RESUMEN

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.

Asunto(s)

Linfangioleiomiomatosis/diagnóstico , Linfangiomioma/patología , Neoplasias Uterinas/patología , Útero/patología , Anciano , Diagnóstico Diferencial , Femenino , Humanos , Histerectomía , Neoplasias Pulmonares/patología , Linfangiomioma/cirugía , Posmenopausia , Salpingooforectomía , Neoplasias Uterinas/cirugía , Útero/cirugía

RESUMEN

Granular cell tumours (GCT) are uncommon, usually solitary tumours of neural/Schwann cell origin that occur at any site of the body, and typically run an indolent clinical course. Treatment by excision is recommended. Distant or nodal metastases are the only reliable signs of malignancy. We describe the case of a 47-year-old woman with a multi-focal, multi-centric GCT involving the pulmonary and gastrointestinal systems, highlighting the imaging and pathological features and the challenge faced in establishing its malignant potential.