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
Primary tumors of the diaphragm are quite rare. About 150 cases have been reported in the literature. Fibrosarcomas are the commonest malignant neoplasms of the diaphragm; however, only a few(less than 20) cases have ...Primary tumors of the diaphragm are quite rare. About 150 cases have been reported in the literature. Fibrosarcomas are the commonest malignant neoplasms of the diaphragm; however, only a few(less than 20) cases have been reported to date. We present the case of an extremely rare tumor of the diaphragm mimicking a liver mass. The patient, a young 28-year-old woman, presented with an 8-month-history of mildly progressive upper abdominal pain and early fullness after meals. Computed tomography scan of the abdomen revealed a mass located in the region of the left lobe of the liver with non visualized left lobe and partial vascular supply of the mass from the left hepatic artery. The tumor was also seen to draw its vascularity from bilateral internal mammary arteries. Surgical excision and hepatectomy was planned, keeping in mind the diagnosis of an atypical left hepatic mass. Laparotomy revealed a left diaphragmatic tumor growing caudally into the upper abdomen, compressing and splaying the liver along the left medial surface where the tumorwas virtually adherent to it. Successful excision of the mass and subsequent histopathological and immunochemistry examination of the specimen revealed low grade fibromyxoid sarcoma of the diaphragm. This case highlights the unusual presentation of a diaphragmatic mesenchymal tumor and how it can be mistaken as an atypical liver mass. It also emphasizes the tumoral vascular supply as an indicator of its organ of origin.展开更多
Introduction: Hyalinizing spindle cell tumor with giant rosettes (HSCT) is a very uncommon mesenchymal tumor that has similar morphological and biological features to the low-grade fibromyxoid sarcoma (LGFMS). Case Re...Introduction: Hyalinizing spindle cell tumor with giant rosettes (HSCT) is a very uncommon mesenchymal tumor that has similar morphological and biological features to the low-grade fibromyxoid sarcoma (LGFMS). Case Report: Reported herein is a case of primary tumour of buttock HSCT that had rare FUS-CREB3L1 fusion transcripts, a product of characteristic chromosomal abnormality t (7;16) (q33;p11) of HSCT and LGFMS. The patient was a 48-year-old man who had a large solitary mass in the buttock. Histologically, it was composed of bland spindle cells with variable cellularity deposited in a densely hyalinized stroma alternating with myxoid areas. Characteristic collagen rosettes were scattered in the cellular areas. Reverse transcription-polymerase chain reaction (RT-PCR) assay using formalin-fixed, paraffin-embedded tissue detected FUS-CREB3L1 fusion transcripts. In our knowledge is the second case may display a variant FUS/CREB3L1 fusion transcript in international literature. Conclusion: LGFMS and HSCT probably have a wider spectrum of morphologic features than previously thought, the awareness of which will help pathologists to avoid diagnostic pitfalls. Demonstration of the t (7;16) translocation will help to diagnose difficult cases with unusual histologic features.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Primary tumors of the diaphragm are quite rare. About 150 cases have been reported in the literature. Fibrosarcomas are the commonest malignant neoplasms of the diaphragm; however, only a few(less than 20) cases have been reported to date. We present the case of an extremely rare tumor of the diaphragm mimicking a liver mass. The patient, a young 28-year-old woman, presented with an 8-month-history of mildly progressive upper abdominal pain and early fullness after meals. Computed tomography scan of the abdomen revealed a mass located in the region of the left lobe of the liver with non visualized left lobe and partial vascular supply of the mass from the left hepatic artery. The tumor was also seen to draw its vascularity from bilateral internal mammary arteries. Surgical excision and hepatectomy was planned, keeping in mind the diagnosis of an atypical left hepatic mass. Laparotomy revealed a left diaphragmatic tumor growing caudally into the upper abdomen, compressing and splaying the liver along the left medial surface where the tumorwas virtually adherent to it. Successful excision of the mass and subsequent histopathological and immunochemistry examination of the specimen revealed low grade fibromyxoid sarcoma of the diaphragm. This case highlights the unusual presentation of a diaphragmatic mesenchymal tumor and how it can be mistaken as an atypical liver mass. It also emphasizes the tumoral vascular supply as an indicator of its organ of origin.
文摘Introduction: Hyalinizing spindle cell tumor with giant rosettes (HSCT) is a very uncommon mesenchymal tumor that has similar morphological and biological features to the low-grade fibromyxoid sarcoma (LGFMS). Case Report: Reported herein is a case of primary tumour of buttock HSCT that had rare FUS-CREB3L1 fusion transcripts, a product of characteristic chromosomal abnormality t (7;16) (q33;p11) of HSCT and LGFMS. The patient was a 48-year-old man who had a large solitary mass in the buttock. Histologically, it was composed of bland spindle cells with variable cellularity deposited in a densely hyalinized stroma alternating with myxoid areas. Characteristic collagen rosettes were scattered in the cellular areas. Reverse transcription-polymerase chain reaction (RT-PCR) assay using formalin-fixed, paraffin-embedded tissue detected FUS-CREB3L1 fusion transcripts. In our knowledge is the second case may display a variant FUS/CREB3L1 fusion transcript in international literature. Conclusion: LGFMS and HSCT probably have a wider spectrum of morphologic features than previously thought, the awareness of which will help pathologists to avoid diagnostic pitfalls. Demonstration of the t (7;16) translocation will help to diagnose difficult cases with unusual histologic features.
