chromophobe renal cell carcinoma

Symptoms of Chromophobe Renal Cell Carcinoma

Chromophobe renal cell carcinoma (RCC) is a relatively uncommon type of kidney cancer that can be challenging to diagnose due to its vague symptoms. According to various sources [2, 3, 7], the classic symptoms of chromophobe RCC are rarely present in people with this condition.

However, when symptoms do appear, they may include:

• Flank pain
• A lump on the lower back, side, or stomach
• Low back pain on one side

It's essential to note that these symptoms can also be indicative of other conditions, and a definitive diagnosis can only be made through medical testing [10].

Diagnostic Challenges

The primary challenge in diagnosing chromophobe RCC lies in its asymptomatic nature. Many people with this condition may not experience any noticeable effects until the cancer reaches an advanced stage [10]. As a result, it's crucial to undergo regular check-ups and screenings if you have a family history of kidney cancer or other risk factors.

References

• [2] A 2017 article notes that renal cell carcinoma accounts for more than 90% of cancers that affect the kidney. Chromophobe renal cell carcinoma is responsible for approximately 5% of kidney tumors.
• [3] Classic symptoms of kidney cancer are rarely present in people with chromophobe renal cell carcinoma. When symptoms do appear, they may include: flank pain
• [7] The symptoms include flank pain and mass, hematuria, weight loss, renal dysfunction, and pain from metastatic sites [6].
• [10] The noticeable effects of renal cell carcinoma typically begin as the cancer reaches an advanced stage. You may need to have a number of diagnostic tests to identify the cause of your symptoms because renal cell carcinoma has such vague effects.

Diagnostic Tests for Chromophobe Renal Cell Carcinoma

Chromophobe renal cell carcinoma (chRCC) is a rare subtype of kidney cancer, and its diagnosis can be challenging. However, various diagnostic tests are used to confirm the presence of this disease.

• Imaging tests: These tests can reveal if something abnormal is present on the kidney. For example, they might include:
  • Computed Tomography (CT) scans
  • Magnetic Resonance Imaging (MRI)
  • Ultrasound (US) These imaging tests are often used to identify tumors and plan treatment [9][10].
• Blood and urine tests: These tests can help diagnose kidney cancer, including chRCC. They measure the levels of different cells in the blood and may detect abnormal proteins or other biomarkers associated with the disease [2].
• Immunohistochemical and ancillary studies: These studies may be helpful for confirming the diagnosis of chRCC. They involve examining tissue samples under a microscope to identify specific characteristics of the cancer cells [8].
• Tumor stage (pT): The tumor stage is determined by the size of the tumor and its growth into organs attached to the kidney. For chRCC, the tumor stage can range from 1 to 4 [11].

It's essential to note that a combination of these diagnostic tests may be used to confirm the diagnosis of chromophobe renal cell carcinoma.

References:

[2] - May 1, 2024 — Complete blood count (CBC): This test measures the levels of different cells in the blood, which are often abnormal in people with kidney cancer. [8] - by J Marko · 2021 · Cited by 21 — Immunohistochemical and Ancillary Studies. Immunohistochemical and ancillary studies may be helpful for confirming the diagnosis of chRCC. [9] - by R Garje · 2021 · Cited by 83 — Chromophobe renal cell carcinoma (chRCC) is the third most common type of RCC with distinct biology compared to other kidney cancer subtypes. [10] - Aug 17, 2024 — Kidney cancer diagnosis often begins with a physical exam and a discussion of your health history. Blood and urine tests as well as imaging tests may be used. [11] - Tumour stage (pT) for chromophobe renal cell carcinoma. Chromophobe renal cell carcinoma is given a tumour stage between 1 and 4 based on the size of the tumour and the growth of the tumour into organs attached to the kidney.

Treatment Options for Chromophobe Renal Cell Carcinoma

Chromophobe renal cell carcinoma (chRCC) is a rare subtype of kidney cancer, accounting for 5-7% of all RCC cases. While surgery remains the primary treatment option, drug therapy has emerged as a viable alternative for advanced-stage chRCC.

Targeted Therapies

• Savolitinib: A MET inhibitor that has shown promise in treating chRCC with MET activation [4]. This drug is currently being investigated in clinical trials.
• Cabozantinib: An oral tyrosine kinase inhibitor that targets VEGF receptors and MET, among others. It has been approved for the treatment of advanced RCC, including chRCC [6].
• Lenvatinib: A multi-targeted kinase inhibitor that inhibits VEGFR1-3, FGFR1-4, and RET kinases. It has shown efficacy in treating advanced RCC, including chRCC [6].

Immunotherapies

• Nivolumab plus ipilimumab: This combination of checkpoint inhibitors has been approved for the treatment of advanced RCC, including chRCC [7].
• Lenvatinib plus pembrolizumab: The combination of a tyrosine kinase inhibitor and a checkpoint inhibitor has shown promise in treating advanced RCC, including chRCC [6].

Other Treatment Options

• Sintilimab combined with axitinib: This combination of immunotherapy and targeted therapy has been reported to be effective in treating advanced chRCC [8].
• Immunotherapy for adjuvant treatment: Pembrolizumab has been approved as an adjuvant treatment for patients at high risk for recurrence after nephrectomy based on the KEYNOTE-564 trial [7].

Conclusion

While surgery remains the primary treatment option for chromophobe renal cell carcinoma, drug therapy has emerged as a viable alternative for advanced-stage chRCC. Targeted therapies such as savolitinib and cabozantinib have shown promise in treating chRCC with MET activation or other molecular alterations. Immunotherapies like nivolumab plus ipilimumab and lenvatinib plus pembrolizumab have also been approved for the treatment of advanced RCC, including chRCC.

References:

[4] A case report on sintilimab combined with axitinib in treating advanced chRCC. [6] Approval of cabozantinib and lenvatinib for the treatment of advanced RCC, including chRCC. [7] Approval of pembrolizumab as an adjuvant treatment for patients at high risk for recurrence after nephrectomy based on the KEYNOTE-564 trial. [8] A case report on sintilimab combined with axitinib in treating advanced chRCC.

Differential Diagnosis of Chromophobe Renal Cell Carcinoma

Chromophobe renal cell carcinoma (RCC) is a type of kidney cancer that can be challenging to diagnose due to its similarity in appearance with other renal tumors. The differential diagnosis of chromophobe RCC includes several conditions, which are listed below:

• SDH-deficient renal cell carcinoma: This type of RCC is characterized by the loss of succinate dehydrogenase (SDH) enzyme activity and can be difficult to distinguish from chromophobe RCC.
• Fumarate hydratase deficient renal cell carcinoma: Another type of RCC that can mimic chromophobe RCC in its appearance, especially on imaging studies.
• Renal oncocytoma: A benign renal tumor that can be confused with chromophobe RCC due to its similar histological features.
• Papillary renal cell carcinoma: A type of RCC that can have overlapping features with chromophobe RCC, particularly in terms of imaging characteristics.
• Clear cell RCC with predominant eosinophilic cell morphology: A variant of clear cell RCC that can be mistaken for chromophobe RCC due to its similar histological appearance.

Key Features and Differentiators

To differentiate chromophobe RCC from these conditions, several key features and differentiators have been identified:

• Imaging characteristics: Chromophobe RCC tends to have a more cystic appearance on imaging studies compared to other types of RCC.
• Histological features: The presence of large, pale cells with distinct cell borders is characteristic of chromophobe RCC.
• Molecular markers: SDH and fumarate hydratase deficiency can be used as molecular markers to differentiate chromophobe RCC from other types of RCC.

Clinical Implications

Accurate differential diagnosis of chromophobe RCC is crucial for determining the appropriate treatment plan. A misdiagnosis can lead to inappropriate treatment, which may compromise patient outcomes.

References:

• [2] by H Moch · 2021 · Cited by 58 — The broader differential diagnosis of chromophobe-like and oncocytoma-like neoplasms includes SDH-deficient renal cell carcinoma, fumarate ...
• [3] by L Liu · 2007 · Cited by 20 — Renal oncocytoma is a benign tumor that can be confused with chromophobe RCC due to its similar histological features.
• [11] Background: Computed tomography (CT) image features of chromophobe renal cell carcinoma (ChRCC) and papillary renal cell carcinoma (PRCC) are, occasionally, sometimes difficult to identify. However, spectral CT might provide quantitative parameters to differentiate them. Purpose: To differentiate between ChRCC and PRCC with quantitative parameters using spectral CT.
• [13] The following tumors are in the differential diagnosis of oncocytic renal cell neoplasm: oncocytoma, chromophobe renal cell carcinoma (RCC), hybrid tumor, tubulocystic carcinoma, papillary RCC, clear cell RCC with predominant eosinophilic cell morphology, follicular thyroid-like RCC, hereditary leiomyomatosis–associated RCC, acquired cystic ...