血浆促肾上腺皮质激素水平升高的肾原始神经外胚层肿瘤：一例报告。
Renal primitive neuroectodermal tumor with elevated plasma adrenocorticotropic hormone levels: A case report.
作者信息
Shimizu Nobuaki, Hasumi Masaru, Hamano Tatsuya, Iijima Misa, Yoshioka Takako, Yamazaki Yuto, Sasano Hironobu
机构信息
Department of Urology Gunma Prefectural Cancer Center Ota Gunma Japan.
Department of Urology Chichibu Municipal Hospital Chichibu Saitama Japan.
出版信息
IJU Case Rep. 2019 Mar 6;2(3):128-131. doi: 10.1002/iju5.12057. eCollection 2019 May.
INTRODUCTION
Primitive neuroectodermal tumors are small round-cell tumors - Ewing sarcoma family, frequently occurring in the extremities, but rarely in the kidney.
CASE PRESENTATION
A 58-year-old woman presented with whole-body edema and weakness of lower limb muscles. Computed tomography revealed a left renal tumor, and the plasma adrenocorticotropic hormone level was elevated. The tumor was surgically removed without complications, her plasma adrenocorticotropic hormone reverted to normal levels, and symptoms disappeared after surgery. Histopathological examination revealed a primitive neuroectodermal tumor arising in her kidney. The patient was alive without metastasis 3 years after the surgery.
CONCLUSION
We report the first case of renal primitive neuroectodermal tumor accompanying elevated plasma adrenocorticotropic hormone levels which are thought to be produced and secreted in an ectopic fashion.
引言
原始神经外胚层肿瘤是小圆细胞肿瘤——尤因肉瘤家族，常发生于四肢，但很少发生于肾脏。
病例报告
一名58岁女性出现全身水肿和下肢肌肉无力。计算机断层扫描显示左肾肿瘤，血浆促肾上腺皮质激素水平升高。肿瘤经手术切除，无并发症，术后其血浆促肾上腺皮质激素恢复正常水平，症状消失。组织病理学检查显示为起源于其肾脏的原始神经外胚层肿瘤。术后3年患者存活，无转移。
结论
我们报告了首例伴有血浆促肾上腺皮质激素水平升高的肾原始神经外胚层肿瘤病例，这种升高被认为是以异位方式产生和分泌的。
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