Open AccessEfficacy of hormonal suppression in a patient with chyluria due to lymphangioleiomyomatosis
Author(s) -
Paola Contini,
M Schiavina,
Riccardo Schiavina,
Francesco Tavalazzi,
Andrea Fabiani,
Valerio Di Scioscio,
Paolo Spagnolo,
Luca Richeldi
Publication year - 2019
Publication title -
multidisciplinary respiratory medicine
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.72
H-Index - 28
eISSN - 2049-6958
pISSN - 1828-695X
DOI - 10.4081/mrm.2011.455
Subject(s) - lymphangioleiomyomatosis , medicine , estrogen , rare disease , pathology , estrogen receptor , hormone , immunohistochemistry , disease , lung , cancer , breast cancer
Lymphangioleiomyomatosis (LAM) is a rare lung disease affecting almost exclusively young women, characterised by abnormal proliferation of atypical smooth muscle cells. We describe a young woman presenting with chyluria secondary to the presence of a large retroperitoneal lymph - angioleiomyoma. Immunohistochemical analysis revealed HMB45-negative LAM cells (HMB45 staining is absent only in rare cases) expressing low levels of estrogen receptors. Estrogen suppressive treatment with triptoreline, a synthetic analogue of Gn-RH, resulted in dramatic reduction of the retroperitoneal mass size. The role of estrogens in the pathogenesis of LAM remains poorly understood, and hormonal therapy is still debated, but this case suggests that at least in some LAM patients, possibly those with HMB45-negative disease and estrogen receptor expression, hormonal therapy may be effective in controlling the disease process.