An unusual renal angiomyolipoma with morphological lymphangioleiomyomatosis features and coexpression of oestrogen and progesterone receptors

Abstract

Angiomyolipoma is the most common mesenchymal renal tumour, the clonal origin of which has recently been demonstrated. It is composed of varying amounts of blood vessels, smooth muscle and fat. In this report, we describe a renal angiomyolipoma, which is unusual owing to the presence of a lymphangioleiomyomatosis-like component, occurring in a 41-year-old woman suffering from sporadic lymphangioleiomyomatosis. The diagnosis was based on histopathological and immunohistochemical findings. The tumour consisted of an intimate admixture of two components: one was typical of a classical angiomyolipoma and the other was reminiscent of lymphangioleiomyomatosis. HMB45 positivity was found on 5% of the cells of the angiomyolipoma component. Ten percent of the nuclei of the lymphangioleiomyomatosis and angiomyolipoma components expressed oestrogen receptors and 5% progesterone receptors. This case illustrates a very unusual pattern of a renal angiomyolipoma containing a lymphangioleiomyomatosis-like component. The oestrogen and progesterone immunoreactivity suggests that angiomyolipoma could be hormonally dependent. Therefore, we have emphasised the morphological and immunohistochemical similarities between angiomyolipoma and lymphangioleiomyomatosis.

Estrogen receptor is significantly associated with the epithelioid variants of renal angiomyolipoma: A clinicopathological and immunohistochemical study of 67 cases

“…versus PR 15.2%) was higher than in a previous report, which found that ER and PR were positive in 18.6%, and 27.9% of 46 AML cases, respectively 7 . However, in another study of AML combined with pulmonary lymphangiomyomatosis, ER and PR positive rates ranged from 0 to 83% and from 5 to 100% of AML cases, respectively 20–22 . Despite the variable data, few studies that tested ER and PR have shown that they are partly associated with AML.…”

“…7 However, in another study of AML combined with pulmonary lymphangiomyomatosis, ER and PR positive rates ranged from 0 to 83% and from 5 to 100% of AML cases, respectively. [20][21][22] Despite the variable data, few studies that tested ER and PR have shown that they are partly associated with AML. Furthermore, it was found that ER and PR were frequently expressed in epithelioid variants, which are presumed to be formed from differentiated myogenic cells from PEC around dysmorphic vessels.…”

Clinical utility of diagnostic guidelines and putative biomarkers in lymphangioleiomyomatosis

“…Obstruction of axial lymphatics by LAM cells can cause lymphadenopathy and chylous collections[4]. In addition, around 60% of patients with sporadic LAM and most patients with TSC-LAM have one or more angiomyolipomas[5][6][7], benign tumours which share a common ancestry with pulmonary LAM cells [3,8-10]. Importantly, high resolution computerised tomography (HRCT) alone is not diagnostic of LAM and patients undergoing transplant for ‘LAM’ have been found to have other diseases after examination of the explanted lungs [11,12].…”