RESUMO
Renal angiomyolipoma (AML) is a typically benign renal tumor that is divided into 2 classes, the classical variant and the more aggressive epithelioid variant. It is extremely rare for an AML to exhibit aggressive features such as vascular invasion. We present the case of a 36-year-old female who presented with right lower quadrant pain for 9 months and was found to have an AML with tumor extension into the renal vein and the IVC. Diagnosis was confirmed with histopathology and the patient was treated with a total nephrectomy. The epithelioid subtype of AML is a rare variant that should be considered in the differential of a renal mass with vascular invasion.
RESUMO
von Economo neurons (VENs) are bipolar, spindle-shaped neurons restricted to layer 5 of human frontoinsula and anterior cingulate cortex that appear to be selectively vulnerable to neuropsychiatric and neurodegenerative diseases, although little is known about other VEN cellular phenotypes. Single nucleus RNA-sequencing of frontoinsula layer 5 identifies a transcriptomically-defined cell cluster that contained VENs, but also fork cells and a subset of pyramidal neurons. Cross-species alignment of this cell cluster with a well-annotated mouse classification shows strong homology to extratelencephalic (ET) excitatory neurons that project to subcerebral targets. This cluster also shows strong homology to a putative ET cluster in human temporal cortex, but with a strikingly specific regional signature. Together these results suggest that VENs are a regionally distinctive type of ET neuron. Additionally, we describe the first patch clamp recordings of VENs from neurosurgically-resected tissue that show distinctive intrinsic membrane properties relative to neighboring pyramidal neurons.