An unusual outcome of papillary renal cell carcinoma with lung metastases: a case report and review of literature

Abstract

Background Renal cell carcinoma (RCC) is a heterogeneous group of malignant epithelial tumors of the kidney. It accounts for more than 90% of all kidney cancers. However, papillary RCC is the second most common histologic subtype representing 10–15% of all RCCs. The mean age of presentation for papillary RCC ranges between 59 and 63 years but more importantly when RCC is diagnosed at a younger age, the possibility of an underlying hereditary kidney cancer syndrome should be considered. RCC potentially metastasizes to many different organs with lung being the commonest site accounting for 45.2%. The treatment for metastatic RCC is mostly multimodal for most patients. However, patients with untreated pulmonary metastases have been observed to have very poor prognosis with a 5-year overall survival rate of only 5% or even less and thus the need to report on the unusual outcome of our patient who had a metastatic disease. Case presentation The present study reports a papillary renal cell carcinoma with multiple lung metastases in a 31-year-old woman who presented with progressive right flank mass and pain with no chest symptoms. She underwent cytoreductive radical nephrectomy via a right subcostal incision. Patient, however, did not undergo metastasectomy nor palliative systemic therapy and was seen 5 years post-nephrectomy. Conclusion Our patient with metastatic RCC, without undergoing metastasectomy nor palliative systemic therapy, remained stable with 5-year progression-free survival post-cytoreductive nephrectomy.

Bilateral multifocal papillary renal cell carcinoma on autopsy: A case report

“…9 Two familial syndromes associated with increased risk of PRCC are hereditary papillary RCC (HPRCC) and hereditary leiomyomatosis RCC (HLRCC). 2 HPRCC is an autosomal dominant syndrome caused by mutation of the MET gene on chromosome 7q31 thus leading to increase in proliferative activity resulting invasion, aggressiveness and angiogenesis. 2 Targeted therapy with MET inhibitors in metastatic HPRCC such as foretinib and tivantinib is given.…”

“…2 HPRCC is an autosomal dominant syndrome caused by mutation of the MET gene on chromosome 7q31 thus leading to increase in proliferative activity resulting invasion, aggressiveness and angiogenesis. 2 Targeted therapy with MET inhibitors in metastatic HPRCC such as foretinib and tivantinib is given. 10 HLRCC syndrome is caused by an inactivating mutation of the fumarate hydratase (FH) gene which encodes the enzyme that converts fumarate to malate in the Krebs cycle and characterised by early age of onset, type 2 PRCC, cutaneous and uterine leiomyomas.…”

“…1 Clinical suspicion of RCC is uncommon and only 10% of patients present with classical triad of hematuria, flank pain and flank mass. 2 Papillary renal cell carcinoma (PRCC) was first reported in 1976 by Mancilla-Jimenez and is a primary malignant tumour of the renal tubular epithelium. 3 PRCC is a slow-growing neoplasm accounting for 5-15% of renal carcinoma and has a relatively favourable prognosis.…”

“…4 The mean age of presentation of PRCC ranges from sixth to seventh decade with male predominance. 2 Major etiologic factors associated are lifestyle changes such as smoking, obesity and hypertension, first-degree relative with kidney cancer as well as familial cancer syndromes mainly Von Hippel-Lindau syndrome. 4 Bilateral RCCs are rare accounting for 3-5% in the epidemiology of the disease and greatly challenging for the surgeons to balance between oncological principles vis-à-vis exploiting renal function to circumvent renal replacement therapy.…”

Ketorolac modulates Rac-1/HIF-1α/DDX3/β-catenin signalling via a tumor suppressor prostate apoptosis response-4 (Par-4) in renal cell carcinoma

“…Renal cell carcinoma (RCC) is the thirteenth most common cancer accounting for more than 90% of kidney tumors 1,2 . Globally, RCC encompasses ~ 2% of the total cancer diagnosed and deaths with an estimate of 400,000 new cases and 175,000 deaths per year [3][4][5] . RCC remains difficult-to-treat disease due to distinct tumor heterogeneity owing to multiple subtypes of diverse molecular features unveiling poor clinical outcome 6 .…”