Patient:
Female, 20 FINAL DIAGNOSIS: Papillary renal cell carcinoma Symptoms: Hemopthysis Medication: Sutent Clinical Procedure: CT guided biopsy Specialty: Oncology.
Objective:
Rare disease.
Background:
Papillary renal cell carcinoma (PRCC) is a rare disease and is a carcinoma of the renal tubular epithelium, comprising only 10-15% of all renal cell carcinoma cases. The majority of cases occur in the sixth decade of life. PRCC rarely occurs before the fourth decade in the absence of family history. This paper describes an aggressive, sporadic case of PRCC in a 20-year-old female without family history and no risk factors.
Case report:
A 20-year-old African American female was admitted for hemoptysis with elevated blood pressure and was found to have left peri-hilar opacification on chest X-ray. Further radiological studies led to the discovery of a large complex left renal lesion within the collecting system, infiltrating the renal artery and causing severe hydronephrosis with para-aortic lymphadenopathy. An MRI also showed signal heterogeneity in the L2 and L3 vertebrae. Biopsies of the left renal mass and a right endobronchial lesion confirmed metastatic PRCC. Treatment was commenced with a tyrosine kinase inhibitor. Within a few weeks, the vertebral metastatic lesions progressed to cause spinal compression. After targeted radiotherapy, the patient was referred to Memorial Sloan Kettering Cancer Center for enrolment in a clinical trial.
Conclusions:
PRCC rarely occurs in the second decade of life and even then, most such early cases occur in family clusters. PRCC also has a relatively benign course, constituting less than 10% of all metastatic renal cell carcinomas, further making this case a unique presentation.