Leathersich Sebastian, Koay Mei Hui Eleanor, Khani Alireza, Malla Bhat Sangeeta
Department of Obstetrics and Gynaecology, King Edward Memorial Hospital for Women, Subiaco, Western Australia, Australia
Department of Obstetrics and Gynaecology, Armadale Health Service, Armadale, Western Australia, Australia.
BMJ Case Rep. 2019 Nov 7;12(11):e231683. doi: 10.1136/bcr-2019-231683.
We report a unique uterine neoplasm, favoured to represent an isolated extrapulmonary lymphangioleiomyoma with unusual pathological features, in a postmenopausal woman without tuberous sclerosis complex. The large neoplasm consisted of smooth muscle fascicles and cystic spaces lined by lymphatic cells, which were negative for the melanocytic staining that is characteristically positive in lymphangioleiomyomatosis (LAM). There are fewer than 30 cases of uterine LAM reported, none of which have demonstrated this morphology or these immunohistochemical findings. The origin of LAM cells in the more typical pulmonary LAM remains unclear; the unusual features in this case may represent a distinct pathological entity or a rare variant of typical extrapulmonary LAM, and may contribute to determining the cellular origin of these rare tumours. Conversely, this may represent a case of 'prepulmonary' LAM, providing supporting evidence for a possible gynaecological origin of these tumours in the broader affected (almost exclusively female) population.
我们报告了一例独特的子宫肿瘤，该肿瘤可能代表一种孤立的肺外淋巴管平滑肌瘤，具有不寻常的病理特征，患者为一名无结节性硬化症复合体的绝经后女性。这个大肿瘤由平滑肌束和由淋巴细胞内衬的囊性空间组成，这些细胞对淋巴管平滑肌瘤病（LAM）中典型呈阳性的黑素细胞染色呈阴性。据报道，子宫LAM病例少于30例，其中无一例表现出这种形态或这些免疫组化结果。在更典型的肺LAM中，LAM细胞的起源仍不清楚；该病例中的不寻常特征可能代表一种独特的病理实体或典型肺外LAM的罕见变体，并可能有助于确定这些罕见肿瘤的细胞起源。相反，这可能代表一例“肺前”LAM，为这些肿瘤在更广泛受影响（几乎全为女性）人群中可能的妇科起源提供了支持证据。
