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病例学习(Number 23)

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楼主 发表于 2011-11-26 16:27|举报|关注(0)
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欢迎讨论:
1.子宫LAM和其它发病部位相互伴随发病的关系?尤其是肺。如果二者同时发生?我们如何看待这两个问题?
2.子宫sporadic LAM 和TSC-LAM在HE形态上有哪些细微差别?及免疫组化在二者之间的差别?
3.什么情况下我们诊断为PEComa,而什么情况下我们又诊断为LAM?二者在HE上有无差别?
4.由于sporadic LAM 的存在,是否提示我们在取材过程中,提高警惕,以免漏过?
5.梭形细胞,上皮样细胞,血管外皮瘤样结构(裂隙样腔隙)的数量在鉴别sporadic LAM ,TSC-LAM中的作用,甚至在归类于PEComa大家族中有无特征性诊断意义?
6.我们有无必要将子宫LAM从PEComa家族中细分出?
7.欢迎大家讨论,由于文章内容太大,无法上传,有感兴趣的同道留下邮箱给我,将原文发送一起讨论

Prevalence of Uterine and Adnexal Involvement in Pulmonary Lymphangioleiomyomatosis: A Clinicopathologic Study of 10 Patients
(Am J Surg Pathol 2011;35:1776–1785)

Abstract: Lymphangioleiomyomatosis (LAM), a systemic disorder
affecting almost exclusively young women, is characterized
by the abnormal proliferation of smooth muscle-like cells (LAM
cells). LAM can occur either in association with the tuberous
sclerosis complex (TSC) (TSC-LAM) or without TSC (sporadic
LAM). Recent studies have demonstrated that LAM is a
neoplasm arising from constitutive activation of the mammalian
target of rapamycin signaling pathway dysregulated by a
functional loss of TSC genes, but the primary organ of origin
remains unclear. Therefore, we performed histologic and
immunohistologic analyses of gynecologic organs in 20 patients,
half with and the other half without pulmonary LAM, to
determine how often LAM involves the uterus. The results
showed that 9 of 10 (90%) patients with pulmonary LAM had
uterine LAM lesions. In contrast, no patients without pulmonary
LAM had so. All uterine LAM lesions were accompanied by
LAM lesions in retroperitoneal or pelvic lymph nodes and LAM
cell clusters, each enveloped by a monolayer of vascular
endothelial growth factor receptor-3–positive lymphatic endothelial
cells. Furthermore, when we compared uterine lesions of
TSC-LAM with those of sporadic LAM, proliferation of
HMB45-positive epithelioid-shaped LAM cells and infiltrates
with a tongue-like growth pattern was more prominent in the
former, whereas the extent of lymphangiogenesis within the
myometrium was greater in the latter. These results indicate that
uterine involvement is a common manifestation of LAM, and,
possibly, that the uterus or an adjacent locale in the retroperitoneum
or pelvic cavity is the primary site of origin of LAM.

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