Angiomyolipoma (AML) is a rare benign renal tumor occurring in about 0.3 to 3% of the general population. Most frequently it takes the form of small single tumors occurring sporadically or accompanying tuberous sclerosis (Bourneville-Pringle disease). In some cases the tumor may reach a very large size and be a cause of various serious complications. This case description concerns a 26-year-old female patient, suffering from hypopituitarism, hypothyroidism and binocular blindness during the course of septo-optic dysplasia, in whom a giant, left renal AML was diagnosed and treated surgically. According to the authors' knowledge this was the first reported case of a huge size AML in a patient with de Morsier syndrome.
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| http://dx.doi.org/10.1186/s40001-014-0046-8 | DOI Listing |
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Article Synopsis
- Mesenchymal neoplasms in the nasal cavity, such as angioleiomyoma (AL) and angiomyolipoma (AML), are rare tumors that involve vascular, smooth muscle, and adipocytic components, with unclear classifications as hamartomas or true neoplasms due to limited genetic research.
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- The study discovered that most cases had a high proportion of smooth muscle, with some showing additional components like blood vessels and fatty tissue, while all lesions were positive for smooth muscle markers, indicating their muscular origin.
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