Download PDF
CC BY-NC-ND 4.0 · South Asian J Cancer 2021; 10(02): 92-96
DOI: 10.1055/s-0041-1731264
Original Article: Genitourinary Cancer

Study of Treatment Outcome in Adults with TFE-Related RCC

Ajaykumar Chandrabhan Singh
1   Department of Medical Oncology, Tata Memorial Hospital, Homi Bhabha National Institute, Mumbai, Maharashtra, India
,
Mahendra Pal
2   Department of Urologic Oncology, Tata Memorial Hospital, Homi Bhabha National Institute, Mumbai, Maharashtra, India
,
Akhil Kapoor
3   Department of Medical Oncology, Mahamana Pandit Madan Mohan Malviya Cancer Center and Homi Bhabha Cancer Hospital (A Unit of Tata Memorial Center, Mumbai), Varanasi, Uttar Pradesh, India
,
Nandini Menon
1   Department of Medical Oncology, Tata Memorial Hospital, Homi Bhabha National Institute, Mumbai, Maharashtra, India
,
Kumar Prabhash
1   Department of Medical Oncology, Tata Memorial Hospital, Homi Bhabha National Institute, Mumbai, Maharashtra, India
,
Vanita Noronha
1   Department of Medical Oncology, Tata Memorial Hospital, Homi Bhabha National Institute, Mumbai, Maharashtra, India
,
Ganesh Bakshi
2   Department of Urologic Oncology, Tata Memorial Hospital, Homi Bhabha National Institute, Mumbai, Maharashtra, India
,
Gagan Prakash
2   Department of Urologic Oncology, Tata Memorial Hospital, Homi Bhabha National Institute, Mumbai, Maharashtra, India
,
Santosh Menon
4   Department of Pathology, Tata Memorial Centre, Homi Bhabha National Institute, Mumbai, Maharashtra, India
,
Nilesh Sable
5   Department of Radiology, Tata Memorial Centre, Homi Bhabha National Institute, Mumbai, Maharashtra, India
,
Devanshi Kalra
6   CRS, Tata Memorial Hospital, Homi Bhabha National Institute, Mumbai, Maharashtra, India
,
Sheetal Kulkarni
6   CRS, Tata Memorial Hospital, Homi Bhabha National Institute, Mumbai, Maharashtra, India
,
Amit Joshi
1   Department of Medical Oncology, Tata Memorial Hospital, Homi Bhabha National Institute, Mumbai, Maharashtra, India
› Author Affiliations
› Further Information
   Permissions and Reprints

Abstract

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.

Keywords

event-free survival - fluorescence in situ hybridization - immunohistochemistry - overall survival - renal cell carcinoma - TFE translocation renal cell carcinoma


Publication History

Article published online:
04 September 2021

© 2021. MedIntel Services Pvt Ltd. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial-License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/).

Thieme Medical and Scientific Publishers Private Ltd
A-12, Second Floor, Sector -2, NOIDA -201301, India

 

  • References

  • 1 Argani P, Ladanyi M. Translocation carcinomas of the kidney. Clin Lab Med 2005; 25 (02) 363-378
    CrossrefPubMedGoogle Scholar
  • 2 Eble J, Sauter G, Epstein JI. WHO Classification of Tumours. Tumours of the Genitourinary and Male Genital Organs. Lyon: IARC Press 2004
  • 3 Argani P, Ladanyi M. Renal carcinomas associated with Xp11.2 translocations/TFE3 gene fusions. In: Eble JN, Sauter G, Epstein J, et al, eds. Pathology and Genetics of Tumors of the Urinary System & Male Genital Organs. Lyon, France: IARC 2004: 37-38
  • 4 Argani P, Olgac S, Tickoo SK. et al. Xp11 translocation renal cell carcinoma in adults: expanded clinical, pathologic, and genetic spectrum. Am J Surg Pathol 2007; 31 (08) 1149-1160
    CrossrefPubMedGoogle Scholar
  • 5 Argani P, Lal P, Hutchinson B, Lui MY, Reuter VE, Ladanyi M. Aberrant nuclear immunoreactivity for TFE3 in neoplasms with TFE3 gene fusions: a sensitive and specific immunohistochemical assay. Am J Surg Pathol 2003; 27 (06) 750-761
    CrossrefPubMedGoogle Scholar
  • 6 Meyer PN, Clark JI, Flanigan RC, Picken MM. Xp11.2 translocation renal cell carcinoma with very aggressive course in five adults. Am J Clin Pathol 2007; 128 (01) 70-79
    CrossrefPubMedGoogle Scholar
  • 7 Rais-Bahrami S, Drabick JJ, De Marzo AM. et al. Xp11 translocation renal cell carcinoma: delayed but massive and lethal metastases of a chemotherapy-associated secondary malignancy. Urology 2007; 70 (01) 178.e3-178.e6
    CrossrefPubMedGoogle Scholar
  • 8 Armah HB, Parwani AV. Renal cell carcinoma in a 33-year-old male with an unusual morphology and an aggressive clinical course: possible Xp11.2 translocation. Pathology 2008; 40 (03) 306-308
    CrossrefPubMedGoogle Scholar
  • 9 Komai Y, Fujiwara M, Fujii Y. et al. Adult Xp11 translocation renal cell carcinoma diagnosed by cytogenetics and immunohistochemistry. Clin Cancer Res 2009; 15 (04) 1170-1176
    CrossrefPubMedGoogle Scholar
  • 10 Zhong M, De Angelo P, Osborne L. et al. Translocation renal cell carcinomas in adults: a single-institution experience. Am J Surg Pathol 2012; 36 (05) 654-662
    CrossrefPubMedGoogle Scholar
  • 11 Parikh J, Coleman T, Messias N, Brown J. Temsirolimus in the treatment of renal cell carcinoma associated with Xp11.2 translocation/TFE gene fusion proteins: a case report and review of literature. Rare Tumors 2009; 1 (02) e53
    CrossrefPubMedGoogle Scholar
  • 12 Numakura K, Tsuchiya N, Yuasa T. et al. A case study of metastatic Xp11.2 translocation renal cell carcinoma effectively treated with sunitinib. Int J Clin Oncol 2011; 16 (05) 577-580
    CrossrefPubMedGoogle Scholar
  • 13 Heng DY, Xie W, Regan MM. et al. Prognostic factors for overall survival in patients with metastatic renal cell carcinoma treated with vascular endothelial growth factor-targeted agents: results from a large, multicenter study. J Clin Oncol 2009; 27 (34) 5794-5799
    CrossrefPubMedGoogle Scholar
  • 14 Kaplan E, Meier P. Nonparametric estimation from incomplete observations. J Am Stat Assoc 1958; 53: 457-481
    CrossrefGoogle Scholar
  • 15 NCCN Guidelines. Kidney Cancer. Version 1. 2021;MS9–MS26
  • 16 Camparo P, Vasiliu V, Molinie V. et al. Renal translocation carcinomas: clinicopathologic, immunohistochemical, and gene expression profiling analysis of 31 cases with a review of the literature. Am J Surg Pathol 2008; 32 (05) 656-670
    CrossrefPubMedGoogle Scholar
  • 17 Choueiri TK, Mosquera JM, Hirsch MS. A case of adult metastatic Xp11 translocation renal cell carcinoma treated successfully with sunitinib. Clin Genitourin Cancer 2009; 7 (03) E93-E94
    CrossrefPubMedGoogle Scholar
  • 18 Pwint TP, Macaulay V, Roberts IS, Sullivan M, Protheroe A. An adult Xp11.2 translocation renal carcinoma showing response to treatment with sunitinib. Urol Oncol 2011; 29 (06) 821-824
    CrossrefPubMedGoogle Scholar
  • 19 Malouf GG, Camparo P, Oudard S. et al. Targeted agents in metastatic Xp11 translocation/TFE3 gene fusion renal cell carcinoma (RCC): a report from the Juvenile RCC Network. Ann Oncol 2010; 21 (09) 1834-1838
    CrossrefPubMedGoogle Scholar
  • 20 Choueiri TK, Lim ZD, Hirsch MS. et al. Vascular endothelial growth factor-targeted therapy for the treatment of adult metastatic Xp11.2 translocation renal cell carcinoma. Cancer 2010; 116 (22) 5219-5225
    CrossrefPubMedGoogle Scholar