kidney clear cell sarcoma

What is Clear Cell Renal Cell Carcinoma (ccRCC)?

Clear cell renal cell carcinoma, also known as ccRCC, is a type of kidney cancer that affects adults. It is the most common form of kidney cancer in this age group, making up about 80% of all renal cell carcinoma cases.

Characteristics and Symptoms

• Clear cell RCC begins in cells that line tiny tubes in the kidney, which filter waste products from the blood.
• The cancer can cause symptoms such as:
  • Blood clots to form in the renal vein
  • Tumors growing inside the kidneys
  • Metastasis (spreading) to other parts of the body
• In some cases, ccRCC may not cause any noticeable symptoms.

Causes and Risk Factors

• Clear cell RCC is more common in adults than children.
• Risk factors for developing ccRCC include:
  • Smoking
  • Obesity
  • Hypertension (high blood pressure)
  • Long-term dialysis (particularly in acquired adult cystic kidney disease)
  • Family history of kidney cancer

Types and Grades

• Clear cell RCC is the most common type of renal cell carcinoma.
• The International Society of Urological Pathology (ISUP) and the World Health Organization (WHO) grade ccRCC based on its microscopic appearance:
  • Grade 1: Inconspicuous and basophilic nucleoli
  • Grade 2: Clearly visible and eosinophilic nucleoli

References

• [1] Clear cell renal cell carcinoma is named after how the tumor looks under the microscope, with cells appearing clear like bubbles. (Source: #15)
• [2-5] ccRCC is the most common form of kidney cancer in adults, making up about 80% of all renal cell carcinoma cases. (Sources: #4, #5, #6, and #14)
• [7-10] Clear cell RCC can cause blood clots to form in the renal vein, metastasize to other parts of the body, and have various risk factors such as smoking and obesity. (Sources: #10, #11, #12, and #13)

Symptoms of Clear Cell Sarcoma

Clear cell sarcoma, also known as clear cell renal cell carcinoma (ccRCC), can cause a range of symptoms, including:

• Painless lump under the skin: The most common symptom is a painless lump or mass under the skin on a leg or foot, which may be easily misattributed to a sports-related injury [7].
• Abdominal mass and pain: A palpable abdominal mass, abdominal or flank pain, hematuria (blood in the urine), anemia, and/or fatigue are also common symptoms [8].
• Swollen lumps and ulcers: In some cases, clear cell sarcoma can cause painful swollen lumps and ulcers on the skin, particularly in the legs, ankles, hands, and feet [3].

Other Possible Symptoms

In addition to these specific symptoms, kidney cancer (including clear cell sarcoma) can also cause more general signs and symptoms, such as:

• Weight loss: Unintentional weight loss is a common symptom of many types of cancer, including kidney cancer.
• Fatigue: Feeling extremely tired or weak is another possible symptom of kidney cancer.
• Blood in the urine: Hematuria (blood in the urine) can be a sign of kidney cancer.

Important Note

It's essential to note that these symptoms can also be caused by other, non-cancerous conditions. If you're experiencing any of these symptoms, it's crucial to consult with a healthcare professional for proper evaluation and diagnosis.

References:

[3] - Context result 13 [7] - Context result 7 [8] - Context result 8

Diagnostic Tests for Kidney Clear Cell Sarcoma

Kidney clear cell sarcoma (CCSK) can be challenging to diagnose, but various diagnostic tests can help confirm the presence of this rare cancer.

• Imaging tests: Imaging tests such as ultrasound, computed tomography (CT), and magnetic resonance imaging (MRI) can show the location and size of a kidney tumor. These tests may also reveal any potential spread of the cancer to other parts of the body [8].
• Biopsy: A biopsy is a procedure where a small sample of tissue from the tumor is taken using a needle. This sample is then examined under a microscope to confirm the presence of CCSK cells [4, 5]. In some cases, a biopsy may be performed after surgery to confirm the diagnosis and determine whether the cancer has spread.
• Blood tests: While

Treatment Options for Clear Cell Renal Cell Carcinoma (ccRCC)

Clear cell renal cell carcinoma (ccRCC) is a type of kidney cancer that can be challenging to treat, especially when it has spread beyond the kidney. While surgery is often the first line of treatment, drug therapy plays a crucial role in managing this disease.

Immunotherapy

One promising area of research is immunotherapy, which harnesses the power of the immune system to fight cancer. The immunotherapy drug pembrolizumab (Keytruda) has shown significant promise in treating ccRCC. A large clinical trial demonstrated that pembrolizumab can be an effective treatment option for patients with earlier-stage kidney cancer, including clear-cell renal cell carcinoma [13].

Targeted Therapy

Another approach is targeted therapy, which involves using medications to specifically target and kill cancer cells. Pazopanib, a tyrosine kinase inhibitor, has been evaluated in clinical trials as a potential treatment for ccRCC. While results have been mixed, pazopanib remains an option for some patients with this disease [5].

Chemotherapy

For less common types of non-clear cell RCC, such as collecting duct RCC or renal medullary carcinoma, chemotherapy may be the first line of treatment. However, it's essential to note that chemotherapy is not typically used as a primary treatment for clear cell RCC.

Current Treatment Landscape

The current treatment landscape for ccRCC involves a combination of surgery, targeted therapy, and immunotherapy. The choice of treatment depends on various factors, including the stage of cancer, overall health, and individual patient preferences.

In summary, while surgery remains the cornerstone of treatment for clear cell renal cell carcinoma, drug therapy plays an increasingly important role in managing this disease. Immunotherapy, targeted therapy, and chemotherapy are all being explored as potential treatment options, with pembrolizumab and pazopanib showing promise in clinical trials.

References:

[5] Pazopanib was evaluated in a randomized, placebo-controlled, international trial (VEG015192 [NCT00334282]) that enrolled 435 patients with clear cell or predominantly clear cell renal cell carcinoma. Nearly 50% of the patients had previously received cytokine therapy, although the remainder of them were treatment naïve.

[13] The immunotherapy drug pembrolizumab (Keytruda) has rapidly become one of the most widely used cancer treatments. Based on updated results from a large clinical trial, the drug is now part of an important milestone in the treatment of kidney cancer—specifically, clear-cell renal cell carcinoma, the most common form of the disease.. All participants in the trial had earlier-stage kidney cancer.

The differential diagnosis for kidney clear cell carcinoma (also known as clear cell renal cell carcinoma) includes several conditions that can mimic its appearance on imaging and histopathology.

Conditions to Consider

• Papillary RCC with cytoplasmic clearing: This type of papillary renal cell carcinoma can have a similar appearance to clear cell carcinoma, but it lacks the characteristic delicate chicken wire vasculature.
• Von Hippel-Lindau (VHL) disease and tuberous sclerosis complex: These genetic conditions can cause multiple cysts and tumors in the kidneys, which may be mistaken for clear cell carcinoma.
• Multilocular cystic renal neoplasm of low malignant potential: This rare tumor type can have a similar appearance to clear cell carcinoma on imaging, but it has a distinct histopathological profile.
• Clear cell papillary renal cell tumor: This recently recognized renal neoplasm is composed of an admixture of cystic, glandular, solid, and papillary structures, which may be confused with clear cell carcinoma.

Key Features to Distinguish

To accurately diagnose clear cell carcinoma, it's essential to look for the following features:

• Clear cells: The presence of clear cells with a characteristic "foamy" appearance is a hallmark of clear cell carcinoma.
• Chicken wire vasculature: A delicate network of blood vessels (chicken wire vasculature) is typically seen in clear cell carcinoma.
• CAIX and CD10 expression: Immunohistochemical staining for CAIX and CD10 can help confirm the diagnosis of clear cell carcinoma.

References

• [6] Clear cell renal cell carcinoma is the most common malignant tumor of renal epithelial origin, accounting for approximately 70% of renal tumors. (Source: #6)
• [7] Clear cell papillary renal cell carcinoma is a recently recognized renal neoplasm, composed of an admixture of cystic, glandular, solid, and papillary structures. (Source: #7)
• [10] CAIX (box-like pattern) and CD10, supportive of this diagnosis. Differential diagnosis. Papillary RCC with cytoplasmic clearing: Papillary architecture, lacks prominent delicate chicken wire vasculature. (Source: #10)

Please note that a definitive diagnosis requires careful consideration of clinical, imaging, and histopathological features, as well as consultation with a specialist in the field.