BACKGROUND Malignant triton tumors(MTTs)comprise a subgroup of malignant peripheral nerve sheath tumors(MPNSTs)that exhibits rhabdomyosarcomatous differen-tiation and follow an aggressive course.MTTs are primarily loc...BACKGROUND Malignant triton tumors(MTTs)comprise a subgroup of malignant peripheral nerve sheath tumors(MPNSTs)that exhibits rhabdomyosarcomatous differen-tiation and follow an aggressive course.MTTs are primarily located along peripheral nerves.Cases of MTTs in the abdominal wall have not been reported.MTT has a poorer prognosis than classic MPNSTs,and accurate diagnosis necessitates a keen understanding of the clinical history and knowledge of its differential diagnosis intricacies.Treatment for MTTs mirrors that for MPNSTs and is predominantly surgical.CASE SUMMARY A 49-year-old woman presented with a subcutaneous mass in her lower abdo-minal wall and a pre-existing surgical scar that had grown slowly over 3-4 months before the consultation.She had previously undergone radical hysterectomy and concurrent chemo-radiotherapy for cervical cancer approximately 5 years prior to the consultation.Abdominal computed tomography(CT)showed a 1.3 cm midline mass in the lower abdomen with infiltration into the rectus abdominis muscle.There was no sign of metastasis(T1N0M0).An incisional biopsy identified sporadic MTT of the lower abdomen.A comprehensive surgical excision with a 3 cm margin inclusive of the peritoneum was executed.Subse-quently,the general surgeon utilized an approach akin to the open peritoneal onlay mesh technique.The patient underwent additional treatment with an excision shaped as a mini-abdominoplasty for the skin defect.No complications arose,and annual follow-up CTs did not show signs of recurrence or metastasis.CONCLUSION An abdominal MTT was efficaciously treated with extensive excision and abdominal wall reconstruction,eliminating the need for postoperative radiotherapy.展开更多
Malignant peripheral nerve sheath tumor(MPNST) is an uncommon sarcoma which arises from pluripotent stem cells of the neural crest and differentiates predominantly towards Schwann cells. Low grade spinal MPNST with sk...Malignant peripheral nerve sheath tumor(MPNST) is an uncommon sarcoma which arises from pluripotent stem cells of the neural crest and differentiates predominantly towards Schwann cells. Low grade spinal MPNST with skeletal muscle differentiation(malignant triton tumor) is vanishingly rare. In this study, we report a case of a 53-year-old female with a homogenously enhancing C2-C4 extradural lesion. The lesion demonstrated a biphasic histologic pattern with a diffusely infiltrating, atypical spindle cell component strongly positive for vimentin and focally positive for S-100. The second component consisted of diffusely scattered clusters of mature skeletal muscle cells which were positive for desmin, fast myosin and muscle specific actin but negative for Myogenin and Myo D-1. The Ki-67 labeling index was low(< 1%) and no necrosis was identified. The present case is remarkable because of its rare location, low grade histology and unusual immunophenotype ofthe skeletal muscle component, which were not previously described.展开更多
Malignant triton tumor (MTT) is a rare variant of malignant peripheral nerve sheath tumor (MPNST) with rhabdomyosarcomatous differentiation. We report the case of a 54-year-old male without a history of neurofibromato...Malignant triton tumor (MTT) is a rare variant of malignant peripheral nerve sheath tumor (MPNST) with rhabdomyosarcomatous differentiation. We report the case of a 54-year-old male without a history of neurofibromatosis type 1 (NF1) who had a growing abdominal wall tumor diagnosed as MTT. Computed tomography (CT), magnetic resonance imaging (MRI) and 2-[F-18]-fluoro-2-deoxy-D-glucose positron emission tomography/CT (FDG-PET/CT) were performed. The MRI and FDG-PET/CT indicated that the lateral component of the tumor was composed of many proliferative cells, corresponding to the histopathological finding of a cellular proliferation of spindle-shaped cells. In light of this case and previous reports, it is apparent that FDG-PET/CT is a helpful tool for distinguishing MTT from benign peripheral nerve sheath tumor.展开更多
Malignant peripheral nerve sheath tumor(MPNST)with rhabdomyoblastic differentiation is called as malignant triton tumor(MTT). It is highly aggressive soft tissue tumor with higher local recurrence rate. MTT has poor p...Malignant peripheral nerve sheath tumor(MPNST)with rhabdomyoblastic differentiation is called as malignant triton tumor(MTT). It is highly aggressive soft tissue tumor with higher local recurrence rate. MTT has poor prognosis than MPNST. MTT seems to be more aggressive in patients with neurofibromatosis(NF-1). We herein, reporting an interesting case of 55 years male with multiple neurofibromas all over the body since 30 years and multiple café-au-lait spots, diagnosed as NF-1. Since 6 years, he had an enlarged mass in left thigh. Wide excision of mass was done. On histopathological examination revealed the diagnosis of MTT and diagnosis of which was confirmed on immunohistochemistry.展开更多
I型神经纤维瘤﹙neurofibromatosis type I,NF1﹚是由神经鞘细胞及纤维母细胞两种成分组成的良性肿瘤,其恶变者报道较少。我院收治1例腮腺区I型神经纤维瘤恶变患者。患者在神经纤维瘤病基础上发生恶变,成为恶性神经鞘瘤,肿块巨大,部位特...I型神经纤维瘤﹙neurofibromatosis type I,NF1﹚是由神经鞘细胞及纤维母细胞两种成分组成的良性肿瘤,其恶变者报道较少。我院收治1例腮腺区I型神经纤维瘤恶变患者。患者在神经纤维瘤病基础上发生恶变,成为恶性神经鞘瘤,肿块巨大,部位特殊,手术难度高,经术后病理已确诊。本文对此病例临床资料进行了详细报道,并结合文献对其诊治要点进行了回顾。展开更多
文摘BACKGROUND Malignant triton tumors(MTTs)comprise a subgroup of malignant peripheral nerve sheath tumors(MPNSTs)that exhibits rhabdomyosarcomatous differen-tiation and follow an aggressive course.MTTs are primarily located along peripheral nerves.Cases of MTTs in the abdominal wall have not been reported.MTT has a poorer prognosis than classic MPNSTs,and accurate diagnosis necessitates a keen understanding of the clinical history and knowledge of its differential diagnosis intricacies.Treatment for MTTs mirrors that for MPNSTs and is predominantly surgical.CASE SUMMARY A 49-year-old woman presented with a subcutaneous mass in her lower abdo-minal wall and a pre-existing surgical scar that had grown slowly over 3-4 months before the consultation.She had previously undergone radical hysterectomy and concurrent chemo-radiotherapy for cervical cancer approximately 5 years prior to the consultation.Abdominal computed tomography(CT)showed a 1.3 cm midline mass in the lower abdomen with infiltration into the rectus abdominis muscle.There was no sign of metastasis(T1N0M0).An incisional biopsy identified sporadic MTT of the lower abdomen.A comprehensive surgical excision with a 3 cm margin inclusive of the peritoneum was executed.Subse-quently,the general surgeon utilized an approach akin to the open peritoneal onlay mesh technique.The patient underwent additional treatment with an excision shaped as a mini-abdominoplasty for the skin defect.No complications arose,and annual follow-up CTs did not show signs of recurrence or metastasis.CONCLUSION An abdominal MTT was efficaciously treated with extensive excision and abdominal wall reconstruction,eliminating the need for postoperative radiotherapy.
文摘Malignant peripheral nerve sheath tumor(MPNST) is an uncommon sarcoma which arises from pluripotent stem cells of the neural crest and differentiates predominantly towards Schwann cells. Low grade spinal MPNST with skeletal muscle differentiation(malignant triton tumor) is vanishingly rare. In this study, we report a case of a 53-year-old female with a homogenously enhancing C2-C4 extradural lesion. The lesion demonstrated a biphasic histologic pattern with a diffusely infiltrating, atypical spindle cell component strongly positive for vimentin and focally positive for S-100. The second component consisted of diffusely scattered clusters of mature skeletal muscle cells which were positive for desmin, fast myosin and muscle specific actin but negative for Myogenin and Myo D-1. The Ki-67 labeling index was low(< 1%) and no necrosis was identified. The present case is remarkable because of its rare location, low grade histology and unusual immunophenotype ofthe skeletal muscle component, which were not previously described.
文摘Malignant triton tumor (MTT) is a rare variant of malignant peripheral nerve sheath tumor (MPNST) with rhabdomyosarcomatous differentiation. We report the case of a 54-year-old male without a history of neurofibromatosis type 1 (NF1) who had a growing abdominal wall tumor diagnosed as MTT. Computed tomography (CT), magnetic resonance imaging (MRI) and 2-[F-18]-fluoro-2-deoxy-D-glucose positron emission tomography/CT (FDG-PET/CT) were performed. The MRI and FDG-PET/CT indicated that the lateral component of the tumor was composed of many proliferative cells, corresponding to the histopathological finding of a cellular proliferation of spindle-shaped cells. In light of this case and previous reports, it is apparent that FDG-PET/CT is a helpful tool for distinguishing MTT from benign peripheral nerve sheath tumor.
文摘Malignant peripheral nerve sheath tumor(MPNST)with rhabdomyoblastic differentiation is called as malignant triton tumor(MTT). It is highly aggressive soft tissue tumor with higher local recurrence rate. MTT has poor prognosis than MPNST. MTT seems to be more aggressive in patients with neurofibromatosis(NF-1). We herein, reporting an interesting case of 55 years male with multiple neurofibromas all over the body since 30 years and multiple café-au-lait spots, diagnosed as NF-1. Since 6 years, he had an enlarged mass in left thigh. Wide excision of mass was done. On histopathological examination revealed the diagnosis of MTT and diagnosis of which was confirmed on immunohistochemistry.
文摘I型神经纤维瘤﹙neurofibromatosis type I,NF1﹚是由神经鞘细胞及纤维母细胞两种成分组成的良性肿瘤,其恶变者报道较少。我院收治1例腮腺区I型神经纤维瘤恶变患者。患者在神经纤维瘤病基础上发生恶变,成为恶性神经鞘瘤,肿块巨大,部位特殊,手术难度高,经术后病理已确诊。本文对此病例临床资料进行了详细报道,并结合文献对其诊治要点进行了回顾。
