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Angiomyolipomas are typically found in the kidney, but have also been commonly found in the liver and less commonly the ovary, fallopian tube, spermatic cord, palate, and colon. The Maclean imaging classification system for renal angiomyolipomas is based on the location of the angiomyolipoma within the kidney. [4]
angiomyolipoma, clear-cell "sugar" tumour (CCST), lymphangioleiomyomatosis, and, clear-cell myomelanocytic tumour of ligamentum teres/falciform ligament. abdominopelvic sarcoma of perivascular epitheloid cells [2] primary extrapulmonary "sugar" tumour [2] Thus, it has been advocated that the above could be classified PEComas. [1]
The RENAL Nephrometry Scoring System is used to measure the complexity of kidney tumors for determining whether a renal mass is appropriate for partial or radical nephrectomy, and is estimated by CT scan as follows: [7] The nephrometery score takes into account the size of the tumor (Radius), how much of the tumor is inside or outside of the ...
Neoplasms are the most common underlying pathology in up to 60% of cases and include renal angiomyolipoma and renal cell carcinoma. Other causes include rupture of renal artery or an arteriovenous malformation, polyarteritis nodosa, cystic medial necrosis, segmental arterial mediolysis, and cystic rupture. [3]
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.
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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