case_images: PMC11396326_01
case_images: Expansile mass in the upper portion of the left kidney (thick arrows in A, B, D), with invasion of the sinusal fat tissue (B, D). A delayed nephrogram is apparent secondary to outflow obstruction due to renal vein thrombosis (thin arrow in C). Large, confluent, retroperitoneal lymphadenopathies can be seen (arrowheads in B, C).
case_images: jbsr-108-1-3687-g1.jpg
case_images: PMC11396326_01_jbsr-108-1-3687-g1.jpg
case_images: F1
case_images: Large, confluent retroperitoneal lymph nodes were also present (Figure 1).
case_images: Gross specimen of the resected kidney.
case_images: jbsr-108-1-3687-g2.jpg
case_images: PMC11396326_01_jbsr-108-1-3687-g2.jpg
case_images: F2
case_images: The gross specimen showed a multinodular solid mass, with necrosis and invasion in the sinusal fat and the renal vein (Figure 2).
case_images: A. H&E - Encapsulated tumor with tubulopapillary architecture. Tumor cells show eosinophilic cytoplasm and pleomorphic hyperchromatic nuclei with Fuhrmann nuclear grade 3. B. Immunohistochemistry markers with PAX8/panCK positivity.
case_images: jbsr-108-1-3687-g3.jpg
case_images: PMC11396326_01_jbsr-108-1-3687-g3.jpg
case_images: F3
case_images: Immunohistochemistry showed absence of FH in the tumor epithelium, indicating a FH-deficient RCC, which additionally supported PAX8+/panCK+ (Figure 3).
case_images: Multiple skin papules/nodules on the upper arm, compatible with cutaneous leiomyoma.
case_images: jbsr-108-1-3687-g4.jpg
case_images: PMC11396326_01_jbsr-108-1-3687-g4.jpg
case_images: F4
case_images: The patient also had multifocal skin lesions, most pronounced on the right arm (Figure 4).