Suzuki Kensuke, Nagasaka Kazunori, Oda Katsutoshi, Abe Hiroyuki, Maeda Daichi, Matsumoto Yoko, Arimoto Takahide, Kawana Kei, Fukayama Masashi, Osuga Yutaka, Fujii Tomoyuki
Department of Obstetrics and Gynecology, Faculty of Medicine, The University of Tokyo, Tokyo, Japan.
Department of Pathology, Faculty of Medicine, The University of Tokyo, Tokyo, Japan.
BMC Cancer. 2016 Jul 4;16:390. doi: 10.1186/s12885-016-2413-z.
Lymphangioleiomyomatosis (LAM) is a rare idiopathic disorder that occurs in women of childbearing age, and consists of a diffuse proliferation of abnormal smooth muscle cells along the thoracic and abdominal lymphogenous route.
We experienced a case of a 47-yo woman with recent history of systemic lupus erythematosus (SLE) diagnosed with endometrial cancer, initially suspected to have metastasized to pelvic and para-aortic lymph nodes based on preoperative diagnostic imaging. Subsequent pathological diagnosis revealed stage IB endometrial cancer without evidence of lymph node involvement. Instead, enlarged pelvic and para-aortic lymph nodes were found to be due to extrapulmonary LAM, from a primary lesion found inside the uterine myometrium. SLE improved after surgery.
This is the first reported case of comorbid endometrial cancer, SLE, and aggressive LAM metastasizing to regional lymph nodes, and strengthens the clinical evidence for a common role of mTOR pathway hyperactivity and estrogen responsiveness in the pathophysiology of metastasizing lesions of the genital tract.
淋巴管平滑肌瘤病（LAM）是一种罕见的特发性疾病，发生于育龄女性，由异常平滑肌细胞沿胸腹部淋巴途径弥漫性增殖组成。
我们遇到一例47岁女性，近期有系统性红斑狼疮（SLE）病史，被诊断为子宫内膜癌，术前诊断性影像学检查最初怀疑已转移至盆腔和腹主动脉旁淋巴结。随后的病理诊断显示为ⅠB期子宫内膜癌，无淋巴结受累证据。相反，发现盆腔和腹主动脉旁淋巴结肿大是由于肺外LAM，其原发灶位于子宫肌层内。术后SLE病情改善。
这是首例合并子宫内膜癌、SLE且侵袭性LAM转移至区域淋巴结的病例报告，强化了mTOR通路过度活跃和雌激素反应性在生殖道转移病变病理生理学中共同作用的临床证据。
