Ovarian TumorsSanjae-Ann Dean

Objectives

Incidence

Types and classification

Special Features

Prognosis

Presentation

Diagnosis

Treatment

Conclusion

Incidence

In 2012

20,785 women in the United States were diagnosed with ovarian cancer.

14,404 women in the United States died from ovarian cancer.

Ovarian cancer is the 5th leading cause of cancer death amongst women. It is also the most common cause of mortality related to gynaecological malignancy in developed countries.

However in the Caribbean, Ovarian malignancies are 2nd to cervical malignancy as the leading cause of mortality related to gynecological tumors

The 5 year survival rate for ovarian malignancies is low. It is approximately 30-35%.

This may be due to the lack of an appropriate screening tool. It may also be accounted for by the fact that most cases present late in which case the disease has already metastasized and palliation is the only appropriate method of management.

Nulliparity

Low parity

Early menarche

Late menopause

Family hx of ovarian, breast, endometrial or colonic ca

BRCA1 or BRCA2 gene

OHSS

Multiparity

Use of OCP

Risk Factors Protective Factors

Classification

Ovarian tumors are quite diverse in nature. Primary tumors arise from three cell types located in normal ovarian tissue.

These are :

1. Coelomic epithelium (multipotent surface epithelium)

2. Germ cells (totipotent)

3. Sex cord-stromal cells

Surface epithelial tumors

Serous tumors

Most common ovarian tumor ( in both benign and malignant category)

60% are benign and will be bilateral in 10% of cases. Peak incidence between 30-40 yrs

25% are malignant, occur in 45-65 yr olds

Morphologically

Most are unilocular and spherical to ovoid in shape

Maximum size of 40cm in diameter

Contain thin serous fluid

Benign tumors are smooth and glistening

Malignant tumors have nodular irregularities

Histologically they resemble tubal epithelium

Cysts are lined by tall columnar epithelium that may be ciliated

Psammoma bodies present commonly at the tips of the papillae

Prognosis

If the tumor appears confined to the ovary, frank carcinomas have a 5-year survival rate of about 70%,

whereas tumors of low malignant potential are associated with nearly 100% survival.

With cancers that have penetrated the capsule, the 10-year survival rate is less than 15%.

Mucinous tumors

80% are benign

10% are malignant

Occur in the same age group as serous tumors

Morphologically They tend to be larger (Up to 25kg) and more often multilocular than serous

counterparts

Content of cysts is mucinous in nature (thick and gelatinous)

Tend to be unilateral ( Helpful in distinguishing from Krukenberg tumor)

They have intralocular papilliferous prejjections and a warty appearance

Associated with Pseudomyxoma peritonei

Histologically they resemble cervical epithelium

The prognosis of mucinous cystadenocarcinoma is somewhat better than with its serous counterpart, although stage rather than histologic type is the major determinant of outcome.

In terms of 5 year survival

Borderline tumors have a 95% survival rate

non-invasive i.e. encapsulated malignancies have a 90% survival rate

invasive malignancies have a 66% survival rate

Endometrioid tumors

15% may develop in association with endometriosis

Up to 30% of women with this type of tumor have endometrial carcinoma

Similar to other endometrial type lesions they have mutations in PTEN tumor suppressor gene

>95% are malignant and 40% of malignant tumors occur bilaterally

Histologically they resemble endometrial cells

They contain tubular glands

These tumors are also estrogen dependent

Brenner tumors

Uncommon

Benign adenofibromas

Are derived from Wolffian duct epithelium

Histologically they resemble the bladder and are composed of nests of transitional cells

Sex cord tumors

Granulosa cell tumors

account for about 5% ovarian tumors

66% occur in post menopausal women

33% in young girls

All are potentially malignant

Hormonally active tumors are more likely to be picked up and diagnosed earlier

May produce large quantities of estrogen

In the young girl may cause

Precocious puberty

Menstrual irregularity

Benign cystic breast disease

In the older woman

Post menopausal bleeding

Endometrial hyperplasia

Endometrial carcinoma

Thecoma/Fibroma

account for about 4% ovarian tumors

very occasionally these tumors are malignant (fibrosarcomas)

Composed of fibroblast +/ theca cells

Hormonally inactive

Meig’s syndrome = Thecoma/Fibroma + ascites + hydrothorax

Sertoli Leydig cell tumors

Peak age 10 – 30 years of age

Hormonally active – secretes androgens

In young girls

Blocks sexual development

In women

Hirsutism

Acne

Infertility

Breast atrophy

Vaginal atrophy

Weight loss

Irregular menstration/ amenorrheoa

Vocal changes

Clitoral enlargement

Germ cell tumors

Accounts for 20% of ovarian tumors

Dysgerminoma

Occurs in the 10-20 age group

Accounts for 2% of ovarian tumors and 100% are malignant

Unilateral in 90% of cases

Associated with gonadal dysgenesis

Rarely may cause elevated hCG levels

Tumors are radiosensitive

Spread through lyphatics to para-aortic, mediastinal and supraclavicular nodes

Prognosis

96% survival if the tumor has not breached the capsule

80% survival if the tumor has spread

NB. Only 1/3 of tumors are clinically aggressive

Choriocarcinoma

Peak incidence 0-30 yrs of age

Unilateral

Of placental origin

Rarely may occur de novo in a pre-pubertal female = Poor prognosis and unresponsive to chemotherapy

Covered in presentation on Gestational Trophoblastic Disease

Yolk Sac tumors

Rare

Malignant

Rapidly expanding

Teratoma

Mature/ Benign/ Dermoid Cyst

Bilateral in 10%, recur in 10%, maximum size of 10cm

Unilocular

Usually cystic

Contains sebaceous material

hair

teeth

other mature tissue

Immature/Malignant teratoma

Rare

Mean age of detection is 18 yrs old

Morphologically

Bulky and solid on cut section

Punctated by areas of necrosis

May contain cystic foci with characteristic of mature teratomas

Specialized teratoma

Rare subtype

Composed entirely of specialized tissue eg.

Struma Ovarii composed of thyroid tissue. May cause hyperthyroidism

Carcinoid tumors. These secrete 5 HT and may cause carcinoid syndrome

Presentation

Asymptomatic

Abdominal pain

Related to acute event such as torsion, rupture or bleeding

Related to nerve compression with pain radiating to medial thigh

Mass

Compressive symptoms

Urinary – frequency, hesitancy, acute/chronic retention

Gastrointestinal – constipation, dyspepsia, vomiting

Hormonal effect

Investigation CBC

U+E

RFT

LFT

Abdominopelvic ultrasound

Plain XRay

CT scan

CXR

Barium enema

IVU

Tumor markers – CA 199, CA 125, MCSF, Tumor associated glycoprotein-72

Hormone levels

Laparoscopy – 30% of cases where disease is thought to be confined to the abdomen there is involvement of abdominal content

Treatment

The mainstay of treatment of ovarian malignancy is surgery. The management of the patient however will be guided by staging.

Early tumors ie. Stage Ia and Ib may be trated with surgery only. Surgery only gives a 5 year survival rate in excess of 90%

Surgery may be

Conservative – unilateral salpingo-oophorectomy +/- bisection of contralateral ovary

Radical – total abdominal hysterectomy (avoided in younger women and those with continued fertility desires)

Aims of surgery are:

i. to remove all of the malignant tissue, and if this is not possible, then to ensure that residual masses are less than 1.6 cm in diameter. This facilitates the efficacy of chemotherapy.

ii. to perform omentectomy since the infra-colic omentum is a common site for micro-and macro- metastases.

iii. to perform a pan-hysterectomy.

iv. to remove pelvic and para-aortic nodes if involved.

v. to obtain peritoneal washings or ascitic fluid for cytology.

In stage Ic to IIIc disease surgery only is not curative. In these cases debulking surgery is followed by chemotherapy

The regimen featured in Box 20.4 is used in epithelial tumors

Dysgerminomas are treated with Vincristine, Actinomycin D and cyclophosphamide

Teratocarcinomas, Yolk sac tumors and Choriocarcinomas are treated with Cisplatin, Vinblastine and Bleomycin

Radiotherapy is useful in stage II and III disease

Particularly so for Dysgerminomas.

Effective for metastases and recurrence

Administered to abdomen and pelvis

Conclusion

Ovarian tumors are diverse and best classified by their cell of origin

Tumors may be solid or cystic

Tumors that elucidate hormones are detected earlier

Staging is more important as a prognostic indicator than histology

Early tumors may be managed with surgery only

Chemotherapy and radiation are important adjuncts in the management of ovarian tumors

Thank You