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JAMA Oncology Clinical Challenge
June 17, 2021

Typical Kidney Tumor With Atypical Presentation

Author Affiliations
  • 1Thoracic Surgery Branch, Center for Cancer Research, National Cancer Institute, National Institutes of Health, Bethesda, Maryland
JAMA Oncol. Published online June 17, 2021. doi:10.1001/jamaoncol.2021.1707

A White woman in her late 50s with a medical history of osteoporosis, hyperlipidemia, and migraines presented with hematuria. Results of computed tomography (CT) imaging showed an 8×6×5-cm right kidney lesion (Figure 1, A). The patient underwent surgical resection with a right nephrectomy and an apparent diagnosis of angiomyolipoma (AML). One year later, the patient underwent excision of a right iliac wing lesion, and pathologic findings demonstrated xanthoma of bone with sclerosis. Three years after this second surgery, she presented with new abdominal pain. Results of CT imaging revealed a 4.3-cm retrocaval mass at the level of the renal right vein. She underwent surgical resection, and results of pathologic evaluation were consistent with the original tumor. Follow-up CT imaging revealed a 1.2-cm left upper lobe lung nodule and a 0.8-cm right upper lobe lung nodule, as well as a pulmonary embolism and a left 6th rib fracture. These lesions were fluorodeoxyglucose-positron emission tomography avid. The patient denied constitutional symptoms such as fatigue, fever, chills, and night sweats. She underwent bilateral video-assisted thoracoscopic surgery wedge resections of the lung lesions, which were also consistent with the primary tumor. Five years later, the patient (now in her late 60s) presented with multiple left pleural-based lung lesions (Figure 1, B). Results of all laboratory testing were within reference ranges. These pleural-based lesions were resected with left video-assisted thoracoscopic surgery partial pleurectomy, and results of pathologic evaluation were positive for HMB45 and Melan-A.

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