Ovarian and Fallopian Tube Pathology 5

Question 1

What are the major considerations in the approach to the patient with an adnexal mass?

Answer 1

• Age of the patient
• Size and characteristics of the mass on both exam and imaging
• Presentation and associated symptoms

Question 2

What are the most common adnexal masses in the following age ranges? 0-13, 13-51, postmenopausal?

Answer 2

• Premenarchal (ages 0-13)
  
  • cancer
• Reproductive age (ages 13-51)
  
  • functional ovarian cysts
• Benign neoplasms
  
  • postmenopausal
  • cancer

Question 3

What are important characteristics of adnexal masses and how are they described?

Answer 3

• Imaging:
  
  • simple: fluid filled, thin wall
  • complex: cystic and solid, septae, surface excrescences, blood flow within the mass
• Exam:
  
  • mobile versus fixed; smooth surfaced versus nodular
• Size:
  
  • <5 cm (generally observe)
  • 5-10 cm (usually remove)
  • > 10 cm (always remove)

Question 4

How do adnexal masses commonly present in terms of history, exam, imaging, lab tests?

Answer 4

• History: how is the patient presenting?
  
  • in acute pain in the ER
  • new intermittent pelvic pain
  • changes in bowel or bladder
  • asymptomatic
• Exam: abdominal and pelvic
  
  • abdominal exam
  • pelvic exam
    
    • speculum exam
    • bimanual exam
    • rectovaginal exam
• Imaging
  
  • Pelvic ultrasound
  • CT or MRI
• Lab tests
  
  • CBC
  • chemistry panel
  • urine pregnancy
• Tumor markers:
  
  • ovarian cancer: CA-125, AFP, inhibin A &B, LDH, hCG
  • other cancers: CA 19-9, CEA, CA 15-3 or CA 27.29

Question 5

What are the common types of functional cysts of the ovary?

Answer 5

• Functional: related to ovulation
• The most common clinically detectable ovarian enlargements during the reproductive years
• Usually resolve spontaneously within 8-12 weeks
• Usually < 10 cm in size
• Examples:
  
  • Follicular
  • Theca-lutein
  • Corpus luteum
  • Polycystic ovaries

Question 6

What are some distinguishing factors of follicular cysts?

Answer 6

• Follicle fails to rupture and the follicle continues to grow and fill with fluid
• Smooth, thin-walled, unilocular

Question 7

What are distinguishing features of corpus luteum cysts?

Answer 7

• Corpus luteum fails to involute and continues to enlarge after ovulation
• Grossly yellow in color

Question 8

What are distinguishing features of theca lutein cysts?

Answer 8

• Luteinized follicular cysts (= lipid accumulation)
• Results when a follicle is overstimulated by hCG hormone
• Usually bilateral
• Most commonly seen in pregnancy states
• Most asymptomatic

Question 9

What are distinguishing features of PCOS?

Answer 9

•Polycystic ovarian syndrome (PCOS)
•Multicystic ovaries on ultrasound
•Two defining characteristics: menstrual dysfunction and hyperandrogenism
•Can also see metabolic disorders such as dyslipidemia and insulin resistance
•
•Ultrasound criteria for establishing the diagnosis of PCOS are 10 or more cysts that are 2-8 mm in diameter and are peripherally arranged like a “string of pearls”
•
•Rarely do polycystic ovaries require surgical management but they may be a source of an enlarged ovary on pelvic exam

Question 10

What are the major types of benign ovarian neoplasms?

Answer 10

• Neoplasm = tumor; new and abnormal growth, may be benign or malignant
• May arise from any cell layer of the ovary
  
  • Surface epithelium
  • Germ cells
  • Sex cord-stromal tissue

Question 11

How are benign ovarian neoplasms treated and why?

Answer 11

• Usually managed surgically for the following reasons:
  
  • rarely resolve spontaneously
  • often appear complex or solid on imaging
  • more likely to be symptomatic

Question 12

What are the common epithelial neoplasms?

Answer 12

• Serous cystadenoma
• Mucinous cystadenoma
• Cystadenofibroma
• Endometrioma
• Brenner tumor

Question 13

What are epithelial neoplasms of the ovary?

Answer 13

• Serous and mucinous cystadenoma
  
  • two of the most common benign ovarian neoplasms
  • can grow to enormous (> 20cm) sizes
  • serous cystadenoma’s more often bilateral
• Endometrioma
  
  • ectopic growth of endometrial tissue on an ovary
  • typically contain thick brown fluid (old blood), described as “chocolate cysts”
  • often stick to surrounding pelvic surfaces and can cause symptoms typical of endometriosis
    
    • pelvic pain, dysmenorrhea, dyspareunia
• Most common benign cause of an elevated serum CA-125, an ovarian cancer tumor marker

Question 14

What are common sex cord-stromal neoplasms of the ovary?

Answer 14

• Derived from the sex cords and specialized stroma of the developing gonad, including cells that produce hormones
• Examples
  
  • Fibroma
  • Fibroadenoma
  • Fibrothecoma
  • Sertoli – leydig cell tumor

Question 15

What are some important features of fibromas of the ovary?

Answer 15

• Most commonly seen in postmenopausal women
• Average diameter 5-10 cm
• Most common benign solid tumor of the ovary
• Proliferation of ovarian fibrous/cortex tissue
• Meig’s syndrome:
  
  • constellation of ovarian fibroma, ascites and a (right) pleural effusion
  • seen in 1-3% of patients with fibromas

Question 16

What are some important features of germ cell neoplasms of the ovary?

Answer 16

• Derived from primordial germ cells of the ovary (undifferentiated cells)
• Mature cystic teratoma (aka “dermoid” cyst)
  
  • most common germ cell neoplasm
  • often bilateral (15-25%)
  • very frequent in younger patients (ages 10-30)
  • can contain mature components of ecto-, meso- and endoderm
  • teeth, hair, and sebum are the most common components

Question 17

What are some major features of borderline tumors of the ovary?

Answer 17

• Also called tumors of low malignant potential (LMP)
• Epithelial tumors (serous, endometrioid, mucinous)
• Tend to remain confined to the ovary for long periods of time but have the ability to metastasize (as a borderline tumor) and sometimes recur a low grade ovarian cancer
• Most common in pts between 30-50 yrs
• Frequently diagnosed at an early age and associated with an excellent prognosis

Question 18

What is the epidemiology of epithelial ovarian cancers?

Answer 18

• Most common type of ovarian cancer
• Most common in the 5th and 6th decades of life
• Typically presents in advanced stages (stages III and IV), no good screening test
• Histologic subtypes: serous, mucinous, endometrioid, clear cell

Question 19

What are the risk factors of epithelial ovarian cancer?

Answer 19

• Age
• Reproductive and endocrine factor - “Incessant ovulation” theory: epithelial damage & repair associated with ovulation
  
  • increased risk of cancer with increased # of ovulatory cycles
    
    • late menopause
    • early menarche
• Genetic predisposition
  
  • genetic mutations account for 10-15% of epithelial ovarian cancer
  • cumulative probability of developing ovarian CA by age 70:
    
    • between16-48% in Hereditary Breast & Ovarian CA Syndrome (usually associated with BRCA1 and BRCA2 mutations)
    • ~12% in patients with Lynch Syndrome

Question 20

How can epithelial ovarian cancer be prevented?

Answer 20

• Oral contraceptives
  
  • 36% risk decrease with ever-use
  • 8-10% decrease for every year of use
  • Recommend ≥ 5 years of use
  • Protection may last for up to 10 years
• Tubal ligation
  
  • studies show decreased risk (30-50%) of ovarian CA after tubal ligation
  • mechanism unclear
• Breastfeeding
  
  • decrease the # of ovulatory cycles
• Pregnancy
  
  • decrease the # of ovulatory cycles
• Surgical removal of the fallopian tubes and ovaries
  
  • mainly for women with a genetic risk of ovarian cancer

Question 21

What are some important serum or tumor markers of epithelial ovarian cancer?

Answer 21

• CA 125
  
  • is elevated in greater than 80% of advanced epithelial ovarian cancers
  • is elevated in 25-50% of stage I cancers
  • has poor specificity (NOT VERY ACCURATE), especially in premenopausal women; can be elevated in a number of benign conditions
  • NOT a screening test for the general population

Question 22

What are the symptoms of epithelial ovarian cancer?

Answer 22

• Historically called “silent killer” because symptoms were not thought to develop until disease was very advanced
• Recent studies disprove this; women with ovarian cancer do have symptoms as early as 3-6 months before disease is detected
• Important points when asking about symptoms:
  
  • persistent
  • change from normal
  • occur almost daily for > 2-3 weeks

Question 23

What are some important findings for epithelial ovarian cancer?

Answer 23

• Symptoms
  
  • abdominal or pelvic pain
  • abdominal distention or bloating
  • early satiety
  • urinary frequency
  • constipation or diarrhea
  • fatigue
• Signs
  
  • abdominal or pelvic mass
  • pleural effusion
  • ascites
• Evaluation
  
  • examination
  • imaging
  • ultrasound and/or CT scan
  • serum tumor markers

Question 24

Describe the FIGO staging for ovarian cancer.

Answer 24

• Stage I: growth limited to the ovaries
  
  • Stages IA – IC
• Stage II: growth involving one or both the ovaries with pelvic extension
  
  • Stage IIA – IIC
• Stage III: same as Stage II with peritoneal implants outside the true pelvis and/or (+) retroperitoneal nodes
  
  • Stage IIIA – IIIC
• Stage IV: growth involving one or both the ovaries with distant metastasis

Question 25

What is the general management strategy for epithelial ovarian cancer?

Answer 25

• Surgery
  
  • determine the extent of disease (stage)
  • remove as much tumor burden as possible
  • goal of < 1 cm residual tumor burden
  • alleviate symptoms
• Adjuvant chemotherapy
  
  • kill any residual small volume disease
• *In most cases patients need both surgery and chemotherapy to have a shot at a cure

Question 26

What are the surgical procedures for epithelial ovarian cancer?

Answer 26

• Surgical Staging (stages I and II)
  
  • pelvic washing
  • total hysterectomy, bilateral salpingo-oophorectomy
  • try and remove the mass intact
  • pelvic and para-aortic lymph node sampling
  • peritoneal and omental biopsies
• Surgical Debulking (stages III and IV)
  
  • remove ascites
  • total hysterectomy, BSO
  • remove the bulk of disease from the pelvis and abdomen, which sometimes necessitates:
  • omentectomy
  • peritoneal stripping
  • splenectomy
  • bowel resection

Question 27

What are the benefits of cytoreductive (debulking) surgery?

Answer 27

• Physiologic
  
  • remove ascites
  • improved GI function
• Tumor Perfusion
  
  • removal of bulky, poorly-vascularized tumor
• Cell Kinetics: improve conditions for chemotherapy to work
  
  • Gompterzian Growth - improve growth fraction (smaller tumors divide more quickly, more susceptible to being killed with chemo)
  • Goldie-Codman hypothesis – tumor cells develop resistance, reasons to use more than one drug

Question 28

What are options of chemotherapy of epithelial ovarian cancer?

Answer 28

• After staging or debulking surgery:
  
  • Carboplatin
  • Paclitaxel
  • given IV or IP, usually for 6-8 cycles
  • outpatient, usually very well tolerated and very effective
• Recurrent ovarian cancer
  
  • many “second line” chemotherapy drugs used to prolong life; cure is no longer a possibility
  • possible for women with recurrent ovarian cancer to live > 5 years on and off chemotherapy