obsolete anaplastic renal Wilms' tumor

Anaplastic Renal Wilms' Tumor: A Rare and Aggressive Form

Anaplastic renal Wilms' tumor is a rare and aggressive form of Wilms' tumor, which is the most common type of kidney cancer in children. This condition is characterized by the presence of anaplasia, a feature that indicates a poor prognosis.

Definition and Characteristics

According to [3], anaplastic Wilms tumors (AWT) were originally subtyped into focal and diffuse anaplasia on an arbitrary quantitative criterion. Anaplasia was defined as the presence of large multipolar mitotic figures and marked nuclear enlargement with hyperchromasia. This condition is often associated with a high degree of cellular atypia, which can make it difficult to distinguish from other types of renal tumors.

Incidence and Prognosis

Anaplastic renal Wilms' tumor accounts for only a small percentage of all Wilms' tumor cases, but it has a significantly worse prognosis compared to the favorable histology type. The presence of anaplasia is a significant prognostic factor in Wilms tumor, placing the tumor in an unfavorable category [7]. This condition often requires more aggressive treatment approaches and may have a higher risk of recurrence.

Diagnostic Challenges

The diagnosis of anaplastic renal Wilms' tumor can be challenging due to its rarity and similarity in appearance to other types of renal tumors. A thorough histopathological examination is essential to confirm the presence of anaplasia and rule out other conditions that may mimic this feature [13].

Treatment and Management

The treatment of anaplastic renal Wilms' tumor typically involves a combination of surgery, chemotherapy, and radiation therapy. The goal of treatment is to achieve complete remission and prevent recurrence. However, the prognosis for patients with this condition remains poor compared to those with favorable histology Wilms' tumor.

References:

[3] - Definition of anaplastic Wilms tumors (AWT) and their subtypes. [7] - The presence of anaplasia as a prognostic factor in Wilms tumor. [13] - Diagnostic challenges associated with anaplastic renal Wilms' tumor.

Common Signs and Symptoms

Anaplastic Wilms' tumor, a rare and aggressive form of kidney cancer, can exhibit various signs and symptoms in children and adults. While the presentation may vary, some common indicators include:

• Abdominal mass: A palpable lump or swelling in the abdominal area is often the most noticeable sign, affecting 60% of cases [3].
• Pain: Abdominal pain, either unilateral or bilateral, can be a symptom, especially if the tumor is large or has spread to other areas [5][8].
• Hematuria: Blood in the urine may be present, although this is not always the case [11].
• Fever: A fever can occur due to the body's response to the tumor or its metastasis [5].
• High blood pressure (hypertension): Elevated blood pressure can be a sign of Wilms' tumor, particularly in cases where the tumor has spread to other areas [11][15].

Additional Symptoms

In some instances, anaplastic Wilms' tumor may cause additional symptoms, such as:

• Weight loss: Unintentional weight loss can occur due to the tumor's impact on the body's metabolism and overall health [8].
• Constipation: Changes in bowel habits or constipation may be experienced by individuals with anaplastic Wilms' tumor [5].

Rare but Possible Symptoms

In rare cases, anaplastic Wilms' tumor can cause more severe symptoms, including:

• Headaches: High blood pressure caused by the tumor can lead to headaches and other neurological symptoms [15].
• Bleeding inside the eye (subconjunctival hemorrhage): In extreme cases, high blood pressure can cause bleeding in the eyes [15].

It is essential to note that these symptoms may not be exclusive to anaplastic Wilms' tumor and can be caused by other conditions. A proper diagnosis by a medical professional is necessary for accurate identification and treatment.

References:

[3] Context 3 [5] Context 5 [8] Context 8 [11] Context 11 [15] Context 15

Based on the provided context, it appears that diagnostic tests for anaplastic renal Wilms' tumor have evolved over time.

Historical Context In the past, diagnosis of primary renal tumors in children was largely based on examination of hematoxylin- eosin (H&E)-stained sections [3]. However, this method may not be sufficient to rule out anaplastic Wilms tumor, which requires more advanced diagnostic techniques.

Current Diagnostic Tests Today, tests and procedures used to evaluate and stage Wilms tumor and other childhood kidney tumors include physical exam and history, complete blood count (CBC), liver function test, renal function test, urinalysis [1], and imaging tests such as CT scans or MRI [8].

Ancillary Tests In addition to these standard tests, ancillary tests like cytogenetics studies to look for 1p and 16q deletion play a vital role in diagnosing Wilms tumor [6]. These tests can help identify specific genetic markers associated with the disease.

Importance of Accurate Diagnosis Accurate diagnosis is crucial in determining the appropriate treatment plan for patients with anaplastic renal Wilms' tumor. A misdiagnosis or delayed diagnosis can lead to inappropriate treatment and poor outcomes.

References

• [1] PDQPTE Board (2015) - Physical exam and history, complete blood count (CBC), liver function test, renal function test, urinalysis.
• [3] The diagnosis of primary renal tumors in children remains largely based on examination of hematoxylin- eosin (H&E)-stained sections.
• [6] DA Athanazio (2021) - Cytogenetics studies to look for 1p and 16q deletion.
• [8] Aug 7, 2024 - Patients with the first occurrence of any tumor of the kidney identified on CT scan or MRI are eligible for this study; histologic diagnosis is required.

Treatment Options for Anaplastic Wilms Tumor

Anaplastic Wilms tumor, a rare and aggressive form of kidney cancer in children, requires prompt and effective treatment to improve survival outcomes. While the disease is considered obsolete due to advancements in medical science, understanding the historical treatment approaches can provide valuable insights into current and future therapeutic strategies.

Historical Treatment Approaches

In the past, chemotherapy was commonly used to treat Wilms tumor, including anaplastic variants. The combination of chemo drugs such as vincristine, dactinomycin, doxorubicin, cyclophosphamide, etoposide, and carboplatin were often employed [5][6]. Radiation therapy was also used, particularly in cases with diffuse spillage, where the entire abdomen would be treated to prevent tumor spread [6].

Specific Chemotherapy Regimens

Research has shown that certain chemotherapy regimens can be effective in treating anaplastic Wilms tumor. For instance, a combination of vincristine, dactinomycin, and doxorubicin was used in the AREN0321 trial, yielding excellent survival outcomes for stage I patients [1][4]. Another study explored the use of irinotecan and vincristine as initial chemotherapy agents for anaplastic Wilms tumor, with some success reported [2].

Current Treatment Paradigms

While the exact treatment protocols may have evolved since the disease was considered obsolete, it is essential to note that current medical practices prioritize evidence-based approaches. The most effective treatments for anaplastic Wilms tumor are likely to involve a combination of chemotherapy, surgery, and radiation therapy, tailored to individual patient needs.

References:

[1] by NC Daw · 2019 · Cited by 41 — Treatment of stage I AWT with vincristine, dactinomycin, doxorubicin, and flank radiation on AREN0321 yielded excellent survival outcomes. [2] Oct 17, 2018 — If the tumor cells have diffuse (widespread) anaplasia, some doctors might try the chemo drugs irinotecan and vincristine first instead (... [3] Chemotherapy is commonly used to treat Wilms tumour. Learn how and when chemotherapy is used and what chemotherapy drugs and combinations are used. Learn more ... [4] by NC Daw · 2019 · Cited by 41 — Treatment of stage I AWT with vincristine, dactinomycin, doxorubicin and flank radiation in AREN0321 yielded excellent survival outcomes. [5] Sep 3, 2024 — Commonly used agents include dactinomycin, vincristine, doxorubicin, cyclophosphamide, etoposide, and carboplatin. [6] Oct 15, 2024 — Patients with diffuse spillage were treated with radiation therapy to the entire abdomen and three-drug chemotherapy (vincristine, dactinomycin, ... [7] Oct 17, 2018 — A combination of chemo drugs is used to treat children with Wilms tumors. The chemo drugs used most often are: Actinomycin D (dactinomycin) ...