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磷酸尿基质肿瘤:单一机构治疗的 10 例患者的治疗和结局。

Phosphaturic mesenchymal tumor: management and outcomes of ten patients treated at a single institution.

机构信息

Division of Orthopaedic Oncology, Department of Orthopaedic Surgery, Massachusetts General Hospital and Harvard Medical School, Boston, MA, 02144, USA.

Department of Pathology, Massachusetts General Hospital and Harvard Medical School, Boston, MA, 02144, USA.

出版信息

Skeletal Radiol. 2024 Aug;53(8):1495-1506. doi: 10.1007/s00256-024-04614-6. Epub 2024 Feb 13.

Abstract

BACKGROUND

Phosphaturic mesenchymal tumor (PMT) is a rare tumor that causes tumor-induced osteomalacia. Patients present with non-specific symptoms secondary to renal phosphate wasting and decreased bone mineralization. We sought to assess: (1) What are the common presenting features, laboratory and imaging findings, histologic findings of phosphaturic mesenchymal tumors? (2) What are the available treatment strategies for phosphaturic mesenchymal tumors and their long-term outcomes in terms of local recurrence and symptom control after treatment?

METHODS

We retrospectively identified patients with a histologic diagnosis of PMT located in the axial or appendicular skeleton, or surrounding soft tissues. A total of 10 patients were finally included in our study.

RESULTS

Median tumor size was 1.9 cm (range, 1.1 to 6.1) and median time from symptom onset to diagnosis was 3 years (range, 0.5 to 15 years). All patients but one presented with hypophosphatemia (median 1.9 mg/dL, range 1.2 to 3.2). Pre-operative FGF-23 was elevated in all cases (median 423.5 RU/mL, range 235 to 8950). Six patients underwent surgical resection, three were treated percutaneously (radiofrequency ablation or cryoablation), and one refused treatment. Only one patient developed local recurrence and no patients developed metastatic disease. At last follow-up, nine patients showed no evidence of disease and one was alive with disease.

CONCLUSION

Phosphaturic mesenchymal tumor is a rare tumor presenting with non-specific symptoms. Surgery is the standard treatment when negative margins can be achieved without significant morbidity. In patients with small tumors in surgically-inaccessible areas, radiofrequency ablation or cryoablation can be performed successfully.

摘要

背景

磷酸化解偶联肿瘤(PMT)是一种罕见的肿瘤,可导致肿瘤性骨软化症。患者表现为肾磷酸盐丢失和骨矿物质化减少引起的非特异性症状。我们旨在评估:(1)磷酸化解偶联肿瘤的常见表现、实验室和影像学发现、组织学发现是什么?(2)磷酸化解偶联肿瘤的可用治疗策略及其在局部复发和治疗后症状控制方面的长期结果是什么?

方法

我们回顾性地确定了组织学诊断为位于轴骨或附肢骨骼或周围软组织中的 PMT 的患者。最终共有 10 名患者纳入我们的研究。

结果

肿瘤大小中位数为 1.9cm(范围 1.1 至 6.1),从症状出现到诊断的中位时间为 3 年(范围 0.5 至 15 年)。除 1 例患者外,所有患者均表现为低磷血症(中位数 1.9mg/dL,范围 1.2 至 3.2)。所有病例的 FGF-23 均升高(中位数 423.5RU/mL,范围 235 至 8950)。6 例患者接受了手术切除,3 例接受了经皮治疗(射频消融或冷冻消融),1 例拒绝治疗。仅 1 例患者发生局部复发,无患者发生转移性疾病。最后一次随访时,9 例患者无疾病证据,1 例患者仍有疾病存活。

结论

磷酸化解偶联肿瘤是一种罕见的肿瘤,表现为非特异性症状。当能够实现无显著发病率的阴性切缘时,手术是标准治疗方法。对于无法手术切除的小肿瘤患者,可以成功进行射频消融或冷冻消融。

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