Clinicopathological analysis of clinically occult extrapulmonary lymphangioleiomyomatosis in intra‐pelvic and para‐aortic lymph nodes associated with pelvic malignant tumors: A study of nine patients.
Clinicopathological analysis of clinically occult extrapulmonary lymphangioleiomyomatosis in intra‐pelvic and para‐aortic lymph nodes associated with pelvic malignant tumors: A study of nine patients.
复制标题
与盆腔恶性肿瘤相关的盆腔内和主动脉旁淋巴结临床隐匿性肺外淋巴管平滑肌瘤病的临床病理学分析：一项对 9 名患者的研究。
DOI：
10.1111/pin.12749
复制
发表时间：
2019
影响因子：
2.2
通讯作者：
Yukihiro Imai.
中科院分区：
文献类型：
--
作者：
Keiichiro Uehara;Fumi Kawakami;Takanori Hirose;Hiroki Morita;Eiji Kudo;Masanori Yasuda;Bruno Markl;Yoh Zen;Tomoo Itoh;Yukihiro Imai.
临床隐匿性肺外淋巴结淋巴血管平滑肌瘤病（LN - LAM）在盆腔恶性肿瘤手术分期中被清扫的临床病理和免疫组织化学特征尚未得到很好的研究。我们评估了9名女性患者（中位年龄61岁）的样本。没有结节性硬化症的病史或家族史，没有LN以外的LAM病变，如肺。原发恶性肿瘤包括4例子宫内膜样癌、1例子宫内膜癌肉瘤、3例卵巢浆液性癌和1例尿路上皮癌。中位随访时间为43个月。受影响的LNs数量从1到15（中位数，2），尺寸从1到13 mm（中位数，3.0）。6例临床隐匿LN - LAM仅存在于盆腔淋巴结，2例仅存在于腹主动脉旁淋巴结，1例同时存在于盆腔和腹主动脉旁淋巴结。免疫组织化学结果显示，9例LAM细胞均表现出β - catenin和E - cadherin强烈的弥漫性阳性。临床隐匿性LN - LAM主要影响围绝经期或绝经后妇女。在极少数情况下，隐匿性LN - LAM可能表现为全身性LAM，包括在肺部。β -连环蛋白和E -钙粘蛋白作为额外的诊断标记具有潜在的效用。
The clinicopathological and immunohistochemical characteristics of clinically occult extrapulmonary lymphangioleiomyomatosis in lymph nodes (LN‐LAM) being dissected during surgical staging of pelvic malignancy have not been well investigated. We assessed samples from nine female patients (median age, 61). None had past or familial history of tuberous sclerosis and had LAM lesions other than LN such as lung. The primary malignancies included four endometrial endometrioid carcinomas, one endometrial carcinosarcoma, three ovarian serous carcinomas and one urothelial carcinoma. Median follow‐up was 43 months. The number of affected LNs ranged from 1 to 15 (median, 2) with sizes ranging from 1 to 13 mm (median, 3.0). Six cases had clinically occult LN‐LAM only within the pelvic LNs, two only within para‐aortic LNs, and one within both pelvic and para‐aortic lymph nodes. Immunohistochemically, LAM cells exhibited a strong diffuse positivity for β‐catenin and E‐cadherin in all nine cases. Clinically occult LN‐LAM mainly affects peri‐ or post‐menopausal women. On rare occasions, occult LN‐LAM may manifest as systemic LAM, including in the lung. β‐catenin and E‐cadherin carry potential utility as additional diagnostic markers.