Giant Renal Angiomyolipomas and Pulmonary Lymphangioleiomyomatosis: Follow-up Report after More than a Decade

Renal angiomyolipomas (AMLs) and pulmonary lymphangioleiomyomatosis (LAM) are two major presentations of tuberous sclerosis (TS), an autosomal dominant multisystem disorder. Renal AMLs can lead to life-threatening complications like hemorrhage and cause progressive renal failure requiring dialysis a...

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Bibliographic Details
Published in:Saudi journal of kidney diseases and transplantation 2023-11, Vol.34 (6), p.671-675
Main Authors: Nasir, Kiran, Ahmad, Aasim
Format: Article
Language:English
Subjects:
Adult
Angiomyolipoma - complications
Angiomyolipoma - diagnostic imaging
Angiomyolipoma - surgery
Female
Hemorrhage - etiology
Humans
Kidney Neoplasms - complications
Kidney Neoplasms - pathology
Kidney Neoplasms - surgery
Lung Neoplasms - complications
Lung Neoplasms - surgery
Lymphangioleiomyomatosis - complications
Lymphangioleiomyomatosis - surgery
Nephrectomy
Time Factors
Tomography, X-Ray Computed
Treatment Outcome
Tuberous Sclerosis - complications
Tuberous Sclerosis - surgery
Tumor Burden
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Summary:Renal angiomyolipomas (AMLs) and pulmonary lymphangioleiomyomatosis (LAM) are two major presentations of tuberous sclerosis (TS), an autosomal dominant multisystem disorder. Renal AMLs can lead to life-threatening complications like hemorrhage and cause progressive renal failure requiring dialysis and kidney transplant. mTOR inhibitors showed promising results in TS patients with renal AMLs, LAM, and subependymal giant cell astrocytomas. This case report is a follow-up of a patient we reported in 2010 with giant AMLs and LAM who required an emergency nephrectomy for massive hemorrhage from giant left-sided AMLs.
ISSN:1319-2442
2320-3838
DOI:10.4103/sjkdt.sjkdt_324_21