首例胰腺周围血管上皮样细胞瘤（PEComa）采用 mTOR 抑制剂治疗。
First pancreatic perivascular epithelioid cell tumor (PEComa) treated by mTOR inhibitor.
机构信息
Centre Hospitalier Universitaire de Nantes, Institut des Maladies de l'Appareil Digestif, Nantes, France.
Centre Hospitalier Universitaire de Nantes, Service d'Oncologie Pédiatrique, Nantes, France.
出版信息
Pancreatology. 2019 Jun;19(4):566-568. doi: 10.1016/j.pan.2019.05.459. Epub 2019 May 18.
BACKGROUND
Perivascular epithelioid cell tumor, an extremely rare mesenchymal tumor, could be ubiquitous but rarely arises from pancreas. Surgery is considered the most appropriate treatment. Nevertheless, activation of mTOR pathway seems to be a common pathogenic event in PEComas paving the way to chemotherapy by mTOR inhibitor.
METHOD
A 17 year-old man presented a hypervascular tumor of 55 mm, located in the head of pancreas without bile duct or pancreatic duct compression.
RESULTS
Histopathology showed epithelioid cells with clear or focally granular eosinophilic cytoplasm with melanocytic (HMB-45, Melan-A) and myoid markers which confirmed diagnosis of PEComa. Given the absence of worrisome feature, we ruled out surgery and decided to initiate treatment with Sirolimus, an mTOR inhibitor. After 3.5 years, we showed a significant reduction in size of the tumor.
CONCLUSION
This first case of pancreatic PEComa treated by mTOR inhibitor without surgery suggests a good efficiency of this therapy.
背景
血管周上皮样细胞瘤是一种极其罕见的间叶性肿瘤，可能无处不在，但很少发生在胰腺。手术被认为是最适当的治疗方法。然而，mTOR 通路的激活似乎是 PEComas 的一个常见发病事件，为通过 mTOR 抑制剂进行化疗铺平了道路。
方法
一名 17 岁男性出现一个 55mm 的富血管性肿瘤，位于胰腺头部，没有胆管或胰管受压。
结果
组织病理学显示上皮样细胞，具有透明或局灶性颗粒状嗜酸性细胞质，伴有黑色素细胞（HMB-45、Melan-A）和肌样标志物，这证实了 PEComa 的诊断。鉴于没有令人担忧的特征，我们排除了手术，并决定开始使用 mTOR 抑制剂西罗莫司进行治疗。3.5 年后，我们显示肿瘤的大小显著缩小。
结论
首例未经手术治疗的胰腺 PEComa 采用 mTOR 抑制剂治疗的案例提示这种治疗方法具有良好的疗效。
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