Are renal cell carcinoma tumors associated with paraneoplastic syndrome?

From the Guidelines

Yes, kidney cancer tumors, particularly renal cell carcinoma (RCC), are associated with paraneoplastic syndromes, with approximately 10-40% of patients with RCC developing some form of paraneoplastic manifestation, as reported in the most recent study 1. The most common paraneoplastic syndromes associated with kidney cancer include:

• Hypercalcemia (due to production of parathyroid hormone-related protein)
• Erythrocytosis (from excessive erythropoietin production)
• Hypertension (related to renin secretion)
• Hepatic dysfunction (Stauffer syndrome)
• Neuromyopathies These syndromes occur because kidney cancer cells can produce hormones and cytokines that affect distant organs and systems, as described in 1. Hypercalcemia is particularly common, affecting about 15% of RCC patients, and may present with symptoms like fatigue, confusion, constipation, and increased thirst, as noted in 1. Recognizing these syndromes is crucial, as they can sometimes be the first indication of kidney cancer and may help in early diagnosis and monitoring of treatment response, as emphasized in the most recent guideline 1. The resolution of these paraneoplastic manifestations often occurs after successful treatment of the primary kidney tumor through surgical removal or systemic therapy, highlighting the importance of prompt diagnosis and treatment, as reported in 1 and 1. In clinical practice, it is essential to consider paraneoplastic syndromes in the differential diagnosis of patients with kidney cancer, as they can significantly impact morbidity, mortality, and quality of life, as noted in 1.

From the Research

Association between Kidney Cancer Tumors and Paraneoplastic Syndrome

• Kidney cancer tumors, specifically renal cell carcinoma (RCC), have been associated with paraneoplastic syndromes in various studies 2, 3, 4, 5, 6.
• Paraneoplastic syndromes are present in up to 20% of patients with RCC, and may be the initial clinical presentation of the disease 2.
• The syndromes associated with RCC include hypercalcemia, Stauffer's syndrome, hypertension, anemia, elevated erythrocyte sedimentation rate (ESR), and persistent leukocytosis 4.
• The presence of a paraneoplastic syndrome in a patient with RCC is neither a marker of metastatic disease nor necessarily indicative of a poor prognosis 3.
• However, some studies suggest that paraneoplastic syndromes may be associated with adverse prognosis among patients with RCC undergoing nephrectomy 5.

Types of Paraneoplastic Syndromes Associated with Kidney Cancer

• Hypercalcemia: a common paraneoplastic syndrome associated with RCC, caused by the production of parathyroid hormone-related protein by tumor cells 2, 4, 6.
• Stauffer's syndrome: a paraneoplastic syndrome characterized by hepatic dysfunction, which is associated with poor outcome in patients with RCC 4.
• Anemia: a common paraneoplastic syndrome associated with RCC, which may be caused by the production of cytokines by tumor cells 4, 5.
• Polycythemia: a paraneoplastic syndrome associated with RCC, caused by the production of erythropoietin by tumor cells 5.
• Renin-producing tumors: a rare paraneoplastic syndrome associated with RCC, which can cause hypertension 6.

Clinical Implications

• Early recognition of paraneoplastic syndromes is important for the diagnosis and management of RCC 2, 3.
• The presence of a paraneoplastic syndrome may facilitate early diagnosis of RCC, and its resolution may be an indicator of effective treatment 2.
• Accurate diagnosis and management of paraneoplastic syndromes are important in palliative care, as they can improve the quality of life of patients with RCC 2, 6.