Scientific articleRenal angiomyolipoma in association with pulmonary lymphangioleiomyomatosis: Forme fruste of tuberous sclerosis?☆
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Cited by (53)
Lymphangioleiomyomatosis
2015, Murray and Nadel's Textbook of Respiratory Medicine: Volume 1,2, Sixth EditionExtrapulmonary lymphangioleiomyomatosis in pelvic lymphadenectomy associated with invasive endometrial carcinoma
2014, Revista Espanola de PatologiaCitation Excerpt :Patients with TSC-associated LAM usually show extrapulmonary manifestations such as angiomyolipomas, axillary lymphadenopathy and abdominal lymphangiomas; however, often the lack of involvement in other organs required to establish the diagnosis of tuberous sclerosis makes this diagnosis difficult in such cases. For this reason the authors propose that the association of abdominal lymphangiomas, angiomyolipomas and pulmonary LAM is a “frustrated form” of tuberous sclerosis with these two conditions being considered the two extremes of the same disease.5,6 LAM has an inexorable course with progressive cystic destruction of the lung parenchyma which leads to a fatal outcome after an average of 8–10 years.
Nonepithelial Neoplasms of the Kidney
2013, Practical Renal Pathology: A Diagnostic ApproachFrequency of undiagnosed cystic lung disease in patients with sporadic renal angiomyolipomas
2012, ChestCitation Excerpt :Although 90% of renal AMLs are sporadic, they are also a well-recognized manifestation of TSC and occur in 60% to 80% of patients with TSC in whom renal AMLs are usually multiple and present at a younger age compared with the sporadic form.4,6,7,13–15 Renal AMLs are also encountered in patients with sporadic LAM, with approximately 30% to 50% exhibiting renal AML, usually as a solitary lesion.16–19 The association of renal AML and LAM has been of interest because LAM cells, characterized by mutations in the TSC1 or TSC2 genes, are found in both lesions.1,20,21
Lymphangioleiomyomatosis in a Post-Menopausal Women
2010, Archivos de BronconeumologiaVascular endothelial growth factors C and D induces proliferation of lymphangioleiomyomatosis cells through autocrine crosstalk with endothelium
2009, American Journal of PathologyCitation Excerpt :These findings support the hypothesis that LAM nodules are a result of distant metastases from the AML lesions. However, only 60% of sporadic LAM cases have co-incident AML, challenging this hypothesis.38–40 It is important to consider though that both AML and LAM lesions are classified within the broad category of perivascular epithelioid cell tumors.
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Presented at the North Central Section meeting of the American Urological Association, Colorado Springs, Colorado, October 23, 1990.