma after a wide excision. Simultaneously, a synchronous mesenteric mass was discovered, which proved to be a leiomyosarcoma.展开更多
Soft tissue sarcomas represent only 1% of all adult cancers;myxofibrosarcoma is the most common type that arises in adult extremities, particularly lower limbs (77%), other less common locations are the trunk (12%) an...Soft tissue sarcomas represent only 1% of all adult cancers;myxofibrosarcoma is the most common type that arises in adult extremities, particularly lower limbs (77%), other less common locations are the trunk (12%) and neck (3%). Usually presenting as a painless, subcutaneous, slow growing mass with tendency for recurrence, they are prone to have higher histological grade and metastatic potential after recurrence;even in optimal multidisciplinary settings patients can have incomplete resections, making metastatic disease more common after misdiagnosis. We present the case of a 69-year-old male patient with a right infraescapular tumor, presenting as a painless 15 × 8 cm, mobile mass, with a slow but progressive growth, history of a previous tumor excised at the same location 5 years prior without histopathological report.展开更多
Background:Myxofibrosarcoma(MFS),especially radiation-Induced MFS(RIMFS)in the head and neck,is an extremely rare malignant fibroblastic tumor.The diagnosis and treatment of MFS remain great challenges.In the present ...Background:Myxofibrosarcoma(MFS),especially radiation-Induced MFS(RIMFS)in the head and neck,is an extremely rare malignant fibroblastic tumor.The diagnosis and treatment of MFS remain great challenges.In the present study,we presented one case of RIMFS.Combined with previous literature,the clinical features,essentials of diagnosis,and treatment modalities of MFS in the head and neck were reviewed to better understand this rare entity.Case presentation:We reported a case of RIMFS under the left occipital scalp in a 20-year-old girl with a history of medulloblastoma surgery and radiotherapy in 2006.A total tumor resection was performed with preservation of the overlying scalp the underlying bone,and no adjuvant therapy was administered after the first operation.The postoperative pathological diagnosis was high-grade MFS.The tumor relapsed 6 months later,and then,a planned extensive resection with negative surgical margins was carried out,followed by radiotherapy.No relapse occurred in a 12-month postoperative follow-up.Conclusions:Planned gross total resection(GTR)with negative margins is the reasonable choice and footstone of other treatments for MFS.Ill-defined infiltrated borders and the complicated structures make it a great trouble to achieve total resection of MFS in the head and neck,so adjuvant radiotherapy and chemotherapy seem more necessary for these lesions.展开更多
文摘Introduction: Primary cardiac myxofibrosarcoma (MFS) is a rare and aggressive malignancy, with limited treatment options and a high recurrence rate. Multimodal imaging is critical for diagnosis, and surgery remains the primary treatment, though recurrence is common. Case Presentation: We present the case of a 41-year-old male with history of intermittent chest pain and palpitations. Initial work-up revealed a left atrial mass, which after thorough evaluation with multimodal imaging and histopathological assessment, was diagnosed as a primary cardiac myxofibrosarcoma. Despite complete surgical resection and low-grade histology, recurrence ensued, prompting reintervention and adjuvant radiotherapy. Discussion: Cardiac MFS poses significant diagnostic challenges. Multimodal imaging and immunohistochemistry are key to diagnosis. While surgery is the mainstay of treatment, high recurrence rates necessitate consideration of adjuvant therapies and long-term monitoring. Conclusion: This case highlights the importance of differential diagnosis of intracardiac masses, as well as reaching a consensus regarding the approach for malignant cardiac tumors.
文摘BACKGROUND Cardiac tumors are rare and complex entities.Surgery represents the cornerstone of therapy,while the role of adjuvant treatment remains unclear and,in case of relapse or metastatic disease,the prognosis is very poor.Lack of prospective,randomized clinical trials hinders the generation of high level evidence for the optimal diagnostic workup and multimodal treatment of cardiac sarcomas.Herein,we describe the multidisciplinary clinical management and molecular characterization of a rare case of cardiac myxofibrosarcoma in an elderly woman.CASE SUMMARY A 73-year-old woman presented signs and symptoms of acute left-sided heart failure.Imaging examination revealed a large,left atrial mass.With suspicion of a myxoma,she underwent surgery,and symptoms were promptly relieved.Histology showed a cardiac myxofibrosarcoma,a rare histotype of cardiac sarcoma.Eight months later,disease unfortunately relapsed,and after a multidisciplinary discussion,a chemotherapy with doxorubicin and then gemcitabine was started,achieving partial radiologic and complete metabolic response,which was maintained up to 2 years and is still present.This report is focused on the entire clinical path of our patient from diagnosis to follow-up,through surgery and strategies adopted at relapse.Moreover,due to their rarity,very little is known about the molecular landscape of myxofibrosarcomas.Thus,we also performed and described preliminary genome analysis of the tumor tissue to get further insight on mechanisms involved in tumor growth,and to possibly unveil new clinically actionable targets.CONCLUSION We report a case of cardiac myxofibrosarcoma that achieved a very good prognosis due to an integrated surgical,cardiac and oncologic treatment strategy.
文摘BACKGROUND A myxofibrosarcoma(MFS)is a malignant fibroblastic tumor that tends to occur in the lower and upper extremities.The reported incidence of head and neck MFSs is extremely rare.We report a 46-year-old male with“a neoplasm in the scalp”who was hospitalized and diagnosed with an MFS(highly malignant with massive necrotic lesions)based on histologic and immunohistochemistry evaluations.The magnetic resonance imaging manifestations did not demonstrate the“tail sign”mentioned in several studies,which resulted in a great challenge to establish an imaging diagnosis.The treatment plan is closely associated with the anatomic location and histologic grade,and more importantly,aggressive surgery and adjuvant radiotherapy may be helpful.Hence,we report the case and share some valuable information about the disease.CASE SUMMARY A 46-year-old male with“a neoplasm in the scalp for 6 mo”was hospitalized.Initially,the tumor was about the size of a soybean,without algesia or ulceration.The patient ignored the growth,did not seek treatment,and thus,did not receive treatment.Recently,the tumor increased to the size of an egg;there was no bleeding or algesia.His family history was unremarkable.No abnormalities were found upon laboratory testing,including routine hematologic,biochemistry,and tumor markers.Computed tomography showed an ovoid mass(6.25 cm×3.29 cm×3.09 cm in size)in the left frontal scalp with low density intermingled with equidense strips in adjacent areas of the scalp.Magnetic resonance imaging revealed a lesion with an irregular surface and an approximate size of 3.55 cm×6.34 cm in the left frontal region,with clear boundaries and visible separation.Adjacent areas of the skull were damaged and the dura mater was involved.Contrast enhancement showed an uneven enhancement pattern.Surgery was performed and postoperative adjuvant radiotherapy was administered to avoid recurrence or metastasis.The post-operative pathologic diagnosis confirmed an MFS.A repeat computed tomography scan showed no local recurrence or distant metastasis 19 mo post-operatively.CONCLUSION The case reported herein of MFS was demonstrated in an extremely rare location on the scalp and had atypical magnetic resonance imaging findings,which serves as a reminder to radiologists of the possibility of this diagnosis to assist in clinical treatment.Given the special anatomic location and the high malignant potential of this rare tumor,combined surgical and adjuvant radiotherapy should be considered to avoid local recurrence and distant metastasis.The significance of regular follow-up is strongly recommended to improve the long-term survival rate.
基金Supported by the Key Project of Jiangsu Province,China,No.ZD2022052。
文摘BACKGROUND Myxofibrosarcoma(MFS)is a fibroblast-derived sarcoma that mainly occurs in subcutaneous tissue.MFS rarely occurs in the gastrointestinal tract,especially in the esophagus.CASE SUMMARY A 79-year-old male patient was admitted to our hospital for dysphagia for a week.Computed tomography and electronic gastroscopy showed that a giant mass was located 30 cm from the incisor and extended to the cardia.There was incomplete esophageal stenosis.Endoscopic pathology showed spindle cell lesions,which were considered inflammatory myofibroblast like hyperplasia.Considering the strong demands of the patient and his family,and the fact that most inflammatory myofibroblast tumors are benign,we decided to perform endoscopic submucosal dissection(ESD)even if the tumor size was giant(9.0 cm×3.0 cm).Postoperative pathological examination resulted in a final diagnosis of MFS.MFS rarely occurs in the gastrointestinal tract,especially in the esophagus.Surgical resection and local adjuvant radiotherapy are the first choices to improve the prognosis.This case report firstly described the ESD for esophageal giant MFS.It suggests that ESD may be an alternative treatment for primary esophageal MFS.CONCLUSION This case report for the first time describe the successful treatment of a giant esophageal MFS by ESD,suggesting that ESD may be an alternative treatment for primary esophageal MFS,especially in elderly high-risk patients with obvious dysphagia symptoms.
文摘Here, we report the case of a 47-year-old male who presented with a painless palpable mass in the right shoulder. This extremity tumor was diagnosed as a high-grade myxofibrosarcoma after a wide excision. Simultaneously, a synchronous mesenteric mass was discovered, which proved to be a leiomyosarcoma.
文摘Soft tissue sarcomas represent only 1% of all adult cancers;myxofibrosarcoma is the most common type that arises in adult extremities, particularly lower limbs (77%), other less common locations are the trunk (12%) and neck (3%). Usually presenting as a painless, subcutaneous, slow growing mass with tendency for recurrence, they are prone to have higher histological grade and metastatic potential after recurrence;even in optimal multidisciplinary settings patients can have incomplete resections, making metastatic disease more common after misdiagnosis. We present the case of a 69-year-old male patient with a right infraescapular tumor, presenting as a painless 15 × 8 cm, mobile mass, with a slow but progressive growth, history of a previous tumor excised at the same location 5 years prior without histopathological report.
文摘Background:Myxofibrosarcoma(MFS),especially radiation-Induced MFS(RIMFS)in the head and neck,is an extremely rare malignant fibroblastic tumor.The diagnosis and treatment of MFS remain great challenges.In the present study,we presented one case of RIMFS.Combined with previous literature,the clinical features,essentials of diagnosis,and treatment modalities of MFS in the head and neck were reviewed to better understand this rare entity.Case presentation:We reported a case of RIMFS under the left occipital scalp in a 20-year-old girl with a history of medulloblastoma surgery and radiotherapy in 2006.A total tumor resection was performed with preservation of the overlying scalp the underlying bone,and no adjuvant therapy was administered after the first operation.The postoperative pathological diagnosis was high-grade MFS.The tumor relapsed 6 months later,and then,a planned extensive resection with negative surgical margins was carried out,followed by radiotherapy.No relapse occurred in a 12-month postoperative follow-up.Conclusions:Planned gross total resection(GTR)with negative margins is the reasonable choice and footstone of other treatments for MFS.Ill-defined infiltrated borders and the complicated structures make it a great trouble to achieve total resection of MFS in the head and neck,so adjuvant radiotherapy and chemotherapy seem more necessary for these lesions.
