ovarian cancer presentation

1. CARCINOMA OVARY:
ETIOPATHOGENESIS, STAGING,
EVALUTION
Dr Parth Patel

2.  Each ovary is 2.5–5.0 cm long, 1.5–3.0 cm thick,
and 0.7–1.5 cm wide and is attached by its
mesenteric attachment to the broad ligament, the
mesovarium.
 The infundibular pelvic ligament, which includes the
ovarian artery and vein is present laterally while the
ovarian ligament is present medially.
 The blood vessels enter the hilus of the ovary.

3.  The germinal epithelium lines the outside, or the
capsule of the ovary.
 The inner medulla of the ovary is a fibromuscular
layer with blood vessels.
 The cortex, including the stroma, consists of
follicles, corpus luteum, and corpus albicans.

5. Blood Supply:
 Arterial - via the ovarian arteries, which have their
origin in the anterior surface of the aorta, inferior to
the renal vessels.
 Left ovarian vein empties into the left renal vein,
which then enters into the vena cava, and the right
ovarian vein empties into the vena cava inferior to
the right renal vein.

6.  The lymphatic drainage of the ovary follows its
blood supply through the infundibulopelvic ligament
to nodes in the para-aortic region.
 Lymphatic drainage through the broad ligament and
parametrial channels can also result in involvement
of pelvic sidewall lymphatics, including the external
iliac, obturator, and hypogastric chains.
 Spread may rarely occur along the course of the
round ligament, resulting in involvement of inguinal
lymph nodes.

7. INTRODUCTION
 Ovarian neoplasms consist of several histopathologic
entities; treatment depends on the specific tumor type.
 Epithelial ovarian cancer - majority of malignant ovarian
neoplasms (about 90%);
 less common histopathologies, including
- ovarian low malignant potential [LMP] tumor,
- malignant germ cell neoplasms,
- carcinosarcomas (malignant mixed Müllerian
tumors [MMMTs] and
- malignant sex cord- stromal tumors.

8.  EOC typically occurs in postmenopausal women & mostly
present in advanced stage.
 Most germ cell tumors present in younger women, and
 Sex cord–stromal tumors may occur at any age.
 other types of ovarian cancer such as germ cell and sex cord–
stromal tumors are
 often localized in distribution,
 more amenable to surgical resection, and have a
 more favorable prognosis.

9. EPIDEMIOLOGY
 Both in the United States and in Europe, ovarian cancer is the fifth
most common cause of cancer death.
 African American women in the United States have a lower
incidence of ovarian cancer (10.3 per 100,000 women)
compared to white women,
 The lifetime risk of developing sporadic epithelial ovarian
cancer is approximately 1.7%, although pts with a familial
predisposition have a much higher lifetime risk, in the range
of 10% to 40%.
 The median age at diagnosis for sporadic disease is 60 years,
although pts with a genetic predisposition may develop this
tumor earlier, often in their fifth decade.

10. RISK FACTORS
 Established risk factors for ovarian cancer include a
 strong family history,
 early menarche,
 late menopause,
 increasing age, and
 nulliparity.

11. REPRODUCTIVE FACTORS: PREGNANCY AND
BREAST FEEDING
 Early menarche and late menopause increase the risk of
ovarian cancer.
 Increased parity, breastfeeding, and the use of OCP
reduce the risk.
 Risk for ovarian cancer is linked to the number of
ovulations that a woman experiences throughout her
lifetime.
 Breast-feeding probably reduces ovarian cancer
incidence through several mechanisms, including
 suppression of ovulation,
 reduced serum concentrations of estradiol and LH, and
 elevated FSH levels.

12. ORAL CONTRACEPTIVES
 Women who use OCP for at least 5 years reduce their risk of
ovarian cancer by an average of 50%, and the level of
protection increases with duration of use.
 Protection against ovarian cancer is probably the most
important non-contraceptive benefit of OCPs.

13. HORMONE REPLACEMENT THERAPY
 a review of the major studies suggests a 30% to 58%
increase in ovarian cancer risk in women who take HRT.
 The risk of ovarian cancer was found to increase with duration
of HRT exposure, and the largest risk was seen among
women who used HRT for over 20 years.
 Most studies show that a treatment duration of less than 5
years carries no statistically increased ovarian cancer risk.

14. SURGERY: TUBAL INTERRUPTION AND
HYSTERECTOMY
 In general, studies have confirmed that both tubal interruption
and hysterectomy at least partially protect against the
development of ovarian cancer.
 Possible explanations for the protective effect of tubal
interruption against ovarian cancer include
- an impaired blood supply to the ovaries/distal tubes through
the superior branch of the uterine artery leading in most
women to earlier menopause (and fewer lifetime ovulations)
- the possibility that occluding the tube blocks the upward flow
of carcinogens from the uterus and reduces pelvic infection
rates.

15. SMOKING,OBESITY
 Smoking is not generally considered a risk factor for
ovarian cancer.
 However, current smoking seems to be a/w an
increased risk of developing a MUCINOUS ovarian
cancer.
 Smoking cessation reduces this risk back to
baseline over 20 years.
 Obesity does not appear to be a/w the most
aggressive types of ovarian cancer

16. PATHOGENESIS AND PATTERNS OF METASTASES
 EOC are thought to arise from the surface epithelium covering the
ovary, which is contiguous with peritoneal mesothelium.
 However, emerging evidence suggests that a subset of epithelial
ovarian cancers may instead originate in the fallopian tube fimbria,
subsequently spreading to the ovary or peritoneal cavity.
 Germ cell tumors most likely originate in cells derived from the
primitive streak that ultimately migrated to the gonads.
 The ovarian stroma consists of granulosa cells, theca cells, and
fibroblasts, which give rise to the sex cord–stromal tumors.

17.  Mutation in the p53 protooncogene occurs in over 50% of
cases, predominantly involving tumors in pts with advanced
stage and high-grade serous histology.
 In contrast, mutations in B-raf, K-ras, PTEN, or β-catenin may
be seen in endometrioid, mucinous, or low-grade
histologies
.
 Amplification of the HER2/neu gene is observed in only
approximately 8% of patients and confers a poorer
prognosis.

18.  Overexpression of pro-apoptotic genes such as BAX is a/w
chemoresponsiveness and a more favorable prognosis.
 Surface adhesion proteins such as CD44H and β-1 integrins
have been shown to mediate transperitoneal spread of this
tumor by promoting the attachment of cancer cells to the
peritoneal mesothelial lining.

19.  The tumor typically spreads to the omentum and to peritoneal
surfaces such as the underside of the diaphragm, paracolic
gutters, and bowel serosa.
 Approx 10% to 15% of pts with ovarian cancer that appears to
be localized to ovaries have metastases to para-aortic LN,
and retroperitoneal LN involvement is found in over 50% of pts
with advanced disease.

20.  MC site of extra-abdominal spread - pleural space
(thought to occur via transdiaphragmatic lymphatics),
where it causes a malignant pleural effusion in some
patients.
 Hematogenous metastases to liver, spleen, or lung can
also occur during the course of disease, but are
relatively uncommon at presentation.
 Bone or CNS metastases may rarely be observed in pts
who have lived for many yrs after initial diagnosis,
during which unusual patterns of disease spread may
occur.

22. Histology Features
Papillary serous The most common type of EOC. May contain psammoma
bodies and is often a/w CA 125 elevation.
Endometrioid Sometimes a/w endometriosis or an independent uterine
cancer of similar histology.
May occur with early stage disease in younger pts, although
advanced disease is also possible.
Mucinous May rarely be a/w pseudomyxoma peritoneii.
CA 125 levels may not be markedly elevated.
Relatively chemoresistant.
D/D of a mucinous ovarian tumor includes metastatic disease
from an appendiceal primary.
Clear cell The most chemoresistant type of ovarian cancer. Sometimes
a/w endometriosis or humorally mediated hypercalcemia.
Table 104.2. Common Histologic Types of Epithelial Ovarian Cancer

23. CLINICAL PRESENTATION AND DIAGNOSTIC
WORKUP
Clinical Presentation of Pts with a Benign Adnexal
Mass or Early-Stage Ovarian Cancer
 Almost all pts with small adnexal tumors are
asymptomatic, and the mass is usually discovered
incidentally during a work-up for other conditions.
 With increasing size, adnexal masses cause pelvic
pressure and pain by compressing surrounding
structures.
 A larger pelvic tumor can cause g.u. symptoms including
urgency, frequency, and dyspareunia.

24.  Posteriorly, a fixed pelvic tumor compresses the
sigmoid colon, causing severe constipation.
 it is impossible to differentiate a benign from a
malignant ovarian mass by clinical examination
alone.
 An ovarian mass becomes a surgical emergency if
a pt with an adnexal mass has sudden onset of
abdominal pain. The differential includes torsion
and possible infarction of the ovarian mass.

25.  Vaginal bleeding in a pt with an adnexal mass could be an
indication of
 synchronous endometrial and ovarian cancers or
 a granulosa cell tumor that produces estrogen, causing
abnormal bleeding.
 Sertoli-Leydig cell tumors can lead to virilization.

26. CLINICAL PRESENTATION OF PATIENTS WITH
ADVANCED OVARIAN CANCER
 General, nonspecific symptoms a/w advanced ovarian
cancer include anorexia, fatigue, early satiety and loss
of appetite.
 Bloating and diffuse, dull, constant abdominal pain
caused by the infiltration of the peritoneum and the
bowel mesentery, or by extensive ascites.
 While weight loss is unusual, tumor cachexia can be a
presenting sign in pts with high-volume disease and
long standing partial bowel obstruction.

27.  If the tumor has metastasized to the omentum,
patients complain of upper abdominal discomfort
with nausea, belching, early satiety and fullness.
 In addition, ascites can cause dyspnea.
 Signs of bowel obstruction, severe urinary
symptoms, intense pelvic pain, and ascites are
likely to indicate miliary dissemination on the
peritoneal surfaces and large-volume advanced
disease.

29. CLINICAL EXMINATION
 A focused clinical exam should begin with an
assessment of SUPRACLAVICULAR LNS, a
breast exam, and percussion of the lungs to detect
a pleural effusion.
 The involvement of the umbilicus by an ovarian
cancer is called “Sister Mary Joseph nodule”
 Examine for the presence of ascites, the size and
mobility of the adnexal mass, and the presence of
enlarged inguinal LNs.

30.  The bimanual and rectovaginal exam should
attempt to evaluate and characterize the adnexal
mass in respect to size, borders (smooth/irregular),
mobility (fixed/mobile), and location.
 Invasive ovarian cancers often have irregular
borders and are often fixed to the pelvic sidewall
and fill the cul-the-sac.

31. LABORATORY TESTS
 The laboratory work-up for patients with suspected
ovarian cancer generally includes
Complete blood count.
LFTs
RFTs

32.  The CA 125 serum level is elevated in > 80% of serous
epithelial ovarian cancers.
 Also elevated in a variety of benign gynecologic
conditions (such as endometriosis, PID, or pregnancy
and nongynecologic malignancies (such as breast, lung
and g.i. cancers).
 Furthermore, the CA 125 level is elevated in only approx
50% of pts with early stage epithelial ovarian cancer
which also limits its value as a screening test.

34.  Other tumor markers, such as CA 19-9, which is
elevated in some mucinous ovarian carcinomas,
and carcinoembryonic antigen (CEA) are less
frequently useful.
 Postoperatively, the CA 125 level provides a
sensitive way to monitor treatment response and
development of disease recurrence.

35.  It is best to avoid percutaneous biopsy during the
initial evaluation, which can result in cyst rupture
and spillage of malignant cells into the peritoneal
cavity.

36. IMAGING OF ADNEXAL MASSES, EARLY AND
ADVANCED OVARIAN CANCER
Ultrasound
 First modality to diagnose or confirm the
clinically suspected adnexal mass.
 Ultrasound can detect and characterize ovarian
size and morphology.
 TVS is better than trans abdominal approach for
assessment of morphological features.

37.  Benign tumors often appear on ultrasound as
 unilateral with smooth walls,
 with a few cysts,
 no solid elements and
 an absence of ascites.
 Functional cysts have thin walls and are fluid filled
 Dermoids are often cystic with hyper-echoic areas
(teeth).

38.  TVU is more sensitive at detecting ovarian tumors
compared to other tests such as CT, and it can
provide qualitative information about the mass that
might suggest malignancy.
 The classic sonographic finding of malignancy is a
“complex” cyst,
-containing both solid and cystic components,
-with septations and
-internal echogenicity,
-irregular wall,
-mural nodules.

39.  Color Doppler imaging evaluates blood flow to an
ovarian mass and can potentially identify a
malignant process based on the presence of
abnormal neovascularization & low resistance flow
pattern.
 Positive predictive value of 98% has been reported
for transvaginal colour doppler.
 CXRs may sometimes be performed to evaluate the
presence of pleural effusions, which occur in 10%
of pts with epithelial ovarian cancer at diagnosis.

40. COMPUTED TOMOGRAPHY/CT
 CT features of ovarian malignancy
- a multilocular cyst with thick internal septations,
- solid mural nodules,
- complex solid-cystic mass or
- lobulated papillary mass.
 Solid components enhanced well with iv contrast.
 In the pelvis, a CT scan allows for characterization of the adnexal mass and
any involvement of surrounding organs (bladder, sigmoid, ureter, pelvic
sidewall involvement).
 In the upper abdomen retroperitoneal adenopathy, omental involvement, and
intrahepatic liver involvement can be detected reliably by CT scans with i.v.
contrast.

42. MAGNETIC RESONANCE IMAGING—MRI
 Morphological features of ovarian cancer on MRI
are similar to CECT , but because of excellent soft
tissue contrast, the details are better demonstrated.
 Contrast enhancement helps to differentiate solid &
necrotic areas.
 MRI is better than other modalities in determining
the origin of pelvic mass.
 Also accurate in differentiate b/w benign and
malignant mass.

43.  MRI features favouring malignant mass
- size > 4 cm,
- large solid component
- wall or septal thickness> 3mm
- nodularity or vegetations
- necrosis.
And ancillary features.
 In recurrent cancer, MRI is especially useful for
differentiating between postsurgical changes and a
recurrence on the vaginal dome, small bowel mesentery,
splenic hilum, liver surface, or diaphragm.

44. SCREENING AND EARLY DETECTION
 A successful screening test for ovarian cancer should be capable
of identifying the majority of pts with precancerous lesions or
early disease.
 Because a positive screening test for ovarian cancer would result
in major surgery with associated morbidity, costs, and even
mortality.
-the false-positive rate must be low, and
-PPV relatively high (at least 10%).
 At present, there are no screening tests for epithelial ovarian
cancer that convincingly meet these criteria.
 The ability of tumor cells to exfoliate from the ovarian surface and
to spread in an asymptomatic fashion impedes the development
of successful screening approaches that would allow for early
diagnosis.

45.  The CA 125 serum level is not a useful screening test when
used alone, since elevations are not specific for ovarian
cancer.
 Elevation may be observed in cirrhosis, peritonitis, pleuritis,
pancreatitis, endometriosis, uterine leiomyoma, benign
ovarian cysts, and pelvic inflammatory disease.
 Elevated in other malignancies such as breast, lung,
colorectal, pancreatic, and gastric cancers.

46.  a number of candidate markers have been discovered
that show promise for enhancing the accuracy of CA
125 levels, such as
- HE4 (human epididymis 4),
- Osteopontin,
- Mesothelin, and
- Osteoblast-stimulating factor-2.
 Levels of OVX-1 and macrophage colony-stimulating
factor have been found to be elevated in pts with
clinically evident ovarian cancer but normal CA 125
levels, which suggests that these markers may be
complementary to CA 125.
 None of these tests has been proven to have sufficient
sensitivity and specificity for routine screening at the
current time.

47.  Measurement of the CA 125 level has been
combined with performance of TVU in an attempt to
improve screening.
 Early studies of TVU suggested a sensitivity of
close to 100% but a specificity of 98%, which is
insufficient to achieve a PPV of 10%.

48. SCREENING TRIALS
 In the US, the Prostate, Lung, Colorectal, Ovarian
Cancer Screening Trial uses measurement of CA
125 level (single threshold elevation of >35 U/mL)
and TVU together, performed annually, as a first-
line screen. If either test is positive, the woman is
referred for surgical consultation.
 no mortality reduction was observed for pts
randomized to the screening arm.

49.  An ongoing trial is assessing screening for ovarian
cancer (UK Collaborative Trial of Ovarian Cancer
Screening [UKCTOCS]) using multimodality
screening with ultrasound and CA-125 versus either
ultrasound alone or no screening.
 Preliminary results suggest that multimodality
screening is more effective at detecting early-stage
cancer.

50.  An earlier study by Jacobs et al. in which 21,935
average-risk postmenopausal women were
assigned to undergo three annual screenings or no
screening.
 The screening protocol used CA 125 level as a first-
line screen and referred the patient for TVU if the
CA 125 level was >30 U/ml. If the TVU revealed an
ovarian mass, the patient was referred for surgical
consultation.
 Statistically significant improvement in survival.
average of 72.9 months in the screening group and
41.8 months in the control group.

51.  The OVA1 test uses 5 markers (including
transthyretin, apolipoprotein A1, transferrin, beta-2
microglobulin, and CA-125).
 The Society of Gynecologic Oncology (SGO), the
FDA, and the Mayo Clinic have stated that the
OVA1 test should not be used as a screening tool
to detect ovarian cancer.

54. HEREDITARY OVARIAN CARCINOMA
 Approx 5% to 10% of pts with EOC carry a germline mutation
that places them at substantially increased risk of developing
this disease.
 The BOCS accounts for approx 90% of hereditary ovarian
cancer and is often suspected whenever
- the pedigree reveals multiple affected family members
with ovarian cancer,
- bilateral or early onset breast cancer,
- both breast and ovarian cancer in the same individual,
or
- a male relative with breast cancer.

55.  The high incidence of breast and ovarian cancers in these
families is d/t inherited germline mutations in
the BRCA1 or BRCA2 genes.
 The BRCA1 gene, located on chr band 17q12-21, and
the BRCA2 gene, located on chr band 13q12-13, were
identified and linked to hereditary breast and ovarian cancers
in the 1990s.
 act as tumor suppressors and play a critical role in the repair
of double-stranded DNA breaks.

56.  The lifetime risk of ovarian cancer is approx
20% to 40% for BRCA1 mutations, &
10% to 20% for BRCA2 mutation carriers.
 The majority of BRCA1-associated cancers are serous
adenocarcinomas, with an average age at diagnosis of 48
years, whereas the mean age for BRCA2-associated ovarian
cancers is 60 years.
 BRCA-associated cancers may have a more favorable course
than sporadic ovarian cancer
- improved response to platinum-based chemotherapy
compared to women with sporadic disease

57.  The HNPCC syndrome accounts for approx 5% to 10% of all
hereditary ovarian cancer cases.
 It is an autosomal dominant genetic syndrome characterized by
- nonpolyposis colon cancer,
- endometrial,
- ovarian,
- hepatobiliary,
- upper gastrointestinal, and
- genitourinary cancers.
 The risk of endometrial cancer 40% to 60% by age 70, compared
to 1.5% in the general population.
 3.5-fold increase in the risk of ovarian cancer in members of
these families.

58.  A germline mutation in one of five genes involved in
DNA mismatch repair is responsible for the HNPCC
phenotype:
 hMSH2 (chr arm 2p),
 hMLH1 (chr arm 3p),
 hPMS1 (chr arm 2q),
 hPMS2 (chr arm 7p), and
 hMSH6 (chr arm 2p).
 The majority of affected patients are found to have
defects in either hMSH2 or hMLH1.
 The estimated cumulative risks for ovarian cancer by
age 70 years can be as high as 24% for those with
germline mutations in MLH1 and MSH2.

59.  It has been suggested that chemoprophylaxis with oral
contraceptives for 5 yrs might decrease ovarian cancer risk by
50% in both the general population and in high-risk women.
 For example, a case-controlled study of 207
known BRCA mutation carriers and their sister controls found
a 60% reduction of ovarian cancer risk with oral contraceptive
use
 Other risk-reducing strategies such as tubal ligation and
hysterectomy have also been a/w a reduced incidence of
ovarian cancer among high-risk women.

60.  Nonetheless, RRSO is currently the most effective
preventative strategy to reduce ovarian cancer risk
in pts with BRCA mutations.
 One study of 259 pts who underwent RRSO found
a 96% decrease in ovarian cancers and an
approximately 50% reduction in subsequent breast
cancer compared to age-matched controls.
 However, there is a residual risk for primary
peritoneal cancer in these high-risk women after
prophylactic salpingo-oophorectomy.

61. STAGING
 Exploratory laparotomy serves three main purposes in the
management of patients with suspected ovarian cancer.
- laparotomy permits histologic confirmation of disease
- the extent of disease (staging), which is critical in determining
whether postoperative treatment will be necessary, as well as to
assess prognosis.
- to permit debulking of as much tumor as possible, since pts who
are optimally cytoreduced (defined as having less than or equal
to 1-cm diameter residual tumor) have a better prognosis
compared to those with greater amounts of residual disease.
 The staging system for ovarian cancer is defined by FIGO
(updated in 2014) and is based on the findings at exploratory
laparotomy .

62.  Thank you

64. MIMICERS
 Gastric, breast (esp ILC), mesothelioma,and colorectal cancers may
present with diffuse peritoneal implants, ascites, and ovarian metastases
 Distinguish by routine light microscopic histologic evaluation,IHC.
 Cytokeratin CK7 is positive and CK20 is negative- primary serous
ovarian cancer, whereas reverse is seen for CRC.
 Gross cystic disease fluid protein (GCDFP) may be positive in up to 50%
of patients with breast cancer.
 GCDFP negative in gastric,colorectal, or ovarian cancer.
 Calretinin – mesothelioma, typically negative in epithelial ovarian
cancer.

65. DIAGNOSIS
 CA 125 elevated in >80% of serous epithelial ovarian cancers.
 Also elevated in a variety of benign conditions (cirrhosis, peritonitis, pleuritis,
pancreatitis, endometriosis, benign ovarian cysts, endometriosis, PID, or pregnancy)
and non gynecologic malignancies (such as breast, lung, and GI cancers).
 CA 125 elevated approx 50% of patients with early stage epithelial ovarian
cancer,[limiting its use as a screening test].
 Other markers, CA 19-9, elevated in some mucinous ovarian carcinomas, and CEA
are less frequently useful.
 CA 125:CEA ratio of ≥25
 Postoperatively, the CA 125 - sensitive way to monitor treatment response and
development of recurrence.
 ROMA - CA 125 +HE4 tests.

66.  TVU is more sensitive at detecting compared to other tests such as
CT, and it can provide qualitative information about the mass that
might suggest malignancy.
 The classic sonographic finding of malignancy is a “complex” cyst,
defined as containing both solid and cystic components, sometimes
with septations and internal echogenicity .
 complex cyst on sonography, in the presence of signs and symptoms
consistent with ovarian cancer, often requires surgery for further
evaluation.
 It is best to avoid percutaneous biopsy -can result in cyst rupture and
spillage of malignant cells into the peritoneal cavity.
 Color Doppler - based on the presence of abnormal
neovascularization.

67. SIMPLE CYSTS
 “thin-walled, fluid-filled, without a mass component, septations,or internal
echogenicity “ on TVU .
 most often benign
 may be found in 5% to 10% of asymptomatic postmenopausal women
during TVU examination, esp in the first decade after menopause.
 Not always require surgical evaluation if associated with normal CA 125
levels.
 Postmenopausal women with simple cysts with elevated CA 125 levels,
simple cysts >5 to 10 cm in diameter, or simple cysts with abnormal
color Doppler flow studies are often referred for surgery.
 Premenopausal women with simple cysts that are persistent or
enlarging, with rising CA 125 level-exclude malignancy.

68.  CT or MR imaging -defining the extent of peritoneal
disease. However, for the patient with a complex ovarian
cyst and clinical signs and symptoms to suggest ovarian
cancer, these studies generally do not obviate the need
for surgical exploration.
 Occasionally, CT may be helpful in distinguishing a
gynecologic malignancy from a metastatic pancreatic
neoplasm, for instance, for which an exploratory
laparotomy may not be warranted.
 CT may also assist in surgical planning by locating the
site of suspected bowel obstruction.

69. HEREDITARY OVARIAN CARCINOMA
 Approximately 5% to 10% of patients with EOC
carry a germline mutation.
 BOCS accounts for approx 90% of hereditary
ovarian cancer.
 suspected whenever the pedigree reveals:
 multiple affected family members with ovarian
cancer,
 bilateral or early onset breast cancer,
 both breast and ovarian cancer in the same
individual,
 a male relative with breast cancer

70.  BRCA1 gene, chr 17q12-21, 20% to 40% lifetime risk serous
adenocarcinomas , avg age at diagnosis of 48 yr.
 BRCA2 gene, chr 13q12-13, 10% to 20% for BRCA2 mutation
carriers,60 yrs.
 BRCA-associated cancers may have a more favorable course
than sporadic ovarian cancer.
Management:
 Risk reduction bilateral salpingo-oophorectomy (RRSO) Even
after RRSO, BRCA mutation carriers are still at small risk for
developing PPSC.
 Considered for patients at high risk for developing ovarian
cancer and completed childbearing, especially if they are at
least 35 years .
 RRSO is currently the most effective preventative strategy to
reduce ovarian cancer risk in patients with BRCA mutations.

71.  HNPCC syndrome - 5% to 10% of all hereditary ovarian
cancer cases. AD.
 c/b nonpolyposis colon cancer, (often right colon), with
increased risk of developing endometrial, ovarian,
hepatobiliary, upper gi & gu cancers.
 Colorectal and uterine cancers comprise the majority of
tumors .
 3.5-fold increase in the risk of ovarian cancer.
 HNPCC account for approx 7% of cases with
synchronous uterine and ovarian cancers, which are
often low grade and of endometrioid histology.

72. PROGNOSTIC FACTORS FOR EPITHELIAL
OVARIAN CANCER
 STAGE, stage IA disease and grade 1 or 2 histology 5 yr survival
is >90% after surgery alone, some include stage IB, grade 1 or 2
disease, in this good prognostic group.
 the relapse rate without adjuvant treatment is 30% to 40% for
stage IC , those with stage I, grade 3 disease, or with stage II.
 These patients comprise a high-risk group of early stage tumors
and experience a 5-year survival rate of approx 80% after
adjuvant therapy.
 VOLUME OF RESIDUAL DISEASE AFTER
CYTOREDUCTIVE SURGERY the 5-year survival
rate stage III optimally debulked, gross residual disease (≤1-cm
diameter residual tumor) is approximately 20% to 30%, and
<10% for patients with suboptimally debulked stage III disease or
those with stage IV tumor.

73.  Histology- mucinous or clear cell histologic have a worse
prognosis, which appears to be related to the relative
chemoresistance.
 Preoperative serum CA 125 levels frequently reflect the
volume of disease.
 Normalization of CA 125 after 3 cycles of chemotherapy is
associated with more favorable outcome.
 Achievement of a CA 125 nadir of ≤10 U/mL upon completion
of treatment-Favorable
 BRCA1 or BRCA2 genes mutation is associated with an
improved prognosis.

74.  optimal cytoreduction residual disease of ≤1 cm in
maximum individual tumor diameter.
 optimal cytoreduction no gross residual disease,
reflecting complete cytoreduction to microscopic
residual status.

76. FACTORS THAT ARE ASSOCIATED WITH SUBOPTIMAL CYTOREDUCTION
 Clinical –
 age > 60 years,
 CA-125 > 500 U/mL, and
 American Society of Anesthesiologists (ASA) Physical Status
classification of 3 or 4
 Radiological -
 Retroperitoneal LN above the renal hilum (including
supradiaphragmatic) > 1 cm,
 diffuse small bowel adhesions or thickening,
 small bowel mesentery lesions>1 cm,
 root of the superior mesenteric artery lesions>1 cm,
 perisplenic lesions>1 cm,
 lesser sac lesions > 1 cm.
 ascites on atleast two-third of CT scan slices