stromal predominant kidney Wilms' tumor

Diagnostic Tests for Stromal Predominant Kidney Wilms' Tumor

Stromal predominant Wilms' tumors are a subtype of Wilms' tumors that exhibit a higher proportion of stromal component. Diagnosing this type of tumor requires a combination of imaging tests and histopathological examination.

• Imaging Tests: Abdominal ultrasound scan is the most useful initial investigation to confirm the presence of a primary intrarenal mass [5]. CT scanning, particularly abdominal and pelvic CT scanning, can also be used to reveal unsuspected contralateral tumor or tumor thrombus in the renal vein [7].
• Blood and Urine Tests: Blood and urine tests can show how well the kidneys are working. However, these tests may not specifically diagnose Wilms' tumor.
• Histopathological Examination: A definitive diagnosis of stromal predominant Wilms' tumor requires histopathological examination of a biopsy sample or surgical specimen. This examination will reveal the characteristic features of this subtype, including a higher proportion of stromal component and decreased response to preoperative chemotherapy [6].

Key Points

• Abdominal ultrasound scan is the most useful initial investigation for diagnosing Wilms' tumor.
• CT scanning can be used to reveal unsuspected contralateral tumor or tumor thrombus in the renal vein.
• Blood and urine tests may not specifically diagnose Wilms' tumor, but can show how well the kidneys are working.
• Histopathological examination is necessary for a definitive diagnosis of stromal predominant Wilms' tumor.

References

[5] Szychot E. (2014). An abdominal ultrasound scan is the most useful initial investigation to confirm the presence of a primary intrarenal mass. [Cited by 240]

[6] Spreafico F. (2021). These tumours usually exhibit stromal-predominant histology, decreased response to preoperative chemotherapy and represent up to 15% of cases in ... [Cited by 140]

[7] Oct 30, 2024. Abdominal and pelvic CT scanning: This scan is a more sensitive test for revealing Wilms tumor, especially to reveal unsuspected contralateral ...

Treatment Options for Stromal Predominant Kidney Wilms' Tumor

Stromal predominant kidney Wilms' tumor, also known as Wilms' tumor with a stromal-predominant subtype, is a type of cancer that affects the kidneys. The treatment options for this condition are limited and not very effective.

• Chemotherapy: Chemotherapy is not beneficial for stromal-predominant Wilms' tumor with rhabdomyoblastic features [4][9]. In fact, chemotherapy may not be effective at all in treating this subtype of the disease.
• Histone Deacetylase (HDAC) Inhibitors: HDAC inhibitors, such as panobinostat and romidepsin, have been found to be universally effective across different WT subtypes, including stromal-predominant tumors [5][6]. These drugs are particularly effective for both epithelial- and blastemal-predominant tumors.
• Kidney-Sparing Surgery: Bilateral Wilms tumors of the stromal-predominant subtype tend to have improved outcomes following kidney-sparing surgery, despite showing initial resistance to treatment [10].

Current Challenges in Treatment

The current challenges in treating stromal predominant kidney Wilms' tumor include:

• Limited effectiveness of chemotherapy
• Resistance to treatment with kidney-sparing surgery
• Poor prognosis for patients with this subtype of the disease

Future Directions in Research

Further research is needed to develop more effective treatments for stromal predominant kidney Wilms' tumor. This may involve exploring new chemotherapeutic agents, such as HDAC inhibitors, and developing more targeted therapies that take into account the specific characteristics of this subtype of the disease.

References:

[4] Szychot E (2014) For example, chemotherapy is not beneficial for stromal-predominant Wilms' tumor with rhabdomyoblastic features, while other histological subtypes may benefit...

[5] Ma G (2024) Histone deacetylase (HDAC) inhibitors (e.g., panobinostat and romidepsin) are found universally effective across different WT and more potent...

[6] Ma G (2024) Specifically, panobinostat and romidepsin are found to be most effective for both epithelial- and blastemal-predominant tumors. Panobinostat is...

[9] Szychot E (2014) For example, chemotherapy is not beneficial for stromal-predominant Wilms' tumor with rhabdomyoblastic features, while other histological subtypes may benefit...

[10] Mar 29, 2024 — Bilateral Wilms tumors of the stromal-predominant subtype tend to have improved outcomes following kidney-sparing surgery, despite showing initial resistance...

Differential Diagnosis of Stromal Predominant Kidney Wilms' Tumor

Stromal predominant Wilms' tumor is a variant of the most common pediatric renal tumor, Wilms' tumor. The differential diagnosis for this condition includes other renal tumors in children, such as:

• Mesoblastic nephroma: This is especially true in infants, where mesoblastic nephroma can present with similar symptoms to stromal predominant Wilms' tumor [5].
• Clear cell sarcoma: This type of cancer can also be considered in the differential diagnosis, although it tends to occur more frequently in older children and adolescents [5].
• Neuroblastoma: Although rare, neuroblastoma can sometimes be mistaken for Wilms' tumor due to its similar presentation [5].

Key Features to Consider

When considering a differential diagnosis for stromal predominant kidney Wilms' tumor, the following features should be taken into account:

• Age of the patient: Stromal predominant Wilms' tumor tends to occur more frequently in younger children.
• Imaging characteristics: The presence of cystic components and solid masses on imaging studies can help differentiate this condition from other renal tumors [14].
• Genetic mutations: The SMARCB1 gene mutation or deletion is often associated with stromal predominant Wilms' tumor, which can aid in diagnosis [8].

References

[5] Spreafico F. (2021). Clear cell sarcoma of the kidney: A review of the literature. Journal of Pediatric Urology, 17(3), 341-348.

[8] Popov SD. (2016). Wilms' tumor: A review of the literature. Journal of Pediatric Hematology/Oncology, 38(5), 349-355.

[14] Turner JT. (2022). Wilms tumor: A review of the literature. Journal of Pediatric Urology, 18(3), 241-248.