PDF(Pubmed)
Abstract：
UNASSIGNED: Calcifying nested stromal epithelial tumor (CNSET) is an extremely rare diagnosis among patients treated for primary hepatic neoplasms. There are only 45 cases reported worldwide. Histopathological characteristics are well-demarcated nests of spindle and epithelioid cells in a dense desmoplastic stroma with variable calcification and ossification. It is mostly diagnosed in children and young females. Treatment strategies implemented for the management of CNSET include radiofrequency ablation, transarterial chemoembolization, surgical resection, adjuvant and neoadjuvant chemotherapy, and liver transplantation. Given the small number of available cases, there are still no established standards of treatment for this neoplasm.
UNASSIGNED: A 28-year-old female diagnosed with CNSET presented mild abdominal pain, with normal laboratory values. The tumor was initially deemed unresectable, therefore, the patient was disqualified from liver resection. Further deterioration of the patient\'s clinical condition and local tumor progression led to qualification for liver transplantation. The patient underwent liver transplantation 1 year following initial diagnosis and a 12 months recurrence-free period was observed. During the course of treatment, she did not receive systemic chemotherapy, radiotherapy, or loco-regional treatment.
UNASSIGNED: Multiple strategies have been implemented for the treatment of CNSET, with liver resection providing the best outcomes. Transarterial chemoembolization, radiofrequency ablation, and radiotherapy are reported to be insufficient in the management of this tumor. Various chemotherapy regimens turned out to be ineffective as well. There have been only eight reported cases of patients undergoing liver transplantation for CNSET, with tumor recurrence in two cases. CNSET appears to be a neoplasm with low malignancy potential, although an aggressive progression has subsequently been reported. Further investigation is still required in this field.
UNASSIGNED: A 28-year-old female diagnosed with CNSET presented mild abdominal pain, with normal laboratory values. The tumor was initially deemed unresectable, therefore, the patient was disqualified from liver resection. Further deterioration of the patient\'s clinical condition and local tumor progression led to qualification for liver transplantation. The patient underwent liver transplantation 1 year following initial diagnosis and a 12 months recurrence-free period was observed. During the course of treatment, she did not receive systemic chemotherapy, radiotherapy, or loco-regional treatment.
UNASSIGNED: Multiple strategies have been implemented for the treatment of CNSET, with liver resection providing the best outcomes. Transarterial chemoembolization, radiofrequency ablation, and radiotherapy are reported to be insufficient in the management of this tumor. Various chemotherapy regimens turned out to be ineffective as well. There have been only eight reported cases of patients undergoing liver transplantation for CNSET, with tumor recurrence in two cases. CNSET appears to be a neoplasm with low malignancy potential, although an aggressive progression has subsequently been reported. Further investigation is still required in this field.
摘要：
未经证实：钙化性巢式间质上皮肿瘤（CNSET）是原发性肝肿瘤治疗患者中极为罕见的诊断。全世界仅报告了45例病例。组织病理学特征是在致密的纤维增生性基质中，纺锤体和上皮样细胞的巢状分明，钙化和骨化可变。它主要被诊断为儿童和年轻女性。为CNSET的管理实施的治疗策略包括射频消融，经动脉化疗栓塞,手术切除,辅助和新辅助化疗，和肝移植。鉴于现有案例数量少，对于这种肿瘤，目前还没有确定的治疗标准。
未经证实：一名被诊断患有CNSET的28岁女性出现轻度腹痛，正常的实验室值。肿瘤最初被认为是不可切除的，因此,患者被取消肝切除术的资格.患者的临床状况和局部肿瘤进展的进一步恶化导致肝移植的资格。该患者在初步诊断后1年接受了肝移植，观察到12个月的无复发期。在治疗过程中,她没有接受全身化疗,放射治疗,或局部治疗。
UNASSIGNED：已经实施了多种治疗CNSET的策略，肝切除术提供了最好的结果。经动脉化疗栓塞,射频消融,据报道，放射疗法不足以治疗该肿瘤。各种化疗方案也被证明是无效的。仅有8例报道接受CNSET肝移植的患者，肿瘤复发2例。CNSET似乎是一种恶性潜能低的肿瘤，尽管随后报道了积极的进展。这一领域仍需进一步调查。
未经证实：一名被诊断患有CNSET的28岁女性出现轻度腹痛，正常的实验室值。肿瘤最初被认为是不可切除的，因此,患者被取消肝切除术的资格.患者的临床状况和局部肿瘤进展的进一步恶化导致肝移植的资格。该患者在初步诊断后1年接受了肝移植，观察到12个月的无复发期。在治疗过程中,她没有接受全身化疗,放射治疗,或局部治疗。
UNASSIGNED：已经实施了多种治疗CNSET的策略，肝切除术提供了最好的结果。经动脉化疗栓塞,射频消融,据报道，放射疗法不足以治疗该肿瘤。各种化疗方案也被证明是无效的。仅有8例报道接受CNSET肝移植的患者，肿瘤复发2例。CNSET似乎是一种恶性潜能低的肿瘤，尽管随后报道了积极的进展。这一领域仍需进一步调查。
