Szpurek Dariusz, Szubert Sebastian, Zielinski Pawel, Frankowski Andrzej, Sajdak Stefan, Moszynski Rafal
Division of Gynecological Surgery, Poznan University of Medical Sciences, Poznan, Poland.
Division of Gynecological Surgery, Poznan University of Medical Sciences, Poznan, Poland.
Taiwan J Obstet Gynecol. 2015 Oct;54(5):603-7. doi: 10.1016/j.tjog.2015.08.015.
The main aim of this case report was to present the method of diagnosis, management, and the 12-year-follow-up of a patient diagnosed with primary uterine lymphangioleiomyomatosis (LAM).
A 47-year-old woman was admitted to the Department of Thoracosurgery due to pulmonary lesions suspected to be metastatic. The potential primary site of the neoplasm was not identified by previous imaging studies and specialist counseling. The patient had a history of total abdominal hysterectomy without ovaries due to a uterine tumor recognized as cellular leiomyoma and left salpingo-oophorectomy due to a solid ovarian tumor also recognized as leiomyoma. She had previously undergone the removal of a left kidney angiomyolipoma. After histopathological examination of the pulmonary lesions and repeated evaluation of the ovarian and uterine tumors, the diagnosis of primary uterine LAM with metastases to the ovary and the lungs was established. Although new metastatic lesions occurred, the patient remained in good condition during the 12-year-follow-up.
The history of our patient and review of the literature suggest that although uterine LAM presents malignant features (i.e., metastasis), the disease is long lasting and the patient can be in good condition for a number of years.
本病例报告的主要目的是介绍一名被诊断为原发性子宫淋巴管平滑肌瘤病（LAM）患者的诊断方法、治疗及12年随访情况。
一名47岁女性因疑似转移性肺部病变入住胸外科。先前的影像学检查和专家会诊未确定肿瘤的潜在原发部位。该患者因子宫肿瘤被诊断为细胞性平滑肌瘤接受了全腹子宫切除术（未切除卵巢），并因实性卵巢肿瘤也被诊断为平滑肌瘤接受了左侧输卵管卵巢切除术。她此前还接受过左侧肾血管平滑肌脂肪瘤切除术。经对肺部病变进行组织病理学检查以及对卵巢和子宫肿瘤进行反复评估后，确诊为原发性子宫LAM伴卵巢和肺转移。尽管出现了新的转移病灶，但患者在12年随访期间状况良好。
我们患者的病史及文献回顾表明，尽管子宫LAM具有恶性特征（即转移），但该疾病病程较长，患者可在数年内保持良好状态。
