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Published on: October 30, 2013
Immune Checkpoint Blockade Response in Mucinous Tubular and Spindle Cell Carcinoma
Simran Makker1, Neil J Shah2, Maria I Carlo2
1Laurel Springs School, Ojai, CA 93023, USA.
Abstract:
Mucinous tubular and spindle cell carcinoma (MTSCC) is a rare kidney tumor which is usually characterized by indolent disease physiology. While several high-grade and sarcomatoid MTSCC tumors have been reported, the clinical experience with contemporary immune checkpoint blockade (ICB) combination therapies extrapolated from treatment paradigms of conventional renal cell carcinoma (RCC) remains limited. Here, we report two patients with metastatic MTSCC treated with first-line ipilimumab plus nivolumab therapy who both achieved great clinical benefit. We subsequently performed immune deconvolution analysis on previously identified MTSCC-like kidney tumors from The Cancer Genome Atlas (TCGA) and discovered significantly higher PD-L1 transcriptomic expression compared to similar papillary RCC tumors, providing additional biomarker data supporting the observed ICB response. These data implicate ICB therapy as an effective treatment for patients with metastatic MTSCC.
Insights
Mucinous tubular and spindle cell carcinoma (MTSCC), a rare kidney cancer, responded well to immune checkpoint blockade (ICB) therapy. This suggests ICB is a promising treatment for metastatic MTSCC patients.
Area of Science:
- Oncology
- Immunotherapy
- Genitourinary Cancers
Background:
- Mucinous tubular and spindle cell carcinoma (MTSCC) is a rare kidney neoplasm typically exhibiting indolent behavior.
- Limited clinical data exist regarding the efficacy of immune checkpoint blockade (ICB) combination therapies in metastatic MTSCC, despite its use in conventional renal cell carcinoma (RCC).
Observation:
- Two patients with metastatic MTSCC experienced significant clinical benefit from first-line ipilimumab plus nivolumab therapy.
- Immune deconvolution analysis of MTSCC-like tumors in The Cancer Genome Atlas (TCGA) revealed elevated PD-L1 transcriptomic expression compared to papillary RCC.
Findings:
- First-line ipilimumab and nivolumab demonstrated substantial clinical benefit in two metastatic MTSCC patients.
- MTSCC tumors exhibit higher PD-L1 transcriptomic expression than papillary RCC, suggesting potential sensitivity to ICB.
Implications:
- Immune checkpoint blockade (ICB) therapy, specifically ipilimumab plus nivolumab, shows promise as an effective treatment for metastatic MTSCC.
- Elevated PD-L1 expression in MTSCC supports its potential as a biomarker for ICB response in this rare kidney cancer subtype.

