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Mucinous ovarian cancer

A rare tumor, accounting for about 3% of all epithelial ovarian cancers.

Its gene-expression profile is distinct from that of serous ovarian cancer which makes up 65% of ovarian cancers.

Incidence has been stable.

65-80% of mutinous ovarian cancer is diagnosed at clinical stage I.

Mucinous ovarian cancers are usually large primary lesions, as big as 15 cm and generate symptoms while the disease is still localized to the ovary.

 

These tumors evolve in a stepwise fashion from a benign epithelium to a pre-invasive lesion, to carcinoma.

They are categorized as benign, borderline, or malignant.

Mucinous ovarian tumors are typically unilateral, multicystic, and large with the mean size of 10 cm if benign, 16 cm if borderline, and 20 cm if malignant.

Patients present early younger age and early stage compared with more common types of ovarian cancer.

The median age at presentation is 49 years versus 62 years for other types of ovarian cancer.

It is diagnosed in 74 to 85% of patients as stage I disease.

Risk factors, such as nulliparity, early menarche, increased, BMI, estrogen use, BRCA, 1/2 oncogenes  are not important factors for mucinous ovarian carcinoma.

Lesions are frequently mixed with areas of mucinous cystadenoma or precancerous lesions.

KRAS mutations are observed in 40-65% of mucinous carcinomas.

Common genetic aberrations, include TP53, KRAS, CDKN2A, and HER2 positivity.

The prognosis of mucinous ovarian carcinoma is much better than for other epithelial ovarian cancer subtypes.

The five-year overall survival for localized mutinous ovarian Cancer exceeds 90%.

Mucinous ovarian cancer that has spread to the peritoneum in the abdominal cavity or beyond, stage III or IV, the estimated median overall survival is between 12 and 33 months.

Mucinous ovarian carcinoma, is characterized by cysts, filled with a mucoid, opaque substance with solid regions.

They are characteristically diagnosed in younger patients than in patients with other epithelial ovarian cancer subtypes.

26% of patients are diagnosed in women than younger than 44 years.

It is the most common histological subtype in patients eligible for fertility sparing surgery.

None of the known risk factors for serous ovarian cancer including: nulliparity, early menarche, late menopause, and germline BRCA1 or BRCA2 mutations are risk factors for mucinous ovarian cancer.

Smoking is a risk factor.

For young patients with stage I disease wishing to preserve fertility a unilateral salpingo-oophorectomy is therapeutic.

For older stage I patients bilateral salpingo-oophorectomy is pref2242ed.

Surgery to prevent tumor rupture is paramount, as rupture changes the staging and influences management.

Compared with other epithelial subtypes peritoneal or appendiceal spread is a rare event.

Nodal spread risk is very low in apparent stage I mucinoud ovarian cases of less than 2%.

The prognosis for stage III or IV ovarian cancer is poorer than ithe prognosis of women with other, more common subtypes particularly serious or endometrioid ovarian cancer, and may be related to a poor response to chemotherapy.

Debulking surgery with the objective of complete resection is the cornerstone of management for advanced mucinous ovarian cancer.

The prognosis with the mucinous ovarian cancer depends on its stage.

Survival is higher for the majority of patients with stage I disease than for those with non-mucinous histological subtypes.

First line treatment of mucinous ovarian carcinoma is complete surgical resection of the tumor, the affected ovary, and associated fallopian tube.

Total abdominal bilateral salpingo-oophorectomy is typically performed for post menopausal patients.

Patients with stage I disease who desire future pregnancy the uterus and contralateral ovary may be left in place.

Patients with stage I mucinous ovarian carcinoma who undergo complete resection have a five-year survival of 90% or higher.

 

The prognosis is inverse for women with stage III mucinous ovarian cancer, who have significantly lower overall survival than women with non-mucinous histological subtypes.

Patients with mucinous ovarian cancer have lower response rates to first line platinum based chemotherapy than among women with serous ovarian cancer.

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