Introduction
Primary ovarian carcinosarcoma is a rare type of cancer and accounts for about 0.5 percent of ovarian cancers. It is aggressive and contains both components of sarcoma (a type of cancer that develops from the mesenchymal cells, which are the connective tissue) and carcinoma (a type of cancer that develops from the epithelial cells that form the lining of the organs). This type of cancer occurs in postmenopausal women who are above 60 years old. This article explores the clinical features, diagnosis, treatment, and prognosis of primary ovarian carcinosarcoma.
What Is the Clinical Presentation of Primary Ovarian Carcinosarcoma?
The clinical presentation is similar to other types of ovarian cancer and may include the following symptoms:
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Abdominal or Pelvic Pain: This is the most common feature, and the pain can be dull or cramping in nature. It may be localized to one side of the abdomen.
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Bloating: Patients may experience abdominal distention or bloating, causing a feeling of fullness.
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Vaginal Bleeding: Postmenopausal women may present with abnormal vaginal bleeding.
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Other: Symptoms such as unexplained weight loss, increased frequency of urination, change in bowel habits, and fatigue can be observed.
What Is the Diagnosis of Primary Ovarian Carcinosarcoma?
Because of its rarity and complex nature, primary ovarian carcinosarcoma requires a multidisciplinary approach to accurately diagnose it, which involves:
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Clinical Presentation: Presence of symptoms such as pain in the abdomen and pelvic region and changes in bowel habits. Most of the symptoms are nonspecific and can be seen in other diseases.
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Imaging Studies: This includes ultrasound, MRI (magnetic resonance imaging), and CT (computed tomography) scan of the pelvic region. These imaging techniques help in assessing the size and characteristics of the tumor. Ultrasound can also aid in detecting the tumor and diagnosing ascites (fluid buildup).
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Cytological Examination: This involves the examination of cells under the microscope using cells obtained from fine needle aspiration, smears, or brushing. The features include both carcinoma and sarcoma components.
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Histopathological Features: This involves testing the tumor tissue obtained by biopsy or surgical resection, which also shows both carcinoma and sarcoma components.
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Immunocytochemistry: It is a diagnostic technique used to identify specific antigens or proteins in the given sample. Specific markers of carcinoma, such as vimentin, are positive for sarcoma. Other markers, such as CK7 (Cytokeratin 7) and EMA (epithelial membrane antigen), which are markers for epithelial cells, are positive for carcinoma.
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Genetic Testing: It may reveal the presence of stable microsatellite instability and may also be associated with BRCA1/2 gene (breast cancer gene 1 and 2) mutations and TP53 (tumor protein 53) mutations.
What Are the Cytological Features of Primary Ovarian Carcinosarcoma?
Cytological features refer to the appearance of cells under the microscope with the help of samples such as fine needle aspiration (FNA), smears, or brushing. The cytological features show different appearances of cells due to their biphasic nature, which involves components of sarcoma and carcinoma. The features based on the components include:
1. Carcinoma Component: This includes atypical glandular cells that are enlarged and irregular, along with anisokaryosis, which indicates a difference in nuclei size and an increased nuclear-to-cytoplasmic ratio. There can be variations in the shape and size of the cells. Carcinoma cells appear in clusters because of their epithelial origin.
2. Sarcoma Components: This includes the presence of mesenchymal cells with a spindle-like morphology, appearing as elongated spindle-shaped cells with less cytoplasm. These cells also show variation in shape and size. Immunocytochemical staining for vimentin shows a positive result in these cells, confirming their mesenchymal nature. In some cases, there can be the presence of rhabdomyoblasts, which are round cells with cross striations and eosinophilic staining. The sarcoma components can include different subtypes, such as:
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Fibrosarcoma: The sarcoma originates from fibroblasts, which produce collagen and other extracellular components. These types of sarcomas are aggressive in nature.
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Leiomyosarcoma: These arise from smooth muscle cells. These components are known for their metastasizing potential.
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Chondrosarcoma: These arise from cartilage cells and are mostly found in bone.
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Others: Primary ovarian carcinosarcoma may also show characteristics of rhabdomyosarcoma, which arises from skeletal muscle cells.
What Is the Management of Primary Ovarian Carcinosarcoma?
The management of primary ovarian carcinosarcoma requires a multidisciplinary approach, which includes:
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Surgery: Surgical removal of affected parts may involve the ovaries, fallopian tubes, uterus, or a part of the omentum. In some cases, suboptimal surgery may be performed due to the larger extent of the tumor, its critical location, or extensive spreading to surrounding organs. Complete resection can result in significant damage.
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Adjuvant Therapies: These may include chemotherapy with a combination of Paclitaxel, Carboplatin, and Bevacizumab. Further treatment may involve Ifosfamide and Cisplatin, along with radiation therapy, to reduce the risk of recurrence.
What Is the Prognosis of Primary Ovarian Carcinosarcoma?
The prognosis of primary ovarian carcinosarcoma is generally poor due to its aggressive nature. Most patients may experience relapse after the first year of initial treatment. Various factors influence the tumors, including age, tumor grade, presence of residual tumor even after surgery, presence of more than 25 percent of sarcomatous elements, overexpression of Ki-67 (a cell proliferation marker), p53 mutation (a tumor suppressor gene), and expression of VEGF (vascular endothelial growth factor), which is a protein involved in the formation of new blood vessels that can support tumor growth by providing oxygen and nutrients.
Conclusion
Primary ovarian carcinosarcoma is an aggressive tumor. Due to its rarity, it can pose challenges in diagnosis and effective management. Early detection and treatment with surgical removal and adjuvant chemotherapy have shown improved outcomes. However, ongoing research is important to further improve the prognosis of the patients.
