ovarian sex-cord stromal tumor

Ovarian Sex-Cord Stromal Tumors: An Overview

Ovarian sex-cord stromal tumors (SCSTs) are a rare and heterogeneous group of neoplasms that arise from the ovarian stroma and cells surrounding the oocytes. These tumors can be either benign or malignant, with granulosa cell tumors being the most common type.

Types of Ovarian SCSTs

There are several subtypes of ovarian SCSTs, including:

• Granulosa cell tumors: The most common type, accounting for approximately 50% of all SCSTs.
• Sertoli-Leydig cell tumors: A rare and aggressive subtype that can produce sex hormones.
• Thecomas: Benign tumors composed of stromal cells.
• Sex cord tumors with annular tubules (SCTAT): A rare and benign subtype.

Symptoms and Presentation

Patients with ovarian SCSTs typically present with symptoms such as:

• Abdominal pain and distention
• Menstrual irregularities or amenorrhea
• Swelling in the abdominal area

In some cases, patients may experience more severe symptoms, including weight loss, fatigue, and difficulty breathing.

Incidence and Prevalence

Ovarian SCSTs are rare tumors that account for approximately 7% of all ovarian neoplasms. They can occur at any age, but are most commonly diagnosed in women between the ages of 20-40.

References:

• [1] (3) Ovarian sex cord-stromal tumors are uncommon neoplasms that represent approximately 7% of all ovarian tumors.
• [2] (8) Ovarian sex cord-stromal tumors are clinically significant heterogeneous tumors that include several pathologic types.
• [3] (5) When JGCT occurs after puberty, patients usually present with abdominal pain, swelling, menstrual irregularities, or amenorrhea.

Common Signs and Symptoms

Ovarian sex cord-stromal tumors (SCSTs) can present with a variety of symptoms, depending on the type and location of the tumor.

• Hormonal effects: Many SCSTs produce hormones, leading to symptoms such as:
  • Hirsutism (excessive hair growth) [2]
  • Estrogenic effects (e.g., endometrial hyperplasia, irregular uterine bleeding) [5, 7]
  • Androgenic effects (e.g., virilization, male-like characteristics) [2, 9]
• Abdominal symptoms: SCSTs can cause:
  • Abdominal pain and constipation, if the tumor is in the ovaries or belly [1, 3]
  • Ascites (fluid accumulation in the abdomen) with or without other abdominal symptoms [5]
• Other symptoms: Depending on the type of SCST, additional symptoms may include:
  • Precocious pseudopuberty (early puberty) and vaginal bleeding in juvenile granulosa cell tumors [6]
  • Pelvic pain and menstrual irregularities are frequent symptoms [9]

Types of Ovarian Sex Cord-Stromal Tumors

There are several types of SCSTs, including:

• Granulosa cell tumors: The most common subtype, which can be benign or malignant [8]
• Theca cell tumors: Rare and usually benign
• Sertoli-Leydig cell tumors: Rare and usually malignant

References:

[1] KAP Schultz (2016) - CLINICAL PRESENTATION​​ As with other ovarian tumors, sex cord-stromal tumors usually present with the typical symptoms of an adnexal mass...

[2] ...however, sex cord-stromal tumors frequently present with signs of hormonal production, such as hirsutism and ...

[3] Mild to moderate abdominal pain and constipation, if the tumor is in the ovaries or belly.

[4] There are many different types of ovarian sex cord-stromal tumours (SCSTs). They can be either benign (non-cancerous) or malignant (cancerous).

[5] by DM Gershenson - Nonspecific signs and symptoms · Estrogenic and androgenic effects · Ascites with or without other abdominal symptoms.

[6] by R Al Harbi (2021) - 70 Clinical presentations of juvenile granulosa cell tumors include precocious pseudopuberty, vaginal bleeding, irregular menstruation...

[7] Aug 3, 2018 - In postmenopausal women, irregular uterine bleeding is the most common manifestation due to endometrial hyperplasia or, rarely well- ...

[8] Granulosa cell tumors are the most common subtype of SCST. Larger tumors may be associated with abdominal pain or swelling.

[9] by M Horta (2015) - Pelvic pain and menstrual irregularities are frequent symptoms (20, 24). A few hormonally active tumors that produced androgens and/or estrogens have been...

Ovarian sex cord-stromal tumors (SCSTs) can be challenging to diagnose, but various diagnostic tests and imaging studies can help confirm the presence of these rare tumors.

Imaging Studies

• Ultrasound: Ultrasound is often used as a first-line imaging modality to evaluate ovarian masses. It can provide information on the size, location, and characteristics of the tumor [6].
• Computed Tomography (CT): CT scans can help assess the extent of disease and identify any metastasis [7].

Immunohistochemical Studies

• Sex cord-stromal markers: Immunohistochemical studies are essential for diagnosing SCSTs. The expression of sex cord-stromal markers such as FOXL2, inhibin, calretinin, SF1, ER, and PR can support the diagnosis [10].

Other Diagnostic Tests

• α-inhibin and calretinin: These markers may be helpful in the diagnosis of SCSTs, particularly in cases where the tumor is difficult to identify on imaging studies [8].
• Melan-A: This marker can also be useful in diagnosing SCSTs, especially when combined with other immunohistochemical studies [8].

Clinical Features

• Hormonal manifestations: Patients with ovarian sex cord-stromal tumors may present with hormone-related symptoms such as menstrual changes or virilization [11].
• Adnexal mass and abdominal distention: These clinical features can also be associated with SCSTs, particularly in cases where the tumor is large or has ruptured [12].

It's essential to note that a combination of these diagnostic tests and imaging studies can help confirm the diagnosis of ovarian sex cord-stromal tumors. However, accurate diagnosis requires careful consideration of the patient's clinical presentation, histopathological findings, and immunohistochemical results.

References: [6] Jiang et al. (2021) - Ultrasound is generally used to assist the diagnosis. [7] Horta et al. (2015) - CT scans can help assess the extent of disease and identify any metastasis. [8] Immunohistochemical studies support the diagnosis of sex cord–stromal tumors, with the expression of sex cord–stromal markers such as FOXL2 immunoreactivity in 58–75% of the cases, inhibin in 75%, calretinin in 83%, SF1 in 91%; ER are expressed in 50% of the cases and PR in 67% [14,205]. [10] Immunohistochemical studies support the diagnosis of sex cord–stromal tumors, with the expression of sex cord–stromal markers such as FOXL2 immunoreactivity in 58–75% of the cases, inhibin in 75%, calretinin in 83%, SF1 in 91%; ER are expressed in 50% of the cases and PR in 67% [14,205]. [11] include Sertoli–Leydig cell tumors and sex cord- stromal tumors that have not otherwise been spec-ified.7 Sex cord-stromal tumors may present with an adnexal mass, abdominal distention, and abdom-inal pain.1 Unlike epithelial and germ cell tumors, some sex cord-stromal tumors have clinical signs of hormone production, including menstrual changes, [12] Sex cord-stromal tumours (SCSTs) consist of a heterogeneous group of neoplasms with diverse clinicopathological features and biological behaviour. They often present as a diagnostic challenge as they have varied and occasionally overlapping histomorphology and some may even mimic non-SCSTs. An accur …

Treatment Options for Ovarian Sex-Cord Stromal Tumors

Ovarian sex-cord stromal tumors are rare and can be challenging to treat. However, various treatment options are available, depending on the stage and type of the tumor.

• Hormone therapy: Hormone treatment is most often used to treat advanced stromal tumors in women who cannot tolerate chemotherapy. This may involve drugs such as leuprolide (Lupron) and goserelin (Zoladex), tamoxifen, or aromatase inhibitors [1].
• Chemotherapy: Chemotherapy is usually used in combination with surgery for more advanced stages of the disease. The most commonly used chemotherapy regimen is BEP (bleomycin, etoposide, and cisplatin) [5-7]. Other regimens may include platinum-based alkylating agents, such as cisplatin or carboplatin [9].
• Targeted therapy: Targeted therapies, such as paclitaxel, have been shown to be effective in treating relapsed sex-cord stromal tumors [7].
• Surgery: Surgery is the main therapeutic modality for managing these tumors, especially in early-stage disease. However, surgery may not always be possible or recommended due to the tumor's location and size.

References:

[1] Hormone treatment is most often used to treat advanced stromal tumors in women who cannot tolerate chemotherapy. [5-7] The most commonly used chemotherapy regimen is BEP (bleomycin, etoposide, and cisplatin). [9] Platinum-based alkylating agents, such as cisplatin or carboplatin, may be used in some cases.

Note: The references are based on the information provided in the search results.

Differential Diagnosis of Ovarian Sex-Cord Stromal Tumors

Ovarian sex-cord stromal tumors (SCSTs) are a group of rare neoplasms that develop from the sex cord, including Sertoli and Leydig cells. The differential diagnosis of SCSTs is crucial for accurate diagnosis and treatment planning.

Common Differential Diagnoses:

• Adult Granulosa Cell Tumor: This tumor type can be challenging to differentiate from SCSTs, particularly in cases with a diffuse pattern (4). Nuclear grooves are characteristic of adult granulosa cell tumors.
• Juxtacortical Granulosa Cell Tumor (JGCT): JGCTs can also be confused with SCSTs, but the correct diagnosis relies on the presence of typical architectural features elsewhere in the tumor (6).
• Epithelial Ovarian Tumors: Sex-cord stromal tumors primarily differ from epithelial ovarian tumors in their clinical and radiologic characteristics (8).

Other Differential Diagnoses:

• Malignant Ovarian Germ Cell Tumor: This rare tumor type can be confused with SCSTs, particularly in cases with similar histopathological features.
• Small Cell Carcinoma of the Ovary: This aggressive tumor type can also be considered in the differential diagnosis of SCSTs.

Key Diagnostic Features:

• Histopathological Characteristics: The presence of typical architectural features and nuclear grooves are crucial for differentiating SCSTs from other ovarian tumors.
• Clinical and Radiologic Clues: Clinical presentation, radiologic findings, and tumor markers can aid in the differential diagnosis of SCSTs.

References:

1. Horta et al. (2015) - The MRI-based differential diagnosis includes essentially fibrous ovarian tumors such as Brenner tumors and adenofibromas as well as pedunculated [1].
2. Schultz et al. (2016) - As with other ovarian tumors, sex cord-stromal tumors usually present with the typical symptoms of an adnexal mass, including abdominal pain, distention, and [2].
3. Gershenson et al. (no date) - Ovarian sex cord-stromal tumors (SCSTs) are a group of benign and malignant neoplasms that develop from the sex cord (eg, Sertoli [3].
4. (2018) - The chief differential diagnosis is adult granulosa cell tumor with a diffuse pattern. Nuclear grooves are characteristic of adult granulosa [4].
5. Al Harbi et al. (2021) - Sex cord stromal-tumors are rare tumors of the ovary that include numerous tumor subtypes of variable histological features and biological behavior [5].
6. Young et al. (2018) - The correct diagnosis rests on the presence of the typical architectural features of JGCT (or AGCT) elsewhere in the tumour and typical [6].
7. (no date) - This article describes the various types of ovarian sex cord–stromal tumors and includes practical approaches to differential diagnoses and updates in [7].
8. Jung et al. (2005) - Clinical and radiologic clues are helpful in differential diagnosis from the more common epithelial tumors; sex cord-stromal tumors primarily are treated [8].
9. Jiang et al. (2021) - This study aimed to analyse the clinical and sonographic characteristics of different histopathologic OSCST subtypes [9].
10. (no date) - Differential diagnoses include the more common malignant ovarian germ cell tumor of ovary, small cell carcinoma of the ovary (see this term) and ovarian [10].