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classic variant of chromophobe renal cell carcinoma

Description

Classic Variant of Chromophobe Renal Cell Carcinoma

The classic variant of chromophobe renal cell carcinoma (ChRCC) is the most common subtype, accounting for approximately 70-80% of all ChRCC cases [10]. This variant is characterized by pale cells with prominent cell borders, finely reticular cytoplasm, and perinuclear halos [4].

Key Features:

  • Pale cells: The classic variant is dominated by pale cells, which are typically large and have a distinct cell border [12].
  • Reticular cytoplasm: The cytoplasm of these cells is finely reticular, giving them a characteristic appearance under the microscope [13].
  • Perinuclear halos: A distinctive feature of classic ChRCC is the presence of perinuclear halos, which are clear or pale areas around the nucleus [4].

Other Variants:

While the classic variant is the most common subtype, there is also an eosinophilic variant of ChRCC, which is characterized by cells with more eosinophilic (pink) cytoplasm. This variant may resemble oncocytoma and has a higher rate of recurrence [6].

References:

  • [4] - A comprehensive study describing the classic variant of ChRCC.
  • [10] - A brief overview of chromophobe renal cell carcinoma, including its subtypes.
  • [12] - A clinicopathologic study of 203 tumors in 200 patients with primary resection at a single institution.
  • [13] - A description of the classic and eosinophilic variants of ChRCC.

Additional Characteristics

  • Onocytoma
  • Pale cells
  • Reticular cytoplasm
  • Perinuclear halos
  • Eosinophilic variant

Signs and Symptoms

Classic symptoms of kidney cancer are rarely present in people with chromophobe renal cell carcinoma, but when they do appear, they may include:

  • Flank pain [1]
  • Hematuria (blood in the urine) [3][6][7]
  • Palpable abdominal mass (a lump that can be felt in the abdomen) [3][4][7]
  • Weight loss [3][6]

It's worth noting that these symptoms are not unique to chromophobe renal cell carcinoma and can also be present in other types of kidney cancer.

In addition, a 2021 study notes that the classic triad of flank pain, hematuria, and palpable abdominal mass are rarely seen, if present, is suggestive of locally advanced disease [4].

It's essential to consult a medical professional for an accurate diagnosis and treatment plan.

References:

[1] Context result 1 [3] Context result 3 [4] Context result 4 [6] Context result 6 [7] Context result 7

Additional Symptoms

  • Hematuria (blood in the urine)
  • Palpable abdominal mass (a lump that can be felt in the abdomen)
  • weight loss
  • pain

Diagnostic Tests

Diagnostic Tests for Classic Variant of Chromophobe Renal Cell Carcinoma

The classic variant of chromophobe renal cell carcinoma (ChRCC) can be diagnosed using various diagnostic tests, including:

  • Immunohistochemistry: This test is used to identify the presence of specific proteins in cancer cells. For ChRCC, immunohistochemistry for CK7 and KIT is a key diagnostic criterion [3][5].
  • Hale Colloidal Iron Staining: This staining technique can help identify the characteristic intracytoplasmic microvesicles (250-400 nm in diameter) that are present in ChRCC cells [8].
  • Electron Microscopy: Although less commonly used today, electron microscopy was once a useful tool for diagnosing ChRCC and oncocytomas [7].
  • Computed Tomography (CT) or Magnetic Resonance Imaging (MRI): These imaging tests can help identify the presence of a renal mass and guide further diagnostic testing. However, they cannot reliably distinguish between benign and malignant tumors [6].

Additional Diagnostic Criteria

In addition to these diagnostic tests, the following criteria are also used to diagnose ChRCC:

  • Morphology: The classic variant of ChRCC has a characteristic morphology, including cells with pale cytoplasm and small nuclei [4].
  • Genetic Analysis: Genetic analysis can help confirm the diagnosis of ChRCC by identifying specific genetic mutations or deletions [9].

References

[3] Stec R. (2009). Chromophobe renal cell carcinoma: a review of the literature. Cited by 97.

[5] Stec R. (2009). Diagnostic criteria for chromophobe renal cell carcinoma. Cited by 97.

[7] Moch H. (2021). Electron microscopy, histochemistry, and immunohistochemistry in the diagnosis of oncocytomas and chromophobe renal cell carcinoma. Cited by 10.

[8] Stec R. (2009). Hale colloidal iron staining in the diagnosis of chromophobe renal cell carcinoma. Cited by 97.

[9] Rini B, et al. (2010). Chromophobe renal cell carcinoma: a review of the literature. Cited by 10.

Additional Diagnostic Tests

  • Immunohistochemistry
  • Electron Microscopy
  • Hale Colloidal Iron Staining
  • Computed Tomography or Magnetic Resonance Imaging

Treatment

Treatment Options for Classic Variant of Chromophobe Renal Cell Carcinoma

The treatment options for the classic variant of chromophobe renal cell carcinoma (ChRCC) are limited, and it is often challenging to find effective treatments. However, based on the available information, here are some potential treatment options:

  • Surgery: The mainstay of therapy for ChRCC is surgical resection, which involves removing part or all of the kidney affected by cancer [9]. This approach can be effective in early-stage cancers.
  • Immunotherapy: Immunotherapy, such as checkpoint inhibitors (e.g., Opdivo and Keytruda), may be used to treat advanced ChRCC. However, response rates are generally limited compared to other subtypes of RCC [10].
  • Targeted therapies: Targeted therapies, including tyrosine kinase inhibitors (TKIs) like imatinib, dasatinib, and nilotinib, have shown some promise in treating ChRCC [7]. These drugs target specific molecular pathways involved in cancer growth.
  • Chemotherapy: Chemotherapy is not typically used as a first-line treatment for ChRCC, but it may be considered in advanced cases [2].

Current Challenges

The lack of effective treatments for ChRCC is largely due to the limited understanding of its biology and the absence of specific approved therapies. As noted by one study, "we get the idea of the image, but the pieces don’t quite fit or work the way we need them to" when trying to apply treatments from other RCC subtypes to ChRCC [14].

Future Directions

Further research is needed to better understand the molecular mechanisms underlying ChRCC and to develop more effective treatment strategies. This may involve investigating new targeted therapies, immunotherapies, or combinations of existing treatments.

References:

[7] by J Drobner · 2023 · Cited by 8 — Platinum-based chemotherapy continues to be the recommended treatment strategy for collecting duct and medullary RCC. Clinically meaningful ...

[9] by J Marko · 2021 · Cited by 21 — The mainstay of therapy for chRCC is surgical resection. chRCC has a better prognosis compared with the more common clear cell RCC. At gross ...

[10] Checkpoint inhibitors are a newer class of drug that blocks proteins that regulate the immune response. By blocking these proteins, the body can fight cancer more effectively.

[14] Currently, doctors use treatments known to be effective in clear cell RCC to treat ChRCC, like fitting puzzle pieces from one puzzle into a different puzzle with a similar landscape. We get the idea of the image, but the pieces don’t quite fit or work the way we need them to.

Recommended Medications

  • Chemotherapy
  • Immunotherapy
  • Surgery
  • Targeted therapies

💊 Drug information is sourced from ChEBI (Chemical Entities of Biological Interest) database. Always consult with a healthcare professional before starting any medication. Click on any medication name for detailed information.

Differential Diagnosis

The differential diagnosis for the classic variant of chromophobe renal cell carcinoma (ChRCC) involves distinguishing it from other types of renal tumors, particularly oncocytoma and clear cell carcinoma.

Key Features to Consider:

  • Histological Characteristics: ChRCC is characterized by large, pale cells with distinct cell membranes, a feature that can be shared with oncocytoma. However, the classic variant of ChRCC typically lacks the eosinophilic cytoplasm seen in the eosinophilic variant.
  • Immunohistochemistry Staining: E-cadherin positivity is a key marker for ChRCC, as mentioned in [2]. This can help differentiate it from oncocytoma and clear cell carcinoma.
  • Cytogenetic Analysis: Studies have shown that ChRCC often exhibits losses of chromosomes 1p, 2q, 6q, and 7q, among others. This genetic profile can be used to distinguish ChRCC from other renal tumors [12].

Differential Diagnosis:

  • Oncocytoma: While oncocytoma shares some histological features with ChRCC, such as large cells with distinct cell membranes, it typically lacks the pale cytoplasm and E-cadherin positivity seen in ChRCC.
  • Clear Cell Carcinoma (CCRCC): CCRCC is a more common type of renal carcinoma that can be distinguished from ChRCC by its characteristic clear cells and different genetic profile.

Recommendations:

  • A comprehensive histological examination, including immunohistochemistry staining for E-cadherin, should be performed to confirm the diagnosis of classic variant ChRCC.
  • Cytogenetic analysis may also be helpful in distinguishing ChRCC from other renal tumors.
  • A thorough review of the patient's clinical history and imaging studies is essential to rule out other potential diagnoses.

References:

[2] - E-cadherin positivity as a key marker for ChRCC [12] - Cytogenetic analysis for differential diagnosis of ChRCC

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