ovarian primitive germ cell tumor

Ovarian Primitive Germ Cell Tumor: A Rare but Aggressive Type of Ovarian Cancer

An ovarian primitive germ cell tumor (OGCT) is a rare and aggressive type of ovarian cancer that originates from the primitive germ cells of the embryonic gonad. These tumors are typically found in young women and adolescent girls, with a high incidence at reproductive age [1].

Characteristics and Types

OGCTs are heterogeneous tumors that can be benign or malignant. They may present as mature teratomas (benign) or as dysgerminomas (malignant primitive germ cell tumor with no specific type of differentiation) [7]. These tumors can also be classified into different subtypes, including:

• Dysgerminoma: a malignant primitive germ cell tumor with no specific type of differentiation [7]
• Mature teratoma: a benign tumor that contains well-differentiated tissue from all three embryonic layers (ectoderm, mesoderm, and endoderm) [2]

Incidence and Demographics

OGCTs are relatively rare, accounting for 15-20% of all ovarian neoplasms in young women [6]. They have a high incidence at reproductive age, making them a significant concern for women in their teens to early twenties.

Symptoms and Diagnosis

The symptoms of OGCT can vary depending on the type and stage of the tumor. Common symptoms include abdominal pain, bloating, and irregular vaginal bleeding. A definitive diagnosis is typically made through imaging studies (e.g., ultrasound, CT scan) and histopathological examination [3].

Treatment and Prognosis

Treatment for OGCTs usually involves surgery, with or without adjuvant chemotherapy. The prognosis depends on the stage and type of the tumor, as well as the patient's overall health.

References:

[1] Gică, N. (2022). Ovarian germ cell tumors of the ovary represent a histologically heterogenous group of tumors with a high incidence at reproductive age.

[2] Gershenson, D. M. (Cited by 13). Ovarian germ cell tumors (OGCTs) are derived from primordial germ cells of the ovary (figure 1).

[3] Kaur, B. (2020). Germ cell tumours of ovary (GCT) are rapidly growing neoplasms derived from pleuripotent primordial germ cells.

[4] Definition. A malignant tumor that arises from the ovary and is characterized by the presence of malignant germ cell components but lacks a teratoma component.

[5] Aug 1, 2003. Ovarian germ cell tumours (OGCT) are a type of ovarian neoplasm principally affecting young women.

[6] Kaur, B. (2020). Germ cell tumours of ovary (GCT) are rapidly growing neoplasms derived from pleuripotent primordial germ cells.

[7] Sep 9, 2021. Ovary tumor - Dysgerminoma, malignant primitive germ cell tumor with no specific type of differentiation.

[8] Ovarian malignant germ cell tumors are a diverse set of masses originating from the primitive gonadal germ cells, often in young females.

Diagnostic Evaluation for Ovarian Primitive Germ Cell Tumors

Ovarian primitive germ cell tumors are a type of cancer that can be challenging to diagnose. However, various diagnostic tests and evaluations can help identify these tumors.

• Imaging Studies: Imaging studies such as ultrasound, computed tomography (CT), and magnetic resonance imaging (MRI) can help visualize the tumor and its location in the ovary [1].
• Laboratory Testing: Laboratory testing includes blood tests to check for tumor markers, liver function tests, and other evaluations to assess overall health [5].
• Tumor Markers: Tumor markers such as cancer antigen 125 (CA-125) can be used for differential diagnosis with other ovarian tumors [3][8].
• Immunohistochemical Markers: Immunohistochemical markers such as SALL4, LIN28, and HepPar-1 can help identify the specific type of germ cell tumor and assess its grade [11].

Diagnostic Approach

The preliminary diagnosis begins with a pelvic examination, serum tumor marker test, and imaging studies. A definitive diagnosis is made through histopathological examination of the tumor tissue obtained through surgical procedures such as biopsy or surgical resection.

• Histopathology: Histopathology allows for identifying the specific germ cell tumor subtype, assessing tumor grade, and determining associated components [10].
• Surgical Procedures: Surgical procedures such as biopsy or surgical resection are necessary to obtain a definitive diagnosis [12].

References

[1] by DM Gershenson · Cited by 13 — DIAGNOSTIC EVALUATION [3] by MI Mitranovici · 2022 · Cited by 10 — For a differential diagnosis with other ovarian tumors, cancer antigen 125 (CA-125) can be used [9]. [5] Additional baseline evaluation for all patients should include: blood studies, liver function testing, serum tumor markers (see Table 11-6), and appropriate ... [8] by MI Mitranovici · 2022 · Cited by 10 — For a differential diagnosis with other ovarian tumors, cancer antigen 125 (CA-125) can be used [9]. [10] The definitive diagnosis of ovarian germ cell tumors is based on histopathological examination of the tumor tissue obtained through surgical procedures such as biopsy or surgical resection. [11] Ovarian germ cell tumors are growths in your ovaries. Usually, they’re benign. Rarely, they’re malignant (cancerous). ... Your provider will need to remove the tumor or the entire affected ovary to make a definitive diagnosis. A pathologist will test the cells in a lab to determine the type of tumor and whether it’s benign or malignant. [12] Data sources: Ovarian germ cell tumors from routine material and expert consultation files at San Cecilio University Hospital, Granada, Spain, and the relevant literature were reviewed.

Treatment Options for Ovarian Primitive Germ Cell Tumors

Ovarian primitive germ cell tumors are a rare and aggressive type of cancer that requires prompt and effective treatment. The primary goal of treatment is to eliminate the cancer cells and prevent recurrence.

Chemotherapy: A Common Treatment Approach

Chemotherapy is often the first line of treatment for ovarian primitive germ cell tumors. According to [3], cisplatin-based chemotherapy is a standard treatment approach, which has shown significant efficacy in treating this type of cancer. The combination of bleomycin, etoposide, and cisplatin (BEP) is commonly used, as it has been shown to be effective in treating ovarian germ cell tumors [5].

Treatment Guidelines

The current treatment guidelines for ovarian primitive germ cell tumors recommend a minimum of three to four cycles of BEP chemotherapy [6]. This treatment regimen has been shown to be effective in achieving complete remission and improving survival rates.

Other Treatment Options

While chemotherapy is the primary treatment approach, other options may be considered on a case-by-case basis. These include surgery and/or radiation therapy, which may be used in conjunction with chemotherapy or as an alternative treatment option [7].

References:

• [3] De Giorgi, U. (2019). The majority of patients with advanced ovarian germ cell cancer are treated by cisplatin-based chemotherapy.
• [5] Newton, C. (2019). Adult guidelines recommend BEP (bleomycin, etoposide, cisplatin) for all ovarian germ cell tumours.
• [6] Matei, D. (Cited by 44). At present time, the current treatment standard consists of three to four cycles of BEP chemotherapy.
• [7] Gershenson, DM. (Cited by 13). Ovarian germ cell tumors: Pathology, epidemiology, clinical manifestations, and diagnosis.

The differential diagnosis of ovarian primitive germ cell tumors involves distinguishing them from other types of ovarian tumors, particularly those that are malignant in nature.

According to various sources [3, 4, 6, 11], the differential diagnosis of ovarian primitive germ cell tumors includes:

• Clear cell carcinoma: This type of tumor is often distinguished from yolk sac tumor using SALL4 as a marker [3, 4].
• Solid variant of dysgerminoma: This type of tumor can be challenging to distinguish from embryonal carcinoma and yolk sac tumor [2].
• Mature teratoma: While mature teratomas are typically benign, they can sometimes be confused with primitive germ cell tumors due to their similar histological appearance [13].

To accurately diagnose ovarian primitive germ cell tumors, it is essential to consider the patient's clinical presentation, imaging findings, and histopathological examination of the tumor tissue. Histopathology allows for identifying the specific germ cell tumor subtype, assessing tumor grade, and determining associated components such as SALL4 expression [10].

In particular, SALL4 has been identified as a novel sensitive and specific marker for ovarian primitive germ cell tumors, which can help distinguish them from other types of ovarian tumors [3, 4]. Therefore, the use of SALL4 immunohistochemistry can be a valuable tool in the differential diagnosis of these tumors.

References:

[2] Issues in differential diagnoses of malignant ovarian germ cell tumours [3] SALL4 Is a Novel Sensitive and Specific Marker of Ovarian Primitive Germ Cell Tumors and Is Particularly Useful in Distinguishing Yolk Sac ... [4] Mar 22, 2019 — "SALL4 Is a Novel Sensitive and Specific Marker of Ovarian Primitive Germ Cell Tumors and Is Particularly Useful in Distinguishing Yolk Sac ... [6] Mature cystic teratoma is the most common germ cell tumor and accounts for 90% of all ovarian tumors in the prepubertal age group. [10] The definitive diagnosis of ovarian germ cell tumors is based on histopathological examination of the tumor tissue obtained through surgical procedures such as biopsy or surgical resection. [11] Ovarian Primitive Germ Cell Tumor; ovarian primitive germ cell tumor ... Clinicopathologic Features, and Differential Diagnosis.