Papillary Renal Cell Carcinoma
Disease Details
Family Health Simplified
- Description
- Papillary renal cell carcinoma (PRCC) is a subtype of kidney cancer characterized by the formation of papillary structures in the renal tubules.
- Type
- Papillary renal cell carcinoma is a type of kidney cancer. It can occur sporadically or can be hereditary. Hereditary forms are often associated with genetic mutations, such as those in the MET proto-oncogene, and follow an autosomal dominant pattern of inheritance.
- Signs And Symptoms
- Due to its asymptomatic nature, PRCC is often undetectable, and the majority of cases are incidentally diagnosed during the radiological workup of unrelated diseases. Its clinical manifestations are similar to those of clear cell RCC, which are the classical triad of renal cell carcinoma (hematuria, flank pain and palpable abdominal mass; only 6-10% of patients) or even nonspecific symptoms including fatigue, weight loss, fever, and anorexia. Since early diagnosis is relatively uncommon, PRCC patients may experience symptoms caused by the metastatic spread to secondary sites. Specifically, metastasis occurs most frequently in the lungs followed by bone and the brain, exhibiting a wide range of symptoms including bone pain to a persistent cough.
- Prognosis
- The five-year survival rate of PRCC has been reported as 82-90%, which is slightly higher than that of other kidney cancers. The reduced survival rate has been positively correlated to several factors, which are high nuclear grade and stage, vascular invasion, DNA aneuploidy, and more. Patients with type 1 PRCC have significantly improved survival rates than those with type 2, which is a reflection of its lower TNM stage with a well-encapsulated tumor. Compared to other common types of RCC, PRCC exhibits a relatively lower risk of tumor recurrence and cancer-related death after nephrectomy. Specifically, the cancer-specific survival rate at five years following surgery with PRCC has reached up to 91%, while clear cell RCC and chromophobe RCC were 72% and 88%, respectively.
- Onset
- Papillary renal cell carcinoma (PRCC) is a type of kidney cancer that often presents subtly, and can include symptoms such as hematuria (blood in the urine), flank pain, or an abdominal mass. It may also be asymptomatic and found incidentally through imaging for unrelated conditions. The exact timing for onset varies, with many cases diagnosed in middle-aged and older adults.
- Prevalence
- Papillary renal cell carcinoma (PRCC) is a type of kidney cancer that accounts for approximately 10-15% of all renal cell carcinomas.
- Epidemiology
- Among different histologic subtypes of RCC, PRCC is the second most predominant type and accounts for 10-15% of all renal tumors. In the case of the United States, it is estimated that the incidence of PRCC will rise to 3,500 to 5,000 cases annually. Generally, PRCC is more prevalent among men than women, while the reported sex ratio (M: F) varies from 1.8:1 to 3.8:1. The mean age at presentation is identified as 52–66 years old; however, no statistically significant difference was found in the incidence of PRCC between the younger (< 40 years) and older adult groups (>40 years). In terms of racial variation, several studies have proven that people with African or Afro-Caribbean ancestry tend to have higher chances of being diagnosed with PRCC. According to the National Cancer Database, PRCC was more common in the Non-Hispanic Black population (38.9%) when compared to other races – Asian American (18.0%), Non-Hispanic White (13.2%), and Hispanic White populations (6.1%).
- Intractability
- Papillary renal cell carcinoma (PRCC) is a type of kidney cancer that can vary in its tractability, depending on the stage at diagnosis, the specific characteristics of the tumor, and the overall health of the patient. In early stages, PRCC may be treatable with surgical interventions such as nephrectomy (removal of the kidney or part of it) and may have a favorable prognosis. However, advanced stages where the cancer has metastasized may be more challenging to treat and could involve a combination of surgery, targeted therapies, and sometimes immunotherapy. The outcomes in such advanced cases can be less favorable, making the disease more difficult to manage effectively.
- Disease Severity
- Papillary renal cell carcinoma (PRCC) is a type of kidney cancer that originates in the renal tubules. The severity of PRCC can vary widely depending on several factors, including the stage and grade of the tumor at diagnosis, its size, and whether it has spread to other parts of the body. Early-stage PRCC that is localized to the kidney generally has a better prognosis and may be treated effectively with surgery. Advanced stages, where the cancer has metastasized, can be more challenging to treat and may require additional therapies such as targeted therapy, immunotherapy, or chemotherapy. Regular monitoring and follow-up are crucial for managing the disease.
- Healthcare Professionals
- Disease Ontology ID - DOID:4465
- Pathophysiology
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Pathophysiology of Papillary Renal Cell Carcinoma (PRCC):
Papillary Renal Cell Carcinoma (PRCC) is a type of kidney cancer that originates in the renal tubules. It is characterized by the formation of papillary structures and is classified into two main subtypes: Type 1 and Type 2.
- **Type 1 PRCC**: This subtype is generally less aggressive. It is often associated with genetic mutations in the MET proto-oncogene on chromosome 7, which leads to abnormal cell growth and proliferation.
- **Type 2 PRCC**: This subtype tends to be more aggressive and is associated with a poorer prognosis. Type 2 PRCC can be associated with various genetic alterations, including mutations in the fumarate hydratase (FH) gene, which is implicated in hereditary leiomyomatosis and renal cell carcinoma (HLRCC).
Both subtypes show a distinctive histopathological appearance featuring papillary or tubular-papillary architecture, with cells lining fibrovascular cores. Commonly implicated in the pathogenesis of PRCC are disruptions in signaling pathways that control cell division, differentiation, and apoptosis, such as abnormalities in the MET, FH, and other related signaling cascades.
PRCC is often identified through imaging studies and confirmed by histological analysis following biopsy or surgery. Treatment strategies may vary based on the subtype and stage of cancer, including surgical resection, targeted therapies, and, in some cases, immunotherapy. - Carrier Status
- Papillary renal cell carcinoma is typically not associated with a clear carrier status because it is a type of cancer rather than a genetic trait passed down through generations in a simple Mendelian fashion. It can, however, be part of hereditary cancer syndromes, such as hereditary papillary renal cell carcinoma, which often involves mutations in the MET gene.
- Mechanism
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Papillary renal cell carcinoma (PRCC) is a type of kidney cancer that arises from the renal tubules.
**Mechanism:**
PRCC is characterized by the formation of small, finger-like projections (papillae) in the tumor. These tumors are often less aggressive compared to other forms of renal cell carcinoma. The mechanism involves abnormal cell proliferation in the renal tubules, leading to tumor development.
**Molecular Mechanisms:**
1. **Genetic Alterations:**
- **MET Gene Mutations:** A significant proportion of PRCC cases involve mutations or amplifications in the MET proto-oncogene, which encodes a receptor tyrosine kinase involved in cellular growth and differentiation.
- **Chromosomal Aberrations:** Common chromosomal changes include trisomy of chromosomes 7, 17, and loss of the Y chromosome in males.
2. **Signaling Pathways:**
- **HGF/MET Pathway:** Aberrations in the MET gene can lead to constitutive activation of the hepatocyte growth factor (HGF)/MET signaling pathway, promoting cell proliferation, angiogenesis, and metastasis.
- **PI3K/AKT Pathway:** Dysregulation of the phosphoinositide 3-kinase (PI3K)/AKT pathway is also implicated, leading to increased survival and growth of cancer cells.
3. **Gene Expression Changes:**
- **Changes in miRNA Expression:** Altered expression of microRNAs (miRNAs) can affect the stability and translation of mRNAs involved in cell cycle regulation and apoptosis.
- **Epigenetic Modifications:** DNA methylation and histone modifications can lead to the inactivation of tumor suppressor genes and activation of oncogenes.
Understanding these mechanisms helps in developing targeted therapies for PRCC, such as inhibitors of the MET pathway. - Treatment
- First-line treatment for metastatic PRCC has not been standardized. Thus, similar treatment approaches for clear cell RCC have been used for PRCC, even though it has a distinct tumor histology.
- Compassionate Use Treatment
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For papillary renal cell carcinoma, compassionate use treatments, off-label medications, and experimental treatments are avenues patients sometimes pursue when standard therapies are not effective. While exact availability and options can depend on individual cases and regulatory status, here are some that may be considered:
1. **Compassionate Use Treatments**:
- **Targeted Therapies**: Drugs like sunitinib (Sutent) and cabozantinib (Cabometyx) might be accessed through compassionate use programs in cases where standard therapies have failed.
2. **Off-Label Treatments**:
- **Erlotinib**: Though primarily used for non-small cell lung cancer, it has been tried off-label for certain renal cancers.
- **Everolimus (Afinitor)**: Known for its use in various cancers, it may be used off-label for papillary renal cell carcinoma.
3. **Experimental Treatments**:
- **Clinical Trials**: Patients have access to investigational drugs and novel therapeutic combinations through clinical trials. These can include new chemotherapeutic agents, immunotherapies, and combination regimens not yet approved for renal cell carcinoma.
- **Immunotherapy**: Research is ongoing with various immune checkpoint inhibitors (like pembrolizumab) in clinical trials specifically targeting non-clear cell renal carcinomas, which includes the papillary subtype.
Consultation with an oncologist is essential to find the most suitable and potentially effective treatment options based on the most recent research and patient-specific factors. - Lifestyle Recommendations
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Lifestyle recommendations for individuals with papillary renal cell carcinoma may include:
1. **Healthy Diet**: Focus on a balanced diet rich in fruits, vegetables, lean proteins, and whole grains to support overall health.
2. **Regular Exercise**: Engage in regular physical activity that is appropriate for your fitness level to boost well-being and manage weight.
3. **Avoid Smoking**: If you smoke, seek help to quit. Smoking is a risk factor for many cancers, including kidney cancer.
4. **Limit Alcohol**: Consume alcohol in moderation, if at all, as excessive drinking can have negative health impacts.
5. **Hydration**: Maintain proper hydration by drinking adequate water daily to support kidney function.
6. **Weight Management**: Maintain a healthy weight, as obesity can increase the risk of cancer recurrence and other health issues.
7. **Regular Check-ups**: Keep up with regular medical appointments and screenings to monitor your health and any potential changes.
8. **Manage Stress**: Adopt stress-reduction techniques such as mindfulness, yoga, or meditation to maintain mental health.
9. **Medication Adherence**: Follow prescribed treatments and medications carefully as directed by your healthcare provider.
10. **Sun Protection**: Protect your skin from excessive sun exposure by using sunscreen and wearing protective clothing when outdoors.
Always consult with your healthcare provider for personalized advice tailored to your specific condition and health status. - Medication
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Papillary renal cell carcinoma (PRCC) is a type of kidney cancer. Treatments often include targeted therapies and immunotherapies. Some medications used for PRCC include:
1. **Sunitinib (Sutent)** - a tyrosine kinase inhibitor.
2. **Pazopanib (Votrient)** - another tyrosine kinase inhibitor.
3. **Cabozantinib (Cabometyx)** - targets multiple tyrosine kinases.
4. **Nivolumab (Opdivo)** - an immune checkpoint inhibitor.
5. **Axitinib (Inlyta)** - a tyrosine kinase inhibitor often used in combination with pembrolizumab (Keytruda).
The choice of medication depends on various factors, including the stage of cancer and individual patient characteristics. Treatment plans should be discussed with a healthcare provider. - Repurposable Drugs
- Currently, there are no specific repurposable drugs that have been widely validated for papillary renal cell carcinoma (pRCC). Standard treatment options typically include surgery, targeted therapies, and immunotherapies. Some research is ongoing to identify potential repurposable drugs, but conclusive results are not yet available. It's important to discuss treatment options with a healthcare professional specializing in this type of cancer.
- Metabolites
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Papillary renal cell carcinoma (PRCC) is a type of kidney cancer characterized by the formation of small, finger-like projections in the tumor. While specific metabolites associated with PRCC may vary, general alterations in metabolic pathways like glycolysis, the tricarboxylic acid (TCA) cycle, and lipid metabolism have been observed in renal cell carcinomas. Metabolomic studies might reveal specific biomarkers and therapeutic targets, but detailed, specific metabolites directly associated with PRCC can be complex and vary between studies.
Regarding "nan," it seems there might be a typo or confusion, as "nan" could be interpreted in various ways, including biological context. However, in this instance, no relevant information is available linking "nan" to PRCC. - Nutraceuticals
- There is limited scientific evidence supporting the use of nutraceuticals specifically for treating papillary renal cell carcinoma (PRCC). Nutraceuticals, which are food-derived products that offer health benefits, might have a role in general cancer prevention and supporting overall health. However, their efficacy in PRCC remains unproven, and they should not replace conventional treatments such as surgery, targeted therapy, or immunotherapy. For PRCC, it is crucial to follow the guidance of healthcare professionals regarding treatment options.
- Peptides
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Papillary renal cell carcinoma (PRCC) is a type of kidney cancer that originates in the lining of the tubules in the kidney. One area of research in treating PRCC involves the use of peptides. Peptides are short chains of amino acids that can be designed to target specific proteins or cellular pathways involved in the cancer's growth and survival. These peptides can serve as therapeutic agents, potentially inhibiting tumor growth, inducing apoptosis (programmed cell death), or improving the immune system's ability to recognize and destroy cancer cells.
Nanotechnology, often referred to as "nan," also plays a significant role in PRCC research and treatment. Nanoparticles can be used to deliver drugs more effectively to cancer cells by enhancing their uptake and reducing systemic side effects. Nanoparticles can be engineered to carry chemotherapeutic agents, peptides, or other therapeutic molecules directly to the tumor site. This targeted delivery system improves the efficacy of the treatment while minimizing damage to healthy tissues.
In summary, peptides and nanotechnology represent promising avenues for the development of novel therapies for papillary renal cell carcinoma, potentially leading to more effective and personalized treatment options.