Multidrug refractory aggressive metastatic TFE3 (+) renal cell carcinoma: A case report
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Published on: April 29, 2014
Nail Paksoy1, Selcuk Erdem2, Mert Karaca3
1Department of Medical Oncology, Institute of Oncology, Istanbul University, Istanbul, Turkey.
Background:
Transcription factor E3 (TFE3) related renal cell carcinomas constitute a very small percent of all renal tumors in adults. Prognosis mainly depends on the stage of the disease at the time of diagnosis which is often poor. There is yet to be a standardized treatment protocol. Treatment options include agents identical to TFE3(-) cell renal carcinoma treatment. We present a case of a young woman with a rapidly progressing metastatic TFE3 (+) renal cell carcinoma.
Case Report:
A 31 year old female presented with abdominal mass, distension, nausea. Initial tests and tumor markers found to be normal. Abdominal CT scan revealed a left retroperitoneal mass along with three other neighboring masses in liver manifesting as metastases. Trucut biopsy and immunohistochemical staining confirmed the retroperitoneal mass as TFE3 (+) renal cell carcinoma.Management and outcome: Sunitinib, pazopanib, nivolumab, axitinib treatments are consecutively given after surgery. It is noteworthy that rapid progression was observed under nivolumab treatment.
Discussion:
During surveillance, rapid progression is noted under consecutive immunotherapy which was unexpected. Thus, there is a need for more standardized treatment protocols and invention of new agents for management of TFE3 (+) renal cell carcinoma.
Insights
This case highlights a rare TFE3 (+) renal cell carcinoma in a young woman that rapidly progressed despite multiple treatments. Further research is needed for effective TFE3 (+) renal cell carcinoma management.
Area of Science:
- Oncology
- Genetics
- Pathology
Background:
- Transcription factor E3 (TFE3) related renal cell carcinomas (RCC) are rare adult renal tumors.
- Prognosis is often poor due to late diagnosis and lack of standardized treatment protocols.
- Current treatments mirror those for TFE3(-) RCC, with limited efficacy data for TFE3(+) subtypes.
Observation:
- A young female patient presented with a rapidly progressing, metastatic TFE3 (+) RCC.
- Initial diagnostic workup revealed a retroperitoneal mass with liver metastases.
- Immunohistochemical staining confirmed the TFE3 (+) status of the renal cell carcinoma.
Findings:
- The patient received sequential treatments including sunitinib, pazopanib, nivolumab, and axitinib post-surgery.
- Unexpected rapid tumor progression was observed during immunotherapy with nivolumab.
- This suggests potential resistance mechanisms to current therapeutic agents in TFE3 (+) RCC.
Implications:
- There is a critical need for developing standardized treatment protocols specifically for TFE3 (+) RCC.
- Novel therapeutic agents targeting TFE3 (+) RCC are required to improve patient outcomes.
- This case underscores the importance of vigilant monitoring for rapid progression, even with immunotherapy.
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