Epithelioid sarcoma(ES), a mesenchymatous malign neoformation, is often diagnosed in later stages and associated with high recurrence index, metastasis and mortality. We report a case of a 65 years old male, with hist...Epithelioid sarcoma(ES), a mesenchymatous malign neoformation, is often diagnosed in later stages and associated with high recurrence index, metastasis and mortality. We report a case of a 65 years old male, with history of abdominal pain and upper gastrointestinal bleeding. Endoscopy demonstrated a posterior duodenal wall perforation communicating with a solid retroperitoneal neoformation. Endoscopic biopsy was performed, with a final report of ES. The patient was submitted for surgical palliation due to the tumor's unresectability. Retroperitoneal ES is an extremely rare condition with limited reports in the literature where guidelines for its optimal treatment are not well established.展开更多
Epithelioid sarcoma is a rare malignant soft tissue neoplasm commonly involving extremities of young adults and rarely occurring in the trunk area. It re-sembles a chronic inflammatory process and mimics benign reacti...Epithelioid sarcoma is a rare malignant soft tissue neoplasm commonly involving extremities of young adults and rarely occurring in the trunk area. It re-sembles a chronic inflammatory process and mimics benign reactive granuloma or other benign diseases. Despite its indolence and slow growth, the prognosis is poor with high recurrence and early lymph node spreading and hematogeneous distant metastasis. Involvement of the spine is extremely rare and diffi-cult to diagnose correctly in clinical presentation and pathology. We describe our experience a case of epi-thelioid sarcoma involving lumbar spine. A pre-ope- rative spine MRI showed a large solid and necrotic paraspinal mass which extended into the spinal canal with cord compression, and bone destruction. Final correct diagnosis was confirmed by immunohisto-chemical studies. When young adults present with a spinal lesion that has unusual large necrotic soft tissue mass, immunohistochemical studies are recommended for promptly determining the possibility of spinal epithelioid sarcoma.展开更多
Inflammatory myofibroblastic tumor(IMT)is a rare neoplasm with intermediate malignancy characterized by a propensity for recurrence but a low metastatic rate.Diagnostic challenges arise from the diverse pathological pre...Inflammatory myofibroblastic tumor(IMT)is a rare neoplasm with intermediate malignancy characterized by a propensity for recurrence but a low metastatic rate.Diagnostic challenges arise from the diverse pathological presentation,variable symptomatology,and lack of different imaging features.However,IMT is identified by the fusion of the anaplastic lymphoma kinase(ALK)gene,which is present in approximately 70%of cases,with various fusion partners,including ran-binding protein 2(RANBP2),which allows confirmation of the diagnosis.While surgery is the preferred approach for localized tumors,the optimal long-term treatment for advanced or metastatic disease is difficult to define.Targeted therapies are crucial for achieving sustained response to treatment within the context of genetic alteration in IMT.Crizotinib,an ALK tyrosine kinase inhibitor(TKI),was officially approved by the US Food and Drug Administration(FDA)in 2020 to treat IMT with ALK rearrangement.However,most patients face resistance and disease progression,requiring consideration of sequential treatments.Combining radiotherapy with targeted therapy appears to be beneficial in this indication.Early promising results have also been achieved with immunotherapy,indicating potential for combined therapy approaches.However,defined recommendations are still lacking.This review analyzes the available research on IMT,including genetic disorders and their impact on the course of the disease,data on the latest targeted therapy regimens and the possibility of developing immunotherapy in this indication,as well as summarizing general knowledge about prognostic and predictive factors,also in terms of resistance to systemic therapy.展开更多
基金Supported by Hospital Juarez de Mexico in Mexico City,Mexico
文摘Epithelioid sarcoma(ES), a mesenchymatous malign neoformation, is often diagnosed in later stages and associated with high recurrence index, metastasis and mortality. We report a case of a 65 years old male, with history of abdominal pain and upper gastrointestinal bleeding. Endoscopy demonstrated a posterior duodenal wall perforation communicating with a solid retroperitoneal neoformation. Endoscopic biopsy was performed, with a final report of ES. The patient was submitted for surgical palliation due to the tumor's unresectability. Retroperitoneal ES is an extremely rare condition with limited reports in the literature where guidelines for its optimal treatment are not well established.
文摘Epithelioid sarcoma is a rare malignant soft tissue neoplasm commonly involving extremities of young adults and rarely occurring in the trunk area. It re-sembles a chronic inflammatory process and mimics benign reactive granuloma or other benign diseases. Despite its indolence and slow growth, the prognosis is poor with high recurrence and early lymph node spreading and hematogeneous distant metastasis. Involvement of the spine is extremely rare and diffi-cult to diagnose correctly in clinical presentation and pathology. We describe our experience a case of epi-thelioid sarcoma involving lumbar spine. A pre-ope- rative spine MRI showed a large solid and necrotic paraspinal mass which extended into the spinal canal with cord compression, and bone destruction. Final correct diagnosis was confirmed by immunohisto-chemical studies. When young adults present with a spinal lesion that has unusual large necrotic soft tissue mass, immunohistochemical studies are recommended for promptly determining the possibility of spinal epithelioid sarcoma.
基金National Science Center 2019/35/O/NZ2/03761(AMC)。
文摘Inflammatory myofibroblastic tumor(IMT)is a rare neoplasm with intermediate malignancy characterized by a propensity for recurrence but a low metastatic rate.Diagnostic challenges arise from the diverse pathological presentation,variable symptomatology,and lack of different imaging features.However,IMT is identified by the fusion of the anaplastic lymphoma kinase(ALK)gene,which is present in approximately 70%of cases,with various fusion partners,including ran-binding protein 2(RANBP2),which allows confirmation of the diagnosis.While surgery is the preferred approach for localized tumors,the optimal long-term treatment for advanced or metastatic disease is difficult to define.Targeted therapies are crucial for achieving sustained response to treatment within the context of genetic alteration in IMT.Crizotinib,an ALK tyrosine kinase inhibitor(TKI),was officially approved by the US Food and Drug Administration(FDA)in 2020 to treat IMT with ALK rearrangement.However,most patients face resistance and disease progression,requiring consideration of sequential treatments.Combining radiotherapy with targeted therapy appears to be beneficial in this indication.Early promising results have also been achieved with immunotherapy,indicating potential for combined therapy approaches.However,defined recommendations are still lacking.This review analyzes the available research on IMT,including genetic disorders and their impact on the course of the disease,data on the latest targeted therapy regimens and the possibility of developing immunotherapy in this indication,as well as summarizing general knowledge about prognostic and predictive factors,also in terms of resistance to systemic therapy.