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The classification of ovarian tumors
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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