obsolete malignant ovarian clear cell neoplasm

Characteristics of Ovarian Clear Cell Carcinoma

Ovarian clear cell carcinoma (OCCC) is a rare and aggressive subtype of epithelial ovarian cancer, accounting for approximately 5-25% of all epithelial ovarian carcinomas [5][10]. It is more common in Asian countries, where it constitutes around 25% of ovarian cancers [4].

Histological Features

Clear cell carcinoma of the ovary is characterized by a predominance of clear and hobnail cells, with an immature glomerular pattern [14]. The tumor often has a solid or cystic appearance, with areas of necrosis and hemorrhage.

Clinical Behavior

OCCC tends to have a worse prognosis than high-grade serous epithelial ovarian cancer (EOC), with clinical experience and research findings confirming the remarkable differences in clinical behavior, molecular alterations, and pathogenesis [12].

Molecular Alterations

The diagnosis of OCCC is typically set at a histological level. However, studies have shown that OCCC often harbors alterations in PIK3CA, ARID1A, TP53, KRAS, and PTEN genes [10]. These molecular changes may contribute to the aggressive behavior of this subtype.

Precancerous Lesions

Endometriosis is a recognized precancerous lesion that carries a 3-fold increased risk of OCCC. This association highlights the importance of considering endometriosis as a potential precursor to ovarian clear cell carcinoma [15].

In summary, ovarian clear cell carcinoma is a rare and aggressive subtype of epithelial ovarian cancer with distinct histological features, clinical behavior, and molecular alterations.

References:

[4] by K Takahashi · 2021 · Cited by 32 [5] by B McNamara · 2023 · Cited by 2 [10] NCI Definition [12] by TY Sia · 2022 · Cited by 13 [14] by BL Kong · 2024 · Cited by 1 [15] by K Takahashi · 2021 · Cited by 32

Treatment Options for Ovarian Clear Cell Carcinoma

Ovarian clear cell carcinoma (OCCC) is a rare and aggressive form of epithelial ovarian cancer that has a poor prognosis due to its resistance to platinum-based chemotherapy. However, various treatment options are being explored to improve patient outcomes.

• Conventional Chemotherapy: While platinum-based chemotherapy is commonly used for treating OCCC, it has shown limited efficacy in this type of cancer [10][11]. Alternative chemotherapeutic agents, such as taxanes and topoisomerase inhibitors, may be considered based on individual patient characteristics and tumor biology.
• Targeted Therapies: Research has identified distinct molecular alterations in OCCC, including ARID1A mutations, which may serve as potential targets for therapy [14]. Investigational targeted therapies, such as those targeting the PI3K/AKT pathway, are being explored in clinical trials.
• Immunotherapy: Immune checkpoint inhibitors have shown promise in treating various types of cancer, including ovarian cancer. However, their efficacy in OCCC remains to be determined and is currently under investigation [2].
• Hormonal Therapy: Given the association between endometriosis and OCCC, hormonal therapies may be considered as a potential treatment option for patients with this underlying condition.

Emerging Treatment Strategies

New approaches are being investigated to improve outcomes in patients with OCCC. These include:

• Combination Therapies: Combining conventional chemotherapy with targeted or immunotherapeutic agents may enhance treatment efficacy and overcome resistance.
• Precision Medicine: Molecular profiling of individual tumors is being explored to identify specific genetic alterations that can guide personalized treatment decisions.

Current Recommendations

While there are no established guidelines for treating OCCC, the following general principles can be applied:

• Surgery: Cytoreductive surgery may be considered in selected patients with early-stage disease.
• Chemotherapy: Platinum-based chemotherapy remains a standard treatment option, although its efficacy is limited. Alternative chemotherapeutic agents and combination therapies are being explored.

Future Directions

Further research is needed to develop effective treatment strategies for OCCC. Investigational studies should focus on identifying novel molecular targets, exploring the potential of immunotherapy and targeted therapies, and evaluating combination regimens that can improve patient outcomes.

References:

[10] by T Laga · 2021 · Cited by 7 [11] by JA Ledermann · 2013 · Cited by 1116 [14] by A Davis · 2014 · Cited by 430

Differential Diagnosis of Malignant Ovarian Clear Cell Neoplasms

Malignant ovarian clear cell neoplasms, also known as clear cell carcinomas (CCCs), are a type of epithelial ovarian cancer. The differential diagnosis for CCCs can be challenging due to their similarity in appearance with other types of tumors.

Key Differential Diagnoses:

• Primitive Germ Cell Tumors: These tumors can mimic the appearance of CCCs, especially in early-stage disease [5].
• Sex Cord Stromal Tumors: Tumors such as fibrothecoma and granulosa cell tumor can be difficult to distinguish from CCCs based on morphology alone [13].
• Metastasis: Clear cell carcinomas can metastasize to the ovaries, making it essential to consider a primary site of origin when diagnosing ovarian tumors [15].

Additional Considerations:

• Papillary Features: Ovarian clear cell carcinoma with papillary features can mimic serous tumor of low malignant potential [11].
• Molecular Differences: Clear cell carcinomas have distinct molecular characteristics that can aid in differential diagnosis, such as negative α-fetoprotein and CD10 expression [12].

Clinical Implications:

Accurate preoperative diagnosis and characterization of benign and malignant adnexal lesions are crucial for patient management. Ultrasound is the primary imaging modality for ovarian neoplasms, but it may not always provide a definitive diagnosis.

References:

[5] D Jelovac (2011) - Patients with ovarian clear cell cancer frequently have a clinical history of endometriosis. [11] Am. J. Surg. Pathol. 32(2), 269–274 - Ovarian clear cell carcinoma with papillary features: a potential mimic of serous tumor of low malignant potential. [12] Ovarian clear cell carcinoma (OCCC) is a special pathological type of epithelial ovarian carcinoma (EOC). [13] Ovarian tumors are classified on the basis of tumor origin as epithelial tumors, germ cell tumors, sex cord–stromal tumors, and ... [15] The differential diagnostic problems usually associated with clear cell carcinoma (CCC) of the ovary have been well characterized.