Abstract: FR-PO1242
Aggressiveness in Disguise: Renal Medullary Carcinoma in a Young Adult with Sickle Cell Trait
Session Information
- Onconephrology: Diagnostic Dilemmas, Therapy-Related Toxicities, and Clinical Cases
October 23, 2026 | Location: Exhibit Hall A, Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Onconephrology
- 1600 Onconephrology
Authors
- Patrick, Steven J., Walter Reed National Military Medical Center, Bethesda, Maryland, United States
- Lieberman, N Samara, Walter Reed National Military Medical Center, Bethesda, Maryland, United States
- Shick, Paul C., The Joint Pathology Center, Silver Spring, Maryland, United States
- Malone, Laura, Walter Reed National Military Medical Center, Bethesda, Maryland, United States
- Watson, Maura A., Walter Reed National Military Medical Center, Bethesda, Maryland, United States
Introduction
Renal medullary carcinoma (RMC) is a rare, aggressive non-clear cell kidney cancer primarily affecting young adults of African descent, particularly males with sickle cell trait (SCT). Diagnosis often occurs post-metastasis, leading to poor prognosis with mean survival under one year. Currently, no standardized screening guidelines exist for at-risk populations. This case highlights a metastatic RMC presentation in a service member (SM), emphasizing diagnostic and screening considerations within the military population.
Case Description
A 26-year-old male SM with SCT presented with right-sided flank pain. Imaging showed a 2.8 cm right interpolar posterior hypodense lesion, diffuse lymphadenopathy with liver and bone lesions, and ascites consistent with metastatic cancer. He was diagnosed with aggressive lymphoma with atypical lymphoid proliferation based on lymph node biopsy and treated 1 cycle of Cyclophosphamide (Cytoxan), Hydroxydaunorubicin (Doxorubicin), Oncovin (Vincristine), and Prednisone (CHOP). Subsequent paracentesis suggested neuroendocrine carcinoma with peritoneal carcinomatosis. Course was complicated by Candida fungemia, portal vein thrombus, respiratory failure requiring intubation, anuric renal failure requiring continuous renal replacement therapy, and PEA arrest with anoxic brain injury. Given his poor prognosis and concern that risks of anti-cancer therapies outweighed any benefit, the decision was made with his family to transition to comfort care. Post-mortem testing revealed a SMARCB1 (INI1)-deficient epithelioid neoplasm, consistent with RMC.
Discussion
RMC must remain a primary differential diagnosis for undifferentiated carcinoma in young Black males with SCT. Because the military routinely screens for hemoglobinopathies, a unique opportunity exists for early RMC surveillance in this high-risk group. Further research is essential to establish optimal screening protocols, identify additional risk factors, and develop effective treatments for this lethal malignancy.
Acknowledgment
Disclaimer: The opinions and assertions expressed herein are those of the author(s) and do not reflect the official policy or position of the Uniformed Services University of the Health Sciences or the Department of War.
Figure 1:
A: H&E stain: Infiltrating islands/strands of epithelioid cells with abundant cytoplasm.
B: Cytokeratin (AE1/AE3): Positive cytoplasmic reactivity in infiltrating tumor cells.
C: INI-1 reactivity: Loss of reactivity in tumor cells; indicates SMARCB1 deficiency