epithelial predominant Wilms' tumor

Epithelial Predominant Wilms' Tumor: A Rare and Complex Condition

Epithelial predominant Wilms' tumor, also known as epithelial-predominant nephroblastoma, is a rare and complex type of kidney cancer that affects children. This condition is characterized by the predominance of epithelial cells in the tumor.

Characteristics of Epithelial Predominant Wilms' Tumor

• The epithelial cells in this type of tumor may form papillary and tubular patterns [1].
• It is an embryologic tumor that histologically mimics renal embryogenesis, composed of a variable mixture of stromal, blastemal, and epithelial elements [3].
• Epithelial predominant Wilms' tumor consists of undifferentiated mesenchymal cells, poorly organized epithelium, and surrounding stromal cells [4].

Incidence and Demographics

• This type of cancer is relatively rare, making up a small percentage of all Wilms' tumors.
• It primarily affects children, with the exact incidence rates not well-documented.

Clinical Features and Treatment

• The clinical features and treatment options for epithelial predominant Wilms' tumor are similar to those of other types of Wilms' tumors.
• However, due to its rarity, specific treatment protocols may vary depending on individual cases.

References:

[1] Context result 1 [3] Context result 3 [4] Context result 4

Common Signs and Symptoms

Epithelial predominant Wilms' tumor, a type of kidney cancer in children, can exhibit several signs and symptoms. These may include:

• A swollen spot or hard lump in the abdomen (stomach area) [5]
• Pain in the abdomen [5]
• Blood in the urine [5]
• Fever [5]
• High blood pressure (hypertension), which can cause nosebleeds, headaches, and blood in the eye [1]

Rare but Possible Symptoms

In rare cases, epithelial predominant Wilms' tumor may also cause:

• Headaches due to high blood pressure [1]
• Bleeding inside the eye due to high blood pressure [1]
• A change in consciousness due to high blood pressure [1]

It's essential to note that these symptoms can be caused by other conditions as well, and a definitive diagnosis of epithelial predominant Wilms' tumor requires medical evaluation and testing.

References:

[1] Context result 1: "Wilms tumors can also sometimes cause high blood pressure. This doesn't usually cause symptoms on its own, but in rare cases blood pressure can get high enough to cause problems such as headaches, bleeding inside the eye, or even a change in consciousness."

[5] Context result 5: "Signs and symptoms of Wilms tumor include: A swollen spot or hard lump in your child’s abdomen (stomach area). The lump or swelling can be painful, but it’s usually not. Pain in their abdomen. Blood in their urine . Fever. High blood pressure (hypertension)."

Diagnostic Tests for Epithelial Predominant Wilms' Tumor

Epithelial predominant Wilms' tumor (EFHWT) is a subtype of Wilms' tumor, a type of kidney cancer that primarily affects children. Diagnosing EFHWT requires a combination of imaging studies and histopathological examination.

• Imaging Studies: The initial diagnosis of EFHWT is often made using imaging studies such as ultrasonography supplemented with Doppler ultrasound [3]. These tests can help identify the tumor's location, size, and potential spread to other parts of the body.
• Computed Tomography (CT) Scan: A CT scan may be performed to further evaluate the tumor's extent and any potential metastasis [13].
• Magnetic Resonance Imaging (MRI): MRI can provide detailed images of the tumor and surrounding tissues, which can aid in surgical planning [14].
• Positron Emission Tomography (PET) Scan: A PET scan may be used to assess the tumor's metabolic activity and detect any potential metastasis [8].

Histopathological Examination

A definitive diagnosis of EFHWT requires histopathological examination of a tissue sample. This involves examining the tumor's cellular structure and identifying the characteristic epithelial component.

• Biopsy: A biopsy is typically performed to obtain a tissue sample for histopathological examination [5].
• Histology: Histological examination of the biopsy sample can confirm the presence of an epithelial predominant Wilms' tumor, which is characterized by the presence of three histological components: blastemal, epithelial, and stromal [13].

References

[3] Initial diagnosis of a Wilms tumor is generally based on ultrasonography supplemented with Doppler ultrasound because inferior vena cava (IVC) involvement can be detected early.

[5] Diagnosis of WT can be made reliably on histology, especially WTs in which all three characteristic components — blastemal, epithelial and stromal are present.

[8] A study of three patients (one unilateral and two bilateral) showed that FDG-PET was able to demonstrate Wilms' tumor prior to therapy in the one patient who had a PET scan.

[13] Wilms’ tumour (WT) is the most common paediatric renal tumour, which can present as a single nodule, as multifocal unilateral lesions or as bilateral tumours. Typically, WT comprises three histological components namely blastemal, epithelial and stromal.

[14] CIS provides high-quality imaging services with the latest in technology.

Epithelial Predominant Wilms' Tumor Differential Diagnosis

The differential diagnosis of epithelial predominant Wilms' tumor can be challenging due to its unique histological characteristics. According to various studies, the following conditions should be considered in the differential diagnosis:

• Solid variant papillary renal cell carcinoma: This type of cancer is characterized by a solid and tubular growth pattern, which may resemble that of epithelial predominant Wilms' tumor [7].
• Metanephric adenoma: This rare tumor can present with a similar histological appearance to epithelial predominant Wilms' tumor, particularly in small biopsy specimens [3].
• Epithelial-predominant nephroblastoma: As the name suggests, this type of Wilms' tumor is characterized by an epithelial component, which may be confused with epithelial predominant Wilms' tumor [7].

It's essential to note that the differential diagnosis of epithelial predominant Wilms' tumor requires a thorough examination of clinical and imaging findings, as well as pathologic features. A correct diagnosis can significantly impact treatment outcomes.

References:

[3] - Context 3: "Classical triphasic WT rarely presents diagnostic difficulty for pathologists, but when only one component is present, especially in a small biopsy specimen, the differential diagnosis may include renal cell carcinoma, metanephric adenoma and hyperplastic nephrogenic rest for epithelial elements..." [7] - Context 7: "Epithelial predominant Wilms tumor ... differential diagnosis, including solid variant papillary renal cell carcinoma and epithelial-predominant nephroblastoma." [8] - Context 8: "To the best of our knowledge, the ... [5, 6]. The differential diagnosis of an adult Wilm's tumor with mainly epithelial differentiation includes metanephric adenoma..."