A 32-year-old male with stage IV chromophobe renal cell carcinoma developed fatal pulmonary lymphangitic carcinomatosis shortly after initiating Ipilimumab and Nivolumab.
Case Report (n=1)
This case illustrates a rare and aggressive manifestation of pulmonary lymphangitic carcinomatosis in a patient with chromophobe renal cell carcinoma.
Abstract Introduction Renal cell carcinoma (RCC) is a common malignancy, with approximately 80% composed of clear cell histology. Consequently, non-clear cell RCC such as chromophobe RCC (chRCC) is less extensively studied. ChRCC represents only 5-7% of all RCC and generally carries a favorable prognosis, with metastasis occurring in 6-7% of cases. While metastatic RCC typically spreads via hematogenous and lymphatic routes, true pulmonary lymphangitic carcinomatosis (PLC) remains exceedingly rare. Description A 32-year-old male with newly diagnosed stage IV high-grade chRCC presented with malaise, dyspnea, and chest pain one day after his first cycle of Ipilimumab and Nivolumab. Admission imaging included computed tomography angiography (CTA) of the chest, which revealed asymmetric ground-glass opacities with interlobular septal thickening, peribronchovascular congestion, along with numerous pulmonary nodules in the hilar and upper lobe regions. Although these findings raised concern for pneumonitis, this was unlikely given he was one day status-post therapy. Thus, lymphangitic spread of chRCC was suspected. CTA findings and bronchial lavage with evidence of neutrophilia further supported this. Given the rapid progression of malignancy, immunotherapy was optimized by adding Cabozantinib. Shortly after, he developed new bilateral upper extremity weakness and blurry vision. Eventually, he required intubation, pressor support, and continuous renal replacement therapy given his hemodynamic instability and unresponsive state. He suffered from multiple arrhythmias before his family decided on comfort measures, and the patient eventually expired. Discussion PLC is a rare and fatal manifestation of metastatic disease, characterized by a diffuse permeation of tumor cells within pulmonary lymphatics. PLC most commonly occurs with primary malignancies of the breast, lung, stomach, or prostate, and is rarely seen in RCC. Clinically, this presents as progressive dyspnea and cough. In patients receiving immune checkpoint inhibitors, it is essential to distinguish PLC from immune-mediated pneumonitis, as management of care will pivot. Specific HRCT and transbronchial biopsy findings can support this diagnosis. In summary, this case illustrates a rare and aggressive manifestation of an otherwise indolent malignancy. The occurrence of PLC in chRCC is uncommon; thus, there is a lack of evidence regarding optimal treatment strategies or preventative approaches. Systemic therapy is the current mainstay of management. Further research is warranted to identify potential biomarkers associated with an increased risk for PLC. In addition, further investigation would need to be explored to determine if early detection and targeted therapy can mitigate this lethal complication. This abstract is funded by: None
Nguyen et al. (Fri,) conducted a case report in Chromophobe renal cell carcinoma (chRCC) with pulmonary lymphangitic carcinomatosis (n=1). Ipilimumab, Nivolumab, and Cabozantinib was evaluated. A 32-year-old male with stage IV chromophobe renal cell carcinoma developed fatal pulmonary lymphangitic carcinomatosis shortly after initiating Ipilimumab and Nivolumab.