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Perivascular epithelioid cell tumors (PEComa) are rare mesenchymal neoplasms composed of cells that express melanocytic and myogenic markers and grow around small blood vessels. PEComa often show benign behaviors but can also be highly aggressive. In frail and more complex patients, many conditions can overlap, compounding the diagnostic and therapeutic difficulties inherent in rare diseases. Moreover, the complexity of modern patients introduces new and significant players in host-microbe interactions, and emerging pathogens represent a relevant challenge to modern healthcare. Among these pathogens is Eggerthella lenta, an anaerobic gram-positive bacterium of the normal gut microbiota associated with life-threatening infections. Here, we present a case of malignant pelvic PEComa with rapid metastatic progression in a 73-year-old man who presented with an E. lenta bloodstream infection …