Extrapulmonary uterine lymphangioleiomyomatosis (LAM) and dysfunctional uterine bleeding: the first presentation of LAM in a tuberous sclerosis complex patient. Issue 2 (25th February 2019)

• Record Type: Journal Article
• Authors: Grant, Lucy; Chipwete, Saliya; Soo Hoo, San; Bhatnagar, Anjali
• Is Part Of: BMJ case reports. Volume 12:Issue 2(2019)
• Publication Date: 2019-02-25
• Subjects: obstetrics and gynaecology -- pathology; Medicine -- Case studies -- Periodicals; 610.5
• DOI: 10.1136/bcr-2018-226358
• Languages: English
• ISSNs: 1757-790X

Abstract

Abstract : Lymphangioleiomyomatosis (LAM) is a rare disease that typically affects women of childbearing age. It most commonly affects the lungs (P-LAM) but can occasionally occur in extra-pulmonary sites (E-LAM). There is a strong association between LAM and the tuberous sclerosis complex (TSC). We report a case of a 42-year-old female TSC sufferer who presented with dysfunctional uterine bleeding. She was not known to have LAM. An endometrial biopsy revealed a spindled-cell lesion suspicious of leiomyosarcoma, which correlated with cross-sectional imaging. She underwent a hysterectomy that showed a bizarre (symplastic) leiomyomatous endometrial polyp with background uterine LAM. We discuss the clinical and pathological implications of this unusual case of E-LAM and the importance of clinicopathological correlation in TSC sufferers. The association of uterine LAM with TSC is important and LAM should be considered as a differential of dysfunctional uterine bleeding and a benign mimic to uterine leiomyosarcoma in patients with TSC.