Ovarian Sarcoma

# Overview
Ovarian carcinosarcoma, which is also known as a malignant mixed mullerian tumor (MMMT) of the ovary, is a rare, aggressive cancer of the ovary with two distinct characteristic cancer types i.e carcinoma and sarcoma. Primary ovarian sarcomas occur as pure sarcomas or mixed mullerian tumors (MMTs). Ovarian sarcoma is one of the least common gynecologic malignancy, constituting approximately 1% of all ovarian malignancies. Prognosis is generally poor, and the 5-year survival rate of patients with ovarian sarcoma is approximately 28.2%. Most of the women are asymptomatic, when present, symptoms may include, pain in the abdomen or pelvic area, bloating or swelling of the abdomen, quickly feeling full when eating, other digestive problems. An elevated concentration of CA-125 in serum is seen in some patients of ovarian sarcoma. Biopsy is the study of choice. Findings on MRI suggestive of ovarian sarcoma include the following. Surgery is the mainstay of treatment for ovarian sarcoma.Among all chemotherapeutic regimens that are being used to treat ovarian sarcoma, they are divided into two groups like platinum containing regimens and non-platinum regimens. Cisplatin, carboplatin are commonly used.

# Historical Perspective
There is limited information available about the historical perspective of ovarian sarcoma

# Classification
- There is no established system for the classification of ovarian sarcoma.
- Primary ovarian sarcomas occur as pure sarcomas or mixed mullerian tumors (MMTs).
- Pure sarcomas are comprised of a single malignant mesenchymal element and are further categorized as:
Stromal cell sarcomas
Fibrosarcomas
Leiomyosarcomas
Neurofibrosarcomas
Rhabdomyosarcomas
Chondrosarcomas
Angiosarcomas
Liposarcomas
- Stromal cell sarcomas
- Fibrosarcomas
- Leiomyosarcomas
- Neurofibrosarcomas
- Rhabdomyosarcomas
- Chondrosarcomas
- Angiosarcomas
- Liposarcomas
- On the other hand mixed mullerian tumors(MMTs) are defined by the presence of both carcinomatous and sarcomatous elements and are more common than pure sarcomas.
- Ovarian MMTs can be further classified as homologous or heterologous on the basis of the tissue components present.
- Homologous tumors contain elements that are native to the ovary whereas heterologous tumors contain elements that normally are not present in the ovary.
The staging of  is based on the .
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There is no established system for the staging of .

# Pathophysiology
- The exact pathogenesis of ovarian sarcoma is not fully understood
- Clonal loss of the wild-type BRCA2 allele as well as the same somatic mutation of the TP53 gene was evident in histologic components

# Causes
The exact causes of ovarian sarcoma have not been identified.

# Differentiating ovarian sarcoma from Other Diseases
On the basis of age of onset, vaginal discharge, and constitutional symptoms, ovarian cancer must be differentiated from tubo-ovarian abscess, ectopic pregnancy, hydrosalpinx, salpingitis, fallopian tube carcinoma, uterine leiomyoma, choriocarcinoma, leiomyosarcoma, pregnancy, appendiceal abscess, appendiceal neoplasm, diverticular abscess, colorectal cancer, pelvic kidney, advanced bladder cancer, and retroperitoneal sarcoma.

# Epidemiology and Demographics
- Ovarian sarcoma is one of the least common gynecologic malignancy, constituting approximately 1% of all ovarian malignancies.
- In 2012, approximately 239,000 women were diagnosed with ovarian cancer worldwide, and 152,000 died of the disease.
- Patients of all age groups may develop sarcoma of the ovary both postmenopausal and premenopausal women.
- The incidence of ovarian Sarcoma increases with age; the median age at diagnosis is 50-60 years.
- There is no racial predilection to ovarian Sarcoma

# Risk Factors
There are no established risk factors for ovarian sarcoma

# Screening
There is insufficient evidence to recommend routine screening for ovarian sarcoma.

# Natural History, Complications, and Prognosis
- Ovarian carcinosarcomas follow a distinct natural history compared to other more common epithelial carcinomas
- Ovarian carcinosarcomas are aggressive neoplasms with a predilection towards early dissemination.
- Prognostic factors for this tumor type remain unclear because of its rarity.
- Prognosis is generally poor, and the 5-year survival rate of patients with ovarian sarcoma is approximately 28.2%.
- Some possible factors such as age and menopausal status have been proposed.

# Diagnostic Study of Choice
- There are no established criteria for the diagnosis of ovarian sarcoma
- Biopsy is the study of choice

# History and Symptoms
Most of the women are asymptomatic, when present, symptoms may include:
- Pain in the abdomen or pelvic area
- Bloating or swelling of the abdomen
- Quickly feeling full when eating
- Other digestive problems

# Physical Examination
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Common physical examination findings of  include , , and .
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The presence of  on physical examination is diagnostic of .
OR
The presence of  on physical examination is highly suggestive of .

# Laboratory Findings
- An elevated concentration of CA-125 in serum is seen in some patients of ovarian sarcoma.
- There are no diagnostic laboratory findings associated with ovarian sarcoma.

# Electrocardiogram
There are no ECG findings associated with ovarian sarcoma.

# X-ray
There are no x-ray findings associated with ovarian Sarcoma.

# Echocardiography or Ultrasound
There are no echocardiography/ultrasound findings associated with ovarian Sarcoma.

# CT scan
- CT scan may be helpful in the diagnosis of ovarian sarcoma . Findings on CT scan suggestive of ovarian sarcoma include:

# MRI
- MRI may be helpful in the diagnosis of ovarian sarcoma. Findings on MRI suggestive of ovarian sarcoma include the following:

# Other Imaging Findings
There are no other imaging findings associated with ovarian sarcoma

# Other Diagnostic Studies
There are no other diagnostic studies associated with .
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 may be helpful in the diagnosis of . Findings suggestive of/diagnostic of  include , , and .
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# Treatment

# Medical Therapy
There is no medical treatment for ovarian sarcoma, the mainstay of therapy is surgery and chemotherapy.

# Surgery
- Surgery is the mainstay of treatment for ovarian sarcoma.
- The management is similar to that of epithelial carcinoma of ovary, consisting of cytoreductive surgery followed by adjuvant chemotherapy.

# Chemotherapy
- Chemotherapy with various regimens has been used in different centers without defined conclusions on efficacy
- Multiple chemotherapeutic regimens have been evaluated with modest response rates ranging from 12% to 100%.
- Among all chemotherapeutic regimens that are being used, they are divided into two groups like platinum containing regimens and non-platinum regimens.
Platinum containing chemotherapy regimens
- Platinum containing chemotherapy regimens
- Carboplatin and ifosfamide (Carbo-I)
- Carboplatin (Carbo)
- Cyclophosphamide, adriamycin and cisplatin (CAP)
- Carboplatin and cyclophosphamide (Carbo-C)
- Epirubicin, carboplatin and 5FU (E-Carbo-F)
- Epirubicin, cisplatin and 5FU (ECF)
- Taxol and Carboplatin (T-Carbo)
- Other chemotherapy regimens
- Doxorubicin (A)
- Doxorubicin and cyclophosphamide (AC)
- Cyclophosphamide (IV)
- Cyclophosphamide (oral)
- Melphalan

# Primary Prevention
There are no established measures for the primary prevention of ovarian sarcoma.

# Secondary Prevention
There are no established measures for the secondary prevention of ovarian sarcoma.