A Rare Case of Missing Primary in Metastatic Renal Cell Carcinoma

Ankita Kapoor,Nikhil Khushalani,Shipra Gandhi

doi:10.7759/cureus.8637

Abstract

Renal cell carcinoma (RCC) can present with a myriad of clinical symptoms and signs. It is also notorious for its initial presentation with distant metastasis. We report a case of a 42-year-old male diagnosed with papillary RCC (PRCC) presenting with pleural and nodal metastases in the absence of a radiographically-detected tumor primary. PRCC was diagnosed on immunohistochemical analysis of the tissue from the pleura and mediastinal lymph nodes and confirmed by gene expression profiling studies. As per treatment guidelines for metastatic RCC, the patient was started on sunitinib with evidence of disease progression after two cycles and palliative care approach was recommended due to rapidly declining performance status. Prospective data on the optimal management of metastatic PRCC are lacking, but drugs used are similar to the treatment of clear cell carcinomas (vascular endothelial growth factor (VEGF) tyrosine kinase inhibitors, mammalian target of rapamycin inhibitors) and checkpoint inhibitors. Further molecular study of these rare tumors is warranted to detect drivers of oncogenesis and identify targets for therapeutic intervention.

Highlights

Renal cell carcinoma (RCC) is amongst the most common genitourinary malignancies and is notorious for its initial presentation with distant metastases [1]
Prospective data on the optimal management of metastatic papillary RCC (PRCC) are lacking, but drugs used are similar to the treatment of clear cell carcinomas (vascular endothelial growth factor (VEGF) tyrosine kinase inhibitors, mammalian target of rapamycin inhibitors) and checkpoint inhibitors
We present a case of a 42-year-old male diagnosed with metastatic papillary RCC (PRCC) to the pleura and the lymph nodes without radiographic evidence of a renal primary

Summary

Introduction

Renal cell carcinoma (RCC) is amongst the most common genitourinary malignancies and is notorious for its initial presentation with distant metastases [1]. Histopathologic evaluation of the metastatic lesion in a patient with asymptomatic RCC with signs and symptoms due to distant metastases, reveals the diagnosis. We present a case of a 42-year-old male diagnosed with metastatic papillary RCC (PRCC) to the pleura and the lymph nodes without radiographic evidence of a renal primary. Subsequent CT-scan showed right-sided loculated effusion with the collapse of the right middle and lower lobes with mediastinal adenopathy (Figure 2). Considering the histology and immune profile, the patient was diagnosed with metastatic PRCC to the pleura and the mediastinal lymph nodes in the absence of a discernable primary renal lesion. Figure A reveals peritoneal carcinomatosis and Figure B reveals pleural metastatic disease marked by an arrow

Discussion

Conclusions

Findings

Disclosures

Meyer PC