J Med Cases

Journal of Medical Cases, ISSN 1923-4155 print, 1923-4163 online, Open Access

Article copyright, the authors; Journal compilation copyright, J Med Cases and Elmer Press Inc

Journal website http://www.journalmc.org

Case Report

Volume 10, Number 7, July 2019, pages 218-221

Lymphangioleiomyomatosis: A Challenging Case

Figures

Figure 1. Mediastinal lesion biopsy: areas with vascular structures with perivascular epithelioid-like cells (a), which showed positivity for the HMB45 in the immunohistochemical study compatible with LAM cells (b).

Figure 2. HRCT scan in 2014 with multiple thin-walled cyst nodes.

Figure 3. HRCT scan in 2017 showing disease progression with parenchymal fibrosis, and higher number of cysts and mediastinal adenopathies.

Table

**Table 1.** Pulmonary Function Tests and Clinical Follow-Up
	FEV 1 (%)	TI (%)	DLCO (%)	SMWT/SpO₂ (m/%)	PaO₂ at rest (mm Hg)	Supplementary O₂ at rest/deambulation
FEV1: forced expiratory volume in 1 s; TI: Tiffeneau index; DLCO: diffusing capacity of the lungs for carbon monoxide; SMWT: six-minute walk test; SpO₂: peripheral capillary oxygen saturation; PaO₂: partial pressure of arterial oxygen. ^aStarted treatment with sirolimus.
01-2014	40.4	75.07	45.1	-/89%	70	No/No
10-2016	44.3	65.07	43.9	460/82%	64	No/No
01-2017	40.8	63.2	44	-	-	No/Yes
07-2017^a	38.7	66.7	33.8	-	53	Yes/Yes
11-2017	49.1	69.3	52	-/86%	84	No/Yes
01-2018	45.7	62.55	43	-	65	No/Yes
03-2018	61	76.54	50.3	-	69	No/Yes
06-2018	52	68.77	46	420 m/86%	83	No/Yes
09-2018	60	80.3	45	-	72	No/Yes
12-2018	66	78.36	49	-	-	No/Yes
03-2019	60	74.14	44	-	78	No/Yes