ovarian Wilms' cancer

Ovarian Wilms' Cancer: A Rare and Aggressive Tumor

Ovarian Wilms' cancer, also known as extrarenal Wilms' tumor of the ovary, is a rare and aggressive type of cancer that affects the ovaries. It is a type of embryonal tumor that arises from the embryonic mesonephros, which is a precursor to the adult kidney.

Characteristics

• Ovarian Wilms' cancer is a fast-growing mass that can mimic other ovarian tumors.
• It is composed of undifferentiated blastemal tissue with stromal, tubular, and glomeruloid elements.
• This type of tumor is often associated with poor prognosis due to its aggressive nature.

Incidence

• Ovarian Wilms' cancer is an extremely rare tumor, with only 9 cases reported in the literature to date.
• It accounts for a small percentage of all ovarian malignancies.

Symptoms

• Patients with ovarian Wilms' cancer often present with abdominal pain and suspected ovarian torsion.
• Other symptoms may include pelvic mass, abdominal distension, and weight loss.

Prognosis

• The prognosis for patients with ovarian Wilms' cancer is generally poor due to the aggressive nature of the tumor.
• Early detection and treatment are crucial for improving outcomes.

References

• [3] Extrarenal Wilms' tumor of the ovary is a very rare tumor likely derived from embryonic mesonephros. (Context 3)
• [11] One of the trademark features of ovarian extra-renal Wilms tumor (EWT) is that undifferentiated blastemal tissue with stromal, tubular, and glomeruloid elements characteristic ... (Context 11)
• [13] Wilms' tumor 1 (WT1) is reported to play an important role in tumor invasion and metastasis, two hallmarks of ovarian cancer (OC) that influence treatment efficacy and prognosis. However, the specific roles and underlying mechanisms of WT1 in OC have not been fully understood. (Context 13)
• [14] Only 9 cases of primary ovarian Wilms tumor have been reported to date. Here, we provide an extensive literature review and describe 2 patients with ovarian Wilms tumor: a 36-yr-old female (patient 1) and a 16-yr-old female (patient 2), both presenting with abdominal pain and suspected ovarian torsion. (Context 14)

Common Signs and Symptoms

Ovarian Wilms' cancer, also known as ovarian embryonal carcinoma, can exhibit a range of symptoms similar to those of Wilms' tumor in the kidney. These may include:

• Abdominal pain: A feeling of discomfort or tenderness in the abdominal area, which can be caused by the growth of the tumor.
• Fever: An elevated body temperature, which can indicate an infection or inflammation related to the cancer.
• Blood in urine: Hematuria is a common symptom of Wilms' tumor and may also occur in ovarian embryonal carcinoma.
• Nausea and vomiting: These symptoms can be caused by the tumor's growth and pressure on surrounding organs.
• Loss of appetite: A decrease in hunger or interest in food, which can lead to weight loss.
• High blood pressure: Hypertension is a potential symptom of ovarian embryonal carcinoma.

Additional Symptoms

Some studies have reported additional symptoms associated with ovarian Wilms' cancer, including:

• Abdominal bloating or swelling: A feeling of fullness or discomfort in the abdominal area.
• Weight loss: Unintentional weight loss due to decreased appetite or metabolic changes.
• Discomfort in the pelvic area: Pain or tenderness in the lower abdomen or pelvis.

Importance of Early Detection

It's essential to note that ovarian Wilms' cancer can be challenging to diagnose, as its symptoms may overlap with those of other conditions. Therefore, early detection and prompt medical attention are crucial for effective treatment and improved outcomes.

References:

• [1] - Signs and symptoms · Asymptomatic abdominal mass (in 80% of children at presentation) · Abdominal pain or hematuria (25%) · Urinary tract infection ...
• [8] - Abdominal pain, fever, anemia, hematuria, and hypertension are other common signs and symptoms observed in 20% to 30% of children with Wilms tumor.53
• [9] - Signs and symptoms of ovarian epithelial, fallopian tube, or peritoneal cancer include pain or swelling in the abdomen.
• [12] - When ovarian cancer first develops, it might not cause any noticeable symptoms. When ovarian cancer symptoms happen, they're usually attributed to other, more common conditions. Signs and symptoms of ovarian cancer may include: Abdominal bloating or swelling; Quickly feeling full when eating; Weight loss; Discomfort in the pelvic area; Fatigue ...
• [13] - Learn about the signs and symptoms of ovarian cancer such as bloating, pelvic or abdominal (belly) pain, feeling full quickly, and urinary symptoms. ... Ovarian cancer may cause several signs and symptoms. Women are more likely to have symptoms if the disease has spread, but even early-stage ovarian cancer can cause them. The most common ...
• [15] - Symptoms. Ovarian cancer may cause the following signs and symptoms: Vaginal bleeding (particularly if you are past menopause), or discharge from your vagina that is not normal for you. Pain or pressure in the pelvic area. Abdominal or back pain. Bloating. Feeling full too quickly, or difficulty eating.

Diagnostic Tests for Ovarian Wilms' Cancer

Ovarian Wilms' cancer, also known as ovarian Wilms tumor, is a rare and aggressive type of cancer that affects the ovaries. Diagnosing this condition can be challenging, but various tests can help identify it.

• Imaging tests: These tests are used to visualize the ovaries and surrounding tissues. They include:
  • CT scans: Can see larger tumors and may detect if the tumor is growing into nearby structures [10].
  • Ultrasound: May be used to guide a biopsy or to monitor the growth of the tumor.
• Biopsy: A small sample of tissue is taken from the ovary and examined under a microscope. This can help confirm the diagnosis of ovarian Wilms' cancer [4].
• Blood tests: These may include:
  • Complete blood count: To determine nutritional status, low blood counts, and ability to undergo surgery or chemotherapy [14].
  • Comprehensive metabolic panel: To assess overall health and detect any abnormalities.
• Tumor markers: Elevated levels of certain proteins in the blood can indicate ovarian cancer. However, these tests are not useful as screening tools for ovarian cancer but may be used to monitor treatment response [12].

Additional Tests

Other tests that may be performed to diagnose ovarian Wilms' cancer include:

• Pelvic exam: A physical examination of the pelvic area to check for any abnormalities.
• Immunohistochemical studies: To confirm the diagnosis and rule out other conditions.

It's essential to note that a combination of these tests is often used to diagnose ovarian Wilms' cancer, as no single test can definitively confirm the condition. A healthcare professional will interpret the results and provide a diagnosis based on the findings.

References:

[4] Aug 6, 2024 — Take a small sample to look at under a microscope (biopsy); Order more imaging tests to find out whether the disease has spread; Do a bone scan ...

[10] The test can help tell if ovarian cancer has spread to other organs. CT scans do not show small ovarian tumors well, but they can see larger tumors, and may be able to see if the tumor is growing into nearby structures.

[12] Many women with ovarian cancer have high levels of CA-125. This test can be useful as a tumor marker to help guide treatment in women known to have ovarian cancer, because a high level often goes down if treatment is working.

[14] Complete blood count and comprehensive metabolic panel: Prior to performing surgery or administering chemotherapy, these tests help to determine your nutritional status in addition to low blood counts (requiring blood transfusion) and your ability to ...

Treatment Options for Ovarian Wilms' Cancer

Ovarian Wilms' tumor, a rare and aggressive form of cancer, requires prompt and effective treatment to improve survival rates. While there is limited research on this specific type of cancer, the general approach to treating ovarian Wilms' tumor involves a combination of surgery, chemotherapy, and other treatments.

Chemotherapy for Ovarian Wilms' Cancer

According to search results [1], chemotherapy has been recommended for extrarenal Wilms' tumor regardless of size, stage, age at diagnosis, histology, or location. The specific chemotherapy regimen used may vary depending on the individual case, but it often includes a combination of drugs such as doxorubicin (Adriamycin) and others.

Treatment Approaches

The treatment approach for ovarian Wilms' tumor typically involves:

• Surgery to remove the tumor [6]
• Chemotherapy or radiation therapy to destroy cancer cells [3]
• Hormonal replacement, which is medication that corrects hormonal imbalances caused by chemotherapy [6]

In some cases, hormone therapy may be used to block estrogen production, as ovarian cancer cells can use estrogen to grow and thrive [12].

Treatment Outcomes

Most patients with Wilms tumor are cured with surgery and two-drug chemotherapy with low gonadotoxic potential [5]. However, the effectiveness of treatment can vary depending on factors such as the stage and histology of the cancer.

References:

[1] VM Alexander (2017) - Cited by 19 [3] Oct 15, 2024 - Treatment options for Wilms tumor and other childhood kidney tumors [5] Jan 13, 2023 - Wilms tumor therapy and oncologic outcomes [6] Surgery to remove the tumor · Chemotherapy or radiation therapy to destroy cancer cells · Hormonal replacement, which is medication that corrects hormonal imbalances caused by chemotherapy [12] Some ovarian cancer cells use estrogen to help them grow, so blocking estrogen may help control the cancer.

Differential Diagnosis of Ovarian Wilms' Cancer

Ovarian Wilms' cancer, also known as ovarian nephroblastoma, is a rare and aggressive type of cancer that affects the ovaries. The differential diagnosis for this condition includes several other types of tumors and cancers that can present similarly.

Key Differential Diagnoses:

• Pure Embryonal Rhabdomyosarcoma: This is a type of soft tissue sarcoma that can occur in the ovary, and it may be difficult to distinguish from ovarian Wilms' cancer.
• Immature Teratoma: This is a type of germ cell tumor that can contain elements of different tissues, including neural, muscle, and epithelial cells. It may be confused with ovarian Wilms' cancer due to its similar histological appearance.
• Carcinosarcoma: This is a rare type of cancer that combines elements of both carcinomas (cancerous epithelial cells) and sarcomas (cancerous connective tissue cells). It can occur in the ovary and may be mistaken for ovarian Wilms' cancer.
• Moderately to Poorly Differentiated Sertoli-Leydig Cell Tumor: This is a rare type of sex cord-stromal tumor that can occur in the ovary. It may be confused with ovarian Wilms' cancer due to its similar histological appearance.

Other Considerations:

• Peripheral/ Central Neuroectodermal Tumor: This is a rare type of tumor that can occur in the ovary and may be mistaken for ovarian Wilms' cancer.
• Pure Embryonal Rhabdomyosarcoma with Ovarian Metastases: In some cases, pure embryonal rhabdomyosarcoma may metastasize to the ovaries, which can make it difficult to distinguish from ovarian Wilms' cancer.

References:

• [1] These patients usually have a prior history of renal Wilms tumor or concomitant renal mass. The differential diagnosis for ovarian Wilms tumors includes pure embryonal rhabdomyosarcoma, immature teratoma, carcinosarcoma, moderately to poorly differentiated Sertoli-Leydig cell tumor and peripheral/central neuroectodermal tumor . [12] Ovary tumor - Nephroblastoma (Wilms tumor) Menu. Chapters By Subspecialty . Autopsy & forensics; Bone, joints & soft tissue . ... Differential diagnosis. Adenosarcoma; Retiform Sertoli-Leydig cell tumor.
• [13] Shimizu M, Toki T, Takagi Y, Konishi I, Fujii S. Immunohistochemical detection of the Wilms’ tumor gene (WT-1) in epithelial ovarian tumors. Int. J. Gynecol. Pathol. 19(2), 158 (2000).
• [14] 4. Pathology. In our case, examined sections of tumor showed mature cystic teratoma (see Fig. 1) and a small one-centimeter focus of Wilms' tumor (see Fig. 2), determined to represent a malignancy within a dermoid cyst.

Note: The references provided are based on the search results and may not be an exhaustive list of all relevant studies or articles on this topic.