ovarian endodermal sinus tumor

Ovarian Endodermal Sinus Tumor: A Rare and Aggressive Malignancy

The ovarian endodermal sinus tumor, also known as yolk sac tumor, is a rare and aggressive type of cancer that affects the ovaries. It is the second most common malignant germ cell tumor of the ovary, accounting for approximately 20% of all malignant germ cell tumors but less than 1% of all ovarian cancers [2].

Characteristics and Symptoms

This type of tumor is often difficult to diagnose due to its rapid growth and aggressive nature [3]. It can appear in various forms, including a solid or cystic mass, and may be accompanied by abdominal pain and an abdominal or pelvic mass [14]. The symptoms are often nonspecific, making it challenging for healthcare providers to identify the condition early on.

Prevalence and Epidemiology

Endodermal sinus tumors occur most frequently in children and young adults, with a peak incidence around the second decade of life [8][9]. They can also appear in older individuals, although this is less common. The exact cause of these tumors is unknown, but they are believed to arise from germ cells that have not yet developed into eggs or sperm.

Histological Features

Microscopically, endodermal sinus tumors exhibit a mixed solid/cystic nature with multiple histologic patterns [1]. They can be difficult to distinguish from other ovarian tumors, such as clear cell epithelial carcinoma, which shares similar morphological and clinical characteristics [15].

Treatment and Prognosis

The standard treatment for ovarian endodermal sinus tumor involves surgery followed by a chemotherapy regimen. The prognosis is generally poor, with a high risk of recurrence and metastasis [10]. However, early detection and aggressive treatment can improve outcomes.

References:

[1] 2C73.5 - endodermal sinus tumor, unspecified site, female [2] Malignant Ovarian Masses. Sean E. McSweeney, Mostafa Atri, in Gynecologic Imaging, 2011 [3] by MF Perry · 2020 · Cited by 5 — Ovarian endodermal sinus tumor with polyvesicular vitelline pattern: report of a case [8] Malignant ovarian germ cell tumors (MOGCT) constitute 5–10% of all ovarian malignancies, and the endodermal sinus tumor (EST) is the second most common member of this subgroup of ovarian tumors. [9] There are similarities between clear cell epithelial ovarian carcinoma and endodermal sinus tumours. Apart from the morphological and clinical characteristics there are immuno-histochemical markers of value in differentiating these 2 tumours and the detection of a raised serum AFP is character-stic of endodermal sinus tumours. [10] Usually, treating ovarian cancer is the same for all stages and types. Surgery should be performed, and a chemotherapy regimen ... [14] The endodermal sinus tumor (EST) is a rare tumor in its pure form, representing only a small fraction of adult germinal tumors. Although a rare histologic subtype among adult males, it is frequently encountered in ovarian germ cell tumors and is the most common germinal tumor of infants. [15] MalaCards integrated disease information for Ovarian Endodermal Sinus Tumor from 75 data sources.

Diagnostic Tests for Ovarian Endodermal Sinus Tumor

Ovarian endodermal sinus tumors (ESTs) are rare and aggressive tumors that can be challenging to diagnose. However, various diagnostic tests can help identify this condition.

• Imaging tests: A transvaginal ultrasound is often the first imaging test performed if an ovarian germ cell tumor is suspected [1]. This test can help detect any abnormalities in the ovaries.
• Blood tests: Blood tests can also be used to diagnose ESTs. Cancer antigen (CA)-125, lactate dehydrogenase (LDH), and α-fetoprotein (AFP) are some of the tumor markers that should be obtained for initial assessment [2].
• CT scan, PET/CT, and/or MRI scan: When the presence of ovarian cancer is detected, a CT scan or another radiologic procedure may be performed to confirm the diagnosis and assess the extent of the disease [3].
• Fine needle aspirate: A fine needle aspirate of the tumor can also be used to diagnose ESTs. This test involves using a thin needle to collect cells from the tumor for examination under a microscope.
• Tumor markers: Blood tests that look for elevated tumor markers, such as AFP and LDH, can help diagnose ovarian germ cell tumors, including ESTs [4].
• Placental alkaline phosphatase (PLAP): Approximately 40-80% of cases of yolk sac tumors are positive for PLAP, a marker of several different types of cancer [5].

These diagnostic tests can help identify ovarian endodermal sinus tumors and provide valuable information for treatment planning.

References:

[1] Context result 2 [2] Context result 4 [3] Context result 3 [4] Context result 5 [5] Context result 6

Treatment Options for Ovarian Endodermal Sinus Tumor

Ovarian endodermal sinus tumors, also known as yolk sac tumors, are a type of germ cell cancer that can be treated with a combination of surgery and chemotherapy.

• Chemotherapy: Combination chemotherapy is the mainstay of treatment for ovarian endodermal sinus tumor. The most commonly used regimen includes bleomycin, etoposide, and cisplatin (BEP) [6]. This chemotherapy protocol has been shown to improve prognosis significantly.
• Surgery: Surgery may be recommended in some cases, particularly if the tumor is localized or residual disease persists after initial chemotherapy [7]. The goal of surgery is to remove as much of the tumor as possible.

Current Treatment Recommendations

Recent studies have emphasized the importance of combination chemotherapy and optimal surgical management in improving prognosis for ovarian endodermal sinus tumors. A review of the literature highlights the effectiveness of BEP chemotherapy, with a significant improvement in survival rates [6].

• BEP Chemotherapy: The use of BEP chemotherapy has been widely adopted as the standard treatment protocol for ovarian endodermal sinus tumor [6].
• Surgical Management: Surgical management is tailored to individual cases and may involve removal of one or both ovaries, fallopian tubes, or other affected tissues [7].

Fertility-Preservation Surgery

In young patients, fertility-preservation surgery followed by immediate combination chemotherapy has been recommended as the treatment of choice [5]. This approach aims to balance cancer treatment with preservation of reproductive potential.

References:

[1] Yolk sac tumors (also known as primitive endodermal tumors or endodermal sinus tumors) are malignant primitive germ cell tumors. They are histologically similar to the mesenchyme of the primitive yolk sac.[1][2]

[5] YST is a curable disease, and that fertility-preservation surgery and subsequent immediate combination chemotherapy is the treatment of choice.

[6] Chemotherapy has dramatically improved the prognosis. Current treatment consists of surgery followed by bleomycin, etoposide, and cisplatin (BEP) chemotherapy.

[7] Surgery: Your provider may recommend an oophorectomy to remove one or both of your ovaries or fallopian tubes. · Chemotherapy: During chemotherapy, your provider ...

Note: The above information is based on the search results provided within the context.

Differential Diagnosis of Ovarian Endodermal Sinus Tumor

Ovarian endodermal sinus tumors (ESTs), also known as yolk sac tumors, are rare and highly malignant germ cell tumors. Diagnosing ESTs can be challenging due to their similarity in appearance with other ovarian lesions. Here are some key points to consider for differential diagnosis:

• Imaging Features: Complex solid and multicystic ovarian lesions, such as those described in [2], may present a diagnostic challenge. However, imaging features like microcystic or reticular patterns associated with elevated alpha-fetoprotein (AFP) levels in the bloodstream can be indicative of yolk sac tumors ([13]).
• Serum Alpha-Fetoprotein Levels: Elevated serum AFP levels are a critical diagnostic tool and monitoring parameter for yolk sac tumors ([13]). This marker is particularly useful in distinguishing ESTs from other ovarian lesions.
• Histological Similarity: Yolk sac tumors can be histologically similar to the mesenchyma of the primitive yolk sac, making differential diagnosis with other germ cell tumors challenging ([10]).
• Age Group and Presentation: Yolk sac tumors are common in the pediatric age group and can present with acute abdominal pain and elevated serum AFP levels ([11]).

Differential Diagnosis Considerations

When considering differential diagnosis for ovarian endodermal sinus tumors, it is essential to rule out other ovarian lesions that may present similarly. These include:

• Endometrioid Carcinoma: This type of carcinoma can be distinguished from yolk sac tumors by its different histological and imaging features ([12]).
• Germ Cell Tumors: Other types of germ cell tumors, such as mature and immature teratomas, dysgerminoma, and embryonal carcinoma, should also be considered in the differential diagnosis.
• Epithelial Tumors: Epithelial ovarian tumors, including serous and mucinous tumors, endometrioid and clear cell carcinomas, Brenner tumor, should be ruled out based on their distinct histological and imaging features.

Conclusion

Differential diagnosis of ovarian endodermal sinus tumors requires a thorough understanding of the imaging features, serum alpha-fetoprotein levels, and histological characteristics of these rare and highly malignant germ cell tumors. By considering these factors and ruling out other ovarian lesions, healthcare providers can make an accurate diagnosis and develop an effective treatment plan for patients with ESTs.