Study of Treatment Outcome in Adults with TFE-Related RCC

• Main Authors: Ajaykumar Chandrabhan Singh, Mahendra Pal, Akhil Kapoor, Nandini Menon, Kumar Prabhash, Vanita Noronha, Ganesh Bakshi, Gagan Prakash, Santosh Menon, Nilesh Sable, Devanshi Kalra, Sheetal Kulkarni, Amit Joshi
• Format: Article
• Language: English
• Published: Thieme Medical and Scientific Publishers Pvt. Ltd. 2021-04-01
• Series: South Asian Journal of Cancer
• Subjects: event-free survival; fluorescence in situ hybridization; immunohistochemistry; overall survival; renal cell carcinoma; tfe translocation renal cell carcinoma
• Online Access: http://www.thieme-connect.de/DOI/DOI?10.1055/s-0041-1731264
• ISSN: 2278-330X; 2278-4306

Introduction TFE Translocation renal cell carcinoma (TRCC) represents 1 to 5% of all cases of renal cell carcinoma, with the highest frequency among children and young adults. Management of these tumors is not very well defined in literature. Although in pediatric age group it has favorable prognosis, in adults it has an aggressive nature, with poor outcome. This is a retrospective analysis of treatment outcome in adult patient 18 years or above treated at our hospital between January 2013 and November 2018. Material and Methods Clinical and pathological data of 26 patients from a single institution diagnosed with TRCC between January 2013 and November 2018 were retrospectively reviewed. All cases of TRCC were confirmed with immunohistochemistry or fluorescence in situ hybridization. We analyzed our data of patients treated with surgery only or who progressed after surgery and treated with systemic therapy or who presented with upfront unresectable or metastatic disease treated with systemic therapy with respect to event-free survival (EFS) and overall survival (OS). Results Between January 2013 and November 2018, 26 adult patients who were treated at our center were eligible for this analysis as per our criteria. Out of 26 patients, 25 patients had radical surgery after evaluation and 1 had metastatic disease who was started on systemic therapy. Out 25 patients who were treated with radical surgery, 16 patients progressed and they were started on systemic therapy except for 1 patient who defaulted. Median time to start systemic therapy among patient treated with curative nephrectomy was 13 months. Median EFS and median OS among overall population were 22 and 30 months, respectively. Among 16 patients who were treated with systemic therapy, median EFS to first-line therapy was 8 months and to second-line therapy was 2.5 months. Median OS was 17 months in patients treated with systemic therapy. Conclusion TRCC is rare in adult population but carries significant risk of disease progression even after initial curative treatment with potential response to targeted therapy for short duration.