Slideshow
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Precon
Computed tomography scans demonstrating 5 cm left and 1 cm right enhancing renal masses in a young patient who proved to harbor type 2 papillary renal cell carcinoma upon resection.
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Postcon
Computed tomography scans demonstrating 5 cm left and 1 cm right enhancing renal masses in a young patient who proved to harbor type 2 papillary renal cell carcinoma upon resection.
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Coronal
Computed tomography scans demonstrating 5 cm left and 1 cm right enhancing renal masses in a young patient who proved to harbor type 2 papillary renal cell carcinoma upon resection.
A 43-year-old man presented with bilateral renal masses. He underwent a left partial nephrectomy, with pathology revealing type 2 papillary renal cell carcinoma. He reported that his twin sister underwent hysterectomy approximately 10 years previously for refractory uterine bleeding.
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This case was prepared by Alexander Kutikov, MD, of Fox Chase Cancer Center in Philadelphia.
REFERENCE
Menko FH, Maher ER, Schmidt S, et al. Hereditary Leiomyomatosis and Renal Cell Cancer (HLRCC): Renal Cancer Risk, Surveillance and Treatment. Fam Cancer 2014;13:637-644.
Answer: D
Young patients with type 2 papillary renal cell carcinoma should be screened for hereditary leiomyomatosis and renal cell cancer syndrome, autosomal dominant conditions associated with fumarate hydratase (FH) mutation. Approximately 15% of patients with FH mutation will develop renal malignancy over their life time. Given the aggressive biological behavior of type 2 papillary lesions, unlike in cases of other hereditary RCC syndromes, resection of the right renal mass should not be delayed in patients with bilateral tumors.
QUESTION
