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Some other kidney tumours contain fat, so the presence of fat is not diagnostic. Distinguishing a fat-poor angiomyolipoma from a renal cell carcinoma (RCC) can be difficult. [6] Both minimal fat AMLs and 80% of the clear-cell type of RCCs display signal drop on an out-of-phase MRI sequence compared to in-phase. [7]
The most common tumours in the PEComa family are renal angiomyolipoma and pulmonary lymphangioleiomyomatosis, both of which are more common in patients with tuberous sclerosis complex. The genes responsible for this multi-system genetic disease have also been implicated in other PEComas. [2]
The most common malignant renal parenchymal tumor is renal cell carcinoma (RCC), which accounts for 86% of the malignancies in the kidney. RCCs are typically isoechoic and peripherally located in the parenchyma, but can be both hypo- and hyper-echoic and are found centrally in medulla or sinus.
Kidney complications such as angiomyolipoma and cysts are common and more frequent in females than males and in TSC2 than TSC1. Renal cell carcinoma is uncommon. Lymphangioleiomyomatosis is only a risk for females with angiomyolipomas. [38] In the brain, the subependymal nodules occasionally degenerate to subependymal giant cell astrocytomas.
The renal condition should not be confused with other conditions which are Müllerian duct anomalies, such as Herlyn-Werner-Wunderlich syndrome. Some sources refer to double uters-hemivagina-renal agenesis as simply Wunderlich syndrome, but Herlyn-Werner-Wunderlich is a better term to distinguish the two.
Post-operatively, the presence of renin granules in pathology specimens as well as immunohistochemical analyses could help differentiating this tumor from other primary renal tumors such as hemangiopericytoma, glomus tumor, metanephric adenoma, epithelioid angiomyolipoma, Wilms tumor, solitary fibrous tumor, and some epithelial neoplasms. [6] [8]
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