Multiple Unilateral Renal Angiomyolipomas with Regional Lymphangioleiomyomatosis

McIntosh, Graham; Dutoit, Stephen Hamilton; Chronos, N.V.; Kaisary, Amir V.

doi:10.1016/s0022-5347(17)39067-5

Cited by 6 publications

(2 citation statements)

References 6 publications

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“…Furthermore, inadequate sampling of the tumour may cause the presence of adipose tissue being missed. Ansari et al [1] described areas reminiscent of LAM in regional lymph nodes of a 46-year-old woman with renal AML extending into the lymph nodes, and McIntosh et al [10] reported a case of multiple renal AML occurring with LAM of the regional lymph nodes in a 55-year-old woman. The presence of LAM-like foci in renal AML was also documented by Chan et al [3].…”

Section: Discussionmentioning

confidence: 99%

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

2001

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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.

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Section: Discussionmentioning

confidence: 99%

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

2001

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“…6,8,12,13 AMLs are benign hamartomatous lesions, with abnormal, heterogenous smooth muscle-like cells similar to those in pulmonary and other extrapulmonary LAM lesions. Unlike these other LAM lesions, AMLs may be associated with adipose tissue and underdeveloped thick-walled blood vessels 12,13,[35][36][37][38] (FIG. 2).…”

Section: Introductionmentioning

confidence: 99%

Lymphatic Involvement in Lymphangioleiomyomatosis

Glasgow

Taveira-DaSilva

Darling

et al. 2008

Annals of the New York Academy of Sciences

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Lymphangioleiomyomatosis (LAM) is a rare, multisystem disease affecting primarily premenopausal women. The disease is characterized by cystic lung disease, at times leading to respiratory compromise, abdominal tumors (in particular, renal angiomyolipomas), and involvement of the axial lymphatics (e.g., adenopathy, lymphangioleiomyomas). Disease results from the proliferation of neoplastic cells (LAM cells), which, in many cases, have a smooth muscle cell phenotype, express melanoma antigens, and have mutations in one of the tuberous sclerosis complex genes (TSC1 or TSC2). In the lung, LAM cells found in the vicinity of cysts are, at times, localized in nodules and may be responsible for cyst formation through the production of proteases. Lymphatic channels, expressing characteristic lymphatic endothelial cell markers, are found within the LAM lung nodules. LAM cells may also be localized within the walls of the axial lymphatics, and, in some cases, penetrate the wall and proliferate in the surrounding adipose tissue. Consistent with extensive lymphatic involvement in LAM, the serum concentration of VEGF-D, a lymphangiogenic factor, is higher in LAM patients than in healthy volunteers.

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Renal Neoplasia

Eble¹,

Liu²

2009

Modern Surgical Pathology

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Multiple Unilateral Renal Angiomyolipomas with Regional Lymphangioleiomyomatosis

Cited by 6 publications

References 6 publications

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

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

Lymphatic Involvement in Lymphangioleiomyomatosis

Renal Neoplasia

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