Treatment outcome for metastatic papillary renal cell carcinoma patients

Abstract

BACKGROUND. Most clinical trial reports in metastatic renal cell carcinoma (RCC) do not distinguish between histologic subtypes, making it difficult to assess specific treatment efficacy. The current retrospective study sought to define clinical features and outcome data for metastatic papillary RCC. METHODS. Clinical features, treatment outcome, and survival were evaluated in 38 patients with metastatic papillary RCC who underwent clinical evaluation at Memorial Sloan‐Kettering Cancer Center (MSKCC) between 1985 and 2005. Twenty‐three of 513 individuals were identified from a clinical trial database, 14 of 1895 from a surgery database, and 1 of 357 from a pathology database. A literature review of systemic therapy in metastatic papillary RCC was performed. RESULTS. Among the 38 patients, 30 had been treated at MSKCC with various systemic therapies, including cytokines. Twelve therapies resulted in stable disease, 30 in initial progression of disease, and 1 in an unknown response. One patient had a partial response to sunitinib, a novel multitargeted tyrosine kinase inhibitor. The median overall survival time for the entire study group was 8 months (95% confidence interval, 5–12). A literature review on treatment of metastatic papillary RCC produced 4 reports, confirming a lack of efficacy for systemic therapy. CONCLUSIONS. A resistance to systemic therapy characterizes patients with metastatic papillary RCC. Further understanding of the genetics and molecular biology and subtypes involved may provide the basis for more effective agents. Treatment with targeted therapies or other experimental agents is warranted. Cancer 2006. © 2006 American Cancer Society.

Differential Diagnosis of Renal Tumors With Clear Cytoplasm: Clinical Relevance of Renal Tumor Subclassification in the Era of Targeted Therapies and Personalized Medicine

“…43 PRCC is less aggressive than CCRCC with metastases reported in 5% to 12% of patients. 19,44 In recent years, PRCC has been divided into 2 subtypes on the basis of cytoplasmic features, nuclear features, and the presence or absence of nuclear pseudostratification. 45 A 2005 study 46 suggested that PRCC can be further subdivided into 4 different morphologic categories, which correspond to 2 different molecular groups, based on gene expression profiling data.…”

“…59 These genetic differences likely underlie the decreased response to sunitinib and other targeted therapies seen in cases of metastatic PRCC. 11,44 There was hope that the study of cases of inherited type 1 PRCC, which are due to activating germline mutations of the c-MET oncogene, might provide insight into the inner workings of sporadic PRCC. Unfortunately, these activating mutations are uncommon outside of the syndromic setting, being present in approximately 10% of sporadic type 1 PRCCs.…”

Occult infiltrating bi-ventricular papillary renal cell carcinoma metastasis found during coronary artery bypass graft surgery

“…5 However, patients with advanced stage unresectable papillary RCC have similar prognoses to those with clear cell RCC with a reported median survival of 8 to 13 months. 6,7 The largest series reporting on 270 patients with papillary RCC demonstrated that about 4% of patients presented with metastatic disease, with lung (47% to 78%) and retroperitoneal lymph nodes (34% to 61%) as the two most common sites of metastasis. 6,7 Treatment has included surgery or chemotherapy alone, or a combination of the two depending on both patient and tumor related factors, such as the site of metastasis and patient performance status.…”

Expression of Angiopoietin-1, 2 and 4 and Tie-1 and 2 in gastrointestinal stromal tumor, leiomyoma and schwannoma

“…Sunitinib (sunitinib malate; SU11248; SUTENT ® ; Pfizer Inc, New York, NY, USA) is a novel multitargeted tyrosine kinase inhibitor with high binding affinity for VEGFR and PDGFR that recently has shown anti-tumor and anti-angiogenic activities [49] . This drug recently received approval from the US Food and Drug Administration (FDA) for two applications: advanced gastrointestinal stromal tumors (GISTs) [50] and renal cell carcinoma [51] , in patients who are resistant or intolerant to treatment with Imatinib. Since the Tie receptor is an RTK, Sunitinib may be suitable to down-regulate the activity of the angiopoietin pathway.…”