ovarian sex cord-stromal benign neoplasm

Ovarian Sex Cord-Stromal Benign Neoplasms

Ovarian sex cord-stromal benign neoplasms are rare, non-cancerous tumors that develop from the cells in the core of the ovary. These tumors can arise from various types of cells, including granulosa cells, theca cells, Sertoli cells, Leydig cells, and fibroblasts.

Types of Benign Neoplasms

The most common type of ovarian sex cord-stromal benign neoplasm is the fibroma, which accounts for up to one-fourth of all benign ovarian neoplasms. Fibromas are characterized by a thick, mucoid material and can sometimes mimic cancerous tumors by producing ascites (abnormal fluid in the abdomen).

Other types of benign sex cord-stromal tumors include:

• Thecomas: These tumors develop from theca cells and are often associated with hormonal imbalances.
• Sertoli cell tumors: These rare tumors are formed by cell proliferations that resemble the Sertoli cells, which play a crucial role in the development of sperm cells.

Characteristics

Benign sex cord-stromal ovarian neoplasms can vary in size and may be asymptomatic or cause symptoms such as abdominal distention, pain, and menstrual irregularities. These tumors are usually diagnosed incidentally during imaging studies for unrelated reasons.

Treatment

The treatment for benign sex cord-stromal ovarian neoplasms typically involves surgical removal of the tumor. In some cases, hormonal therapy may be used to manage associated symptoms.

References

• [1] (Source 10)
• [2] (Source 11)
• [3] (Source 14)

Note: The references provided are based on the search results and may not be a comprehensive list of all relevant sources.

Common Signs and Symptoms

Ovarian sex cord-stromal tumors (SCSTs) can present with a range of symptoms, depending on the type and location of the tumor. Here are some common signs and symptoms:

• Abdominal Mass: A palpable mass in the abdomen is often the first symptom, especially if the tumor is large enough to cause discomfort or pain.
• Mild to Moderate Abdominal Pain: Some women may experience mild to moderate abdominal pain, which can be caused by the growing tumor or constipation.
• Constipation: Constipation is another common symptom, particularly if the tumor is located in the ovaries or belly.
• Shortness of Breath or Wheezing: If the tumor is located in the chest, it can cause shortness of breath or wheezing.

Hormonal Effects

Some SCSTs are hormonally active, which means they can produce estrogen or androgen hormones. This can lead to a range of symptoms, including:

• Estrogenic and Androgenic Effects: These effects can manifest as uterine bleeding, endometrial hyperplasia, or even endometrial carcinoma.
• Precocious Pseudopuberty: In rare cases, SCSTs can cause precocious pseudopuberty in young girls.

Rare but Serious Symptoms

In some cases, SCSTs can cause more serious symptoms, including:

• Sudden, Severe Abdominal Pain: This can occur if the tumor starts to bleed or causes ovarian torsion.
• Ascites with or without other abdominal symptoms: Ascites (fluid accumulation in the abdomen) can be a sign of advanced disease.

References

1. [1] CLINICAL PRESENTATION​​ As with other ovarian tumors, sex cord-stromal tumors usually present with the typical symptoms of an adnexal mass, including abdominal pain and constipation.
2. [3] Nonspecific signs and symptoms · Estrogenic and androgenic effects · Ascites with or without other abdominal symptoms.
3. [6] 70 Clinical presentations of juvenile granulosa cell tumors include precocious pseudopuberty, vaginal bleeding, irregular menstruation and, in rare cases, ...
4. [8] SIGNS & SYMPTOMS. SCSTs are frequently hormonally active. Tumors which secrete estrogen may cause uterine bleeding, endometrial hyperplasia, or even endometrial carcinoma.
5. [9] Affected women experience estrogen-related symptoms such as uterine bleeding, endometrial hyperplasia, and endometrial carcinoma; the latter has been reported ...

Diagnostic Tests for Ovarian Sex Cord-Stromal Benign Neoplasms

Ovarian sex cord-stromal tumors are a group of rare and usually benign neoplasms that develop from the sex cord or stroma of the ovary. When an ovarian sex cord-stromal tumor is suspected, several diagnostic tests can be used to confirm the diagnosis.

Blood Tests

• Inhibin levels: Inhibin levels may be elevated in ovarian sex cord-stromal tumors, making it a useful marker for diagnosis [1].
• Other tumor markers: Other tumor markers such as calretinin, CD56, SF-1, Melan-A, WT1, and FOXL2 may also be helpful in the diagnosis of sex cord-stromal tumors [6].

Imaging Studies

• Ultrasonography: Ultrasonography can be used to evaluate the size and location of the tumor, as well as its relationship to surrounding structures [4].
• Computed Tomography (CT): CT scans can provide more detailed information about the tumor's size, shape, and location, as well as any potential spread to lymph nodes or other organs [4].
• Magnetic Resonance Imaging (MRI): MRI can also be used to evaluate the tumor's characteristics and its relationship to surrounding structures [4].

Other Tests

• Physical examination: A physical examination by a healthcare provider may reveal an abdominal mass, which can suggest the presence of an ovarian sex cord-stromal tumor.
• Imaging-guided biopsy: In some cases, imaging-guided biopsy may be necessary to obtain tissue samples for histopathological examination.

It's worth noting that while these diagnostic tests can help confirm the diagnosis of an ovarian sex cord-stromal benign neoplasm, a definitive diagnosis can only be made through histopathological examination of tissue samples.

Treatment Options for Ovarian Sex Cord-Stromal Benign Neoplasms

Ovarian sex cord-stromal tumors are a rare type of tumor that can be either benign or malignant. While surgery is often the primary treatment option, drug therapy may also play a role in managing these tumors.

• Chemotherapy: Chemotherapy is not typically used as a first-line treatment for ovarian sex cord-stromal benign neoplasms. However, if the tumor is large or has spread to other parts of the body, chemotherapy may be considered.
  • The combination of bleomycin, etoposide, and cisplatin (BEP) is one chemotherapy regimen that has been used for ovarian sex cord-stromal tumors [4].
• Hormonal therapy: Hormonal therapies such as tamoxifen or aromatase inhibitors may be considered in some cases, particularly if the tumor is hormone-sensitive [1].
• Targeted therapy: Targeted therapies such as bevacizumab have been investigated for use in ovarian sex cord-stromal tumors, but more research is needed to determine their effectiveness [5].

Key Points

• Surgery remains the most effective treatment option for ovarian sex cord-stromal benign neoplasms.
• Chemotherapy may be considered if the tumor is large or has spread to other parts of the body.
• Hormonal therapy and targeted therapy may also play a role in managing these tumors, particularly if they are hormone-sensitive.

References

[1] Apr 11, 2018 - Treatment with a drug such as leuprolide (Lupron) and goserelin (Zoladex), the drug tamoxifen, or an aromatase inhibitor may be considered for ovarian sex cord-stromal tumors. [4] If your child needs chemotherapy, they may get the drugs cisplatin, etoposide, and bleomycin. These chemotherapy medicines have been used together for nearly 50 years to treat various types of cancer, including ovarian sex cord-stromal tumors. [5] Adding bevacizumab to weekly paclitaxel did not improve progression-free survival in women with relapsed sex cord-stromal tumors [5].

Differential Diagnosis of Ovarian Sex Cord-Stromal Benign Neoplasms

Ovarian sex cord-stromal tumors (SCSTs) are a rare type of tumor that can be either benign or malignant. When it comes to differential diagnosis, the following conditions should be considered:

• Fibrous ovarian tumors: These include Brenner tumors and adenofibromas, which are essentially fibrous in nature [1].
• Pedunculated tumors: These are a type of tumor that is attached to the ovary by a stalk-like structure.
• Adult granulosa cell tumors: This is the most common type of malignant sex cord-stromal tumor, and it can be difficult to distinguish from other types of ovarian tumors [4].
• Benign ovarian SCSTs: These account for less than 4% of all ovarian benign neoplasms [3].

Key Features to Consider

When making a differential diagnosis, the following features should be taken into consideration:

• Age and sex: Ovarian SCSTs typically present in the first two to three decades of life, with the exception of adult granulosa cell tumors [2].
• Clinical presentation: The chief differential diagnosis is often adult granulosa cell tumor with a diffuse pattern.
• Nuclear grooves: These are characteristic of adult granulosa cell tumors.

References

[1] Horta, M. (2015). MRI-based differential diagnosis of ovarian sex cord-stromal tumors. Cited by 197.

[2] Schultz, KAP. (2016). Ovarian sex cord-stromal tumors: A review of the literature. Cited by 132.

[3] Gershenson, DM. (Cited by 3). Benign ovarian SCSTs account for <4% of ovarian benign neoplasms.

[4] (2018). Adult granulosa cell tumor with a diffuse pattern: A case report. Cited by 1.

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