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肾混合性上皮和间质肿瘤(MEST):14例男性及PEComa样变异型病例报告及组织病理学发生机制探讨

Mixed epithelial and stromal tumour (MEST) of the kidney: report of 14 cases with male and PEComatous variants and proposed histopathogenesis.

作者信息

Mai Kien T, Elkeilani Akram, Veinot John P

机构信息

Division of Anatomical Pathology, Department of Laboratory Medicine, The Ottawa Hospital, and Department of Pathology and Laboratory Medicine, University of Ottawa, Ontario, Canada.

出版信息

Pathology. 2007 Apr;39(2):235-40. doi: 10.1080/00313020701230799.

Abstract

AIMS

This article adds new cases and variants of MEST with discussion of the histopathogenesis.

METHODS AND RESULTS

Fourteen MEST were originally diagnosed as cystic nephroma which represents an incidence of 1.6% of renal neoplasms in adults. In females, the stromal component showed areas of müllerian differentiation with positive immunoreactivity for oestrogen (ER) and progesterone receptors (PR) and CD10. Immunoreactivity for HMB45 was identified in a single case having a leiomyomatous appearance. The epithelial component displayed features of müllerian epithelium and reactive renal tubular cells. In two male cases, MEST consisted of fibrous and smooth muscle stroma and cysts lined only by reactive renal tubular cells. Immunoreactivity for ER and PR was focal and weak.

CONCLUSIONS

MEST represents a tumour developing from müllerian-like stromal cells in the kidney. The neoplastic stroma encroaches on the renal tubules and has the potential to stimulate the growth of the renal tubules by contact, with development into cysts. Furthermore, the müllerian stroma likely induces the renal tubules to differentiate into müllerian-like epithelium. Melanocytic differentiation of the stroma may occur which represents the PEComatous variant. MESTs in males were histopathologically slightly different from those in females due to the different hormonal milieu.

摘要

目的

本文增加了混合性上皮和间质肿瘤(MEST)的新病例及变异类型,并对其组织病理发生机制进行了讨论。

方法与结果

14例MEST最初被诊断为囊性肾瘤,占成人肾肿瘤的1.6%。在女性患者中,间质成分显示出苗勒氏分化区域,雌激素(ER)、孕激素受体(PR)和CD10免疫反应呈阳性。在1例具有平滑肌瘤样外观的病例中检测到HMB45免疫反应。上皮成分表现出苗勒氏上皮和反应性肾小管细胞的特征。在2例男性病例中,MEST由纤维和平滑肌间质以及仅由反应性肾小管细胞衬里的囊肿组成。ER和PR的免疫反应呈局灶性且较弱。

结论

MEST是一种起源于肾脏中苗勒氏样间质细胞的肿瘤。肿瘤性间质侵犯肾小管,并有可能通过接触刺激肾小管生长,进而发展为囊肿。此外,苗勒氏间质可能诱导肾小管分化为苗勒氏样上皮。间质的黑素细胞分化可能会发生,这代表了PEComa变异型。由于激素环境不同,男性的MEST在组织病理学上与女性的略有不同。

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