The Ovary and Its Disorders.

Question 1

What are ovarian symptoms?

Answer 1

Ovarian masses are often silent and detected when large and cause distension, or incidentally on USS. Acute
presentation is associated with ‘accidents’.

Question 2

Define ovarian cyst.

Answer 2

fluid filled sac in the ovarian tissue

Question 3

Describe the aetiology of ovarian cysts.

Answer 3

• Endometrioma: cyst formed via endometrial tissue on the ovary
• Functional cysts (occur around ovulation)
• Follicular: unruptured Graffian follicle (failed rupture of dominant; failed degeneration of non- dominant)
• Luteal: following rupture, it reseals and distends with fluid
• Haemorrhagic: bleeding into a functional cyst

Question 4

How common are ovarian cysts? What are the RFs?

Answer 4

• 8% of pre-menopausal woman have one

RF: PCOS, Obesity, early menarche, 1^st trimester of pregnancy and endometriosis

Question 5

How do ovarian cysts present?

Answer 5

• Presentation: lower abdo pain, deep dyspareunia, pressure symptoms, abdominal swelling, asymptomatic
• Acute accident: severe right/ left iliac fossa pain accompanied by vomiting in torsion. Includes:
• Torsion
• Haemorrhage
• Rupture
• Infection

Question 6

How do we investigate ovarian cysts?

Answer 6

• Acute presentation: exclude pregnancy
• TVUSS 🡪 hyper/hypoechoic regions
• Bloods:
  
  • FBC
  • G&S
  • CA-125
  • bHCG, LDH, AFP 🡪 germ cell tumour
• Doppler ultrasonography
• Consider: MRI and laparoscopic investigation
• RMI should be calculated
• Ovarian torsion most likely to occur with mature cystic teratomas
• Rupture of endometriotic and dermoid cysts causes most severe symptoms

Question 7

How should we manage a simple cyst?

Answer 7

• <50mmm 🡪 do not require follow-up, should resolve in 3 menstrual cycles
• 50-70mm 🡪 yearly USS
• 70mm+ 🡪 consider MRI or surgery

Question 8

How do we manage a complex cyst?

Answer 8

• Complex cyst (multi-loculated):

Laparoscopy/laparotomy 🡪 discuss oophorectomy

Question 9

How should we manage an acute presentation of an ovarian cyst?

Answer 9

• ABC approach
• Ovarian cystectomy with oophorectomy if there is any necrosis
• Broad spectrum Abx

Question 10

How should we manage a Solid/persistent/growing cysts?

Answer 10

should always be removed

Question 11

What are the complications of ovarian cysts?

Answer 11

Complications: cyst accident, subfertility, malignant change, oophorectomy

All ovarian cysts that are suspicious of malignancy in a postmenopausal woman, as indicated by a RMI I greater than or equal to 200, CT findings, clinical assessment or findings at laparoscopy, require a full laparotomy and staging procedure.

Question 12

What is an ovarian cyst accident? How should they be managed?

Answer 12

• Rupture of contents of ovarian cyst into peritoneal cavity → intense pain, especially if endometrioma
  or dermoid cyst
• Haemorrhage into a cyst or peritoneal cavity also causes pain
• Torsion of the pedicle causes infarction of the ovary + tube with severe pain
  o Urgent surgery and detorsion required

Question 13

What are the disorders of ovarian function?

Answer 13

• PCOS: oligomenorrhoea + hirsutism + sub-fertility - Cysts = multiple, poorly developed follicles
• Premature menopause: last period before 40 years of age
• Problems of gonadal development: gonadal dysgeneses – most common = Turner’s Syndrome

Question 14

How do we classify primary neoplasms?

Answer 14

 Can be benign or malignant
 Classified together as benign cyst can undergo malignant change

Question 15

What are epithelial tumours? Who do they present in?

Answer 15

Tumour derived from epithelial layer covering the ovary (90%). Mainly postmenopausal women

Question 16

How do we classify epithelial tumours?

Answer 16

By histology

Question 17

What are the types of epithelial tumours? How do we manage them?

Answer 17

• Borderline - malignant histological feature are present but not invasive
  
  • May become malignant so surgery is advised .
  • In younger women - close observation may be offered following removal of the cyst or unaffected overy to preserve fertility
  • Recurrence can occur up to 20 yrs later.
• Serous cystadenoma
  (adenocarcinoma)
  – most common malignant ovarian neoplasm 50% malignancies
  
  • Classed as high grade or low grade
• Mucinous cystadenoma
  (adenocarcinoma) → can become v large
  – 3% of ovarian malignancies
  
  • Abdominal cavity fills with gelatinous mucin secretions
• Pseudomyxoma peritonei
  – borderline variant of
  mucinous cystadenoma
  → filling of abdominal
  cavity with mucinous
  secretions
• Endometroid Carcinoma
  – 10% of ovarian malignancies,
  similar histologically to
  endometrial Ca → poor prognosis
• Clear cell carcinoma - malignant varinat that accounts for 10% of ovarian malignancies
• Brenner Tumour
  Rare, small, usually
  benign

Question 18

What are germ cell tumours and who do they present in?

Answer 18

Undifferentiated primordial
germ cells of gonad and present in young, premenopausal women.

Question 19

What are the types of germ cell tumours? How do we manage them?

Answer 19

• Teratoma (dermoid cyst)
  
  • Common and benign
  • Contains fully differentiated tissue of all cell lines (commonly hair and teeth)
  • Commonly bilateral
  • Asymptomatic
  • Rupture = painful
  • A malignant form, the solid teratoma, also occurs in this age group but v rare.
  • Surgical removal
• Yolk sac tumour
  
  • Highly malignant and present in young women/children.
• Dysgerminoma
  
  • Female equivalent of seminoma
  • Most common malignancy in younger women
  • Sensitive to radiotherapy

Question 20

Define sex cord tumours. Who does it present in?

Answer 20

Tumour from the stroma of the gonad (the cells that hold the ovaries together and produce female hormones). Mainly young premenopausal or post-menopausal

Question 21

What are the types of sex cord tumours?

Answer 21

• Granulosa cell tumour
  
  • Rare, malignant but slow growing
  • post-menopausal women
  • Secrete oestrogens and inhibin
  • Stimulation of endometrium → PMB, endometrial hyperplasia and precocious puberty
  • Serum inhibin used as a marker
• Thecoma
  
  • Rare, usually benign
  • Secrete androgens or oestrogens
• Fibroma
  
  • Rare and benign
  • Meig’s Syndrome: ascites and right pleural effusion found with small ovarian mass
  • Cured by removal of the mass

Question 22

What are secondary malignancies?

Answer 22

Ovary = common site for metastases, particularly from breast and GIT

Secondaries = 10% malignant ovarian masses

Question 23

Describe the epidemiology of ovarian cancer.

Answer 23

In the United Kingdom each year, ovarian cancer is diagnosed in over 6500 women and causes approximately 4400 deaths.

Many women present with advanced disease with little prospect of cure.

The 5 year survival rate for advanced ovarian cancer between 2005–2009 was 43%

Question 24

Describe the aetiology of ovarian cancers.

Answer 24

• Premalignant phase not normally recognised
• Risk factors relate to number of ovulations
  o Early menarche
  o Late menopause
  o Nulliparity
• Protective
  o Pregnancy
  o Lactation
  o COCP
• OCa may be familial via BRCA1, BRCA2 and HNPCC
  o Two relatives affected, lifetime risk = 13.1%
  o With BRCA1: 50%

Nulliparity, fertility tx, FH, Hx of breast cancer, high fat diet, HNPCC, Lynch syndrome, early menarche/late menopause, IUD, endometriosis

Question 25

What are the clinical features of ovarian cancers?

Answer 25

* History * Vague/absent symptoms * 70% present with stage 3-4 * Sx include persistent abdominal distension, feeling full, loss of appetite, pelvic/abdominal pain, increased urinary urgency or frequency * Many symptoms similar to IBS → unusual to present for first time in older women * Ask about breast and GI symptoms as could be metastasis * Examination * Cachexia * Abdominal/pelvic mass * Ascites

Question 26

How do ovarian carcinomas spread?

Answer 26

 Ovarian adenocarcinoma spreads directly within the pelvis and abdomen = transcoelomic spread  Also lymphatic and blood-borne spread

Question 27

What are the stages of ovarian cancers?

Answer 27

Question 28

What tests should be done in a primary care setting?

Answer 28

For any woman who has normal serum CA125 (less than 35 IU/ml), or CA125 of 35 IU/ml or greater but a normal ultrasound: assess her carefully for other clinical causes of her symptoms and investigate if appropriate if no other clinical cause is apparent, advise her to return to her GP if her symptoms become more frequent and/or persistent. **Malignancy indices** Calculate a risk of malignancy index I (RMI I) score (after performing an ultrasound ) and refer all women with an RMI I score of 250 or greater to a specialist multidisciplinary team.

Question 29

How should we establish the diagnosis in secondary care?

Answer 29

* Establishing Diagnosis (secondary care) * Ca125 + USS * Women under 40: measure AFP and hCG (both raised in germ cell tumours) * Calculate risk of malignancy index (RMI) * RMI = U x M x Ca125 level * U = USS Score * 1 point for each of the following characteristics: * Multilocular cysts * Solid areas * Metastases * Ascites * Bilateral lesions * 1 point, U =3; 2-5 points (max score) U=3 * M = menopausal status * 1 = premenopausal * 3 = postmenopausal * All women with RMI \>250 referred to specialist MDT * CT abdo-pelvis is performed for initial assessment of extent of disease

Question 30

How do we think of managing ovarian cancers?

Answer 30

* Adapt management to stage (may need to do additional ix) * Support/Palliative care

Question 31

When should **systematic retroperitoneal lymphadenectomy** be done?

Answer 31

Perform retroperitoneal lymph node assessment as part of optimal surgical staging in women with suspected ovarian cancer whose disease appears to be confined to the ovaries (that is, who appear to have stage I disease). Lymph node assessment involves sampling of retroperitoneal lymphatic tissue from the para-aortic area and pelvic side walls if there is a palpable abnormality, or random sampling if there is no palpable abnormality. ## Footnote Do not include systematic retroperitoneal lymphadenectomy (block dissection of lymph nodes from the pelvic side walls to the level of the renal veins) as part of standard surgical treatment in women with suspected ovarian cancer whose disease appears to be confined to the ovaries (that is, who appear to have stage I disease).

Question 32

When should **Adjuvant systemic chemotherapy for stage I disease** be offered?

Answer 32

_Do not offer_ adjuvant chemotherapy to women who have had optimal surgical staging and have low-risk stage I disease (grade 1 or 2, stage Ia or Ib). Offer women with high-risk stage I disease (grade 3 or stage Ic) adjuvant chemotherapy consisting of 6 cycles of carboplatin. Discuss the possible benefits and side effects of adjuvant chemotherapy with women who have had suboptimal surgical staging and appear to have stage I disease.

Question 33

What is optimal surgical staging?

Answer 33

Optimal surgical staging constitutes: midline laparotomy to allow thorough assessment of the abdomen and pelvis; a total abdominal hysterectomy, bilateral salpingo-oophorectomy and infracolic omentectomy; biopsies of any peritoneal deposits; random biopsies of the pelvic and abdominal peritoneum; and retroperitoneal lymph node assessment

Question 34

How do we manage advanced (Stage II to IV) ovarian cancer?

Answer 34

If performing surgery for women with ovarian cancer, whether before chemotherapy or after neoadjuvant chemotherapy, the objective should be complete resection of all macroscopic disease. ## Footnote Stage 2-4: carboplatin or cisplatin alone or combination with paclitaxel o 2/3 of women whose tumours initially respond to first-line chemo will relapse within 2 years of completing tx

Question 35

What are the different types of chemotherapy?

Answer 35

Question 36

What is the follow up after ovarian cancer tx?

Answer 36

Levels of Ca125 CT scanning Interval debulking of residual tissue Chemotherapy prolongs short term survival and improves QOL Poor prognostic indicators o Advanced stage o Poorly differentiated tumours o Slow or poor response to chemotherapy Death due to bowel obstruction or perforation Prognosis improving for those detected early

Question 37

What forms part of the palliative care on ovarian cancers?

Answer 37

Palliative care: achieve total care of the patient whose disease is incurable  Improve QOL  Address sx such as pain, nausea, bleeding, sx of obstruction & also psychological, social and spiritual needs  Usually coordinated by GP, specialist practitioner e.g. macmillan nurse and specialist hospices/ gynaecology units

Question 38

How do we control symptoms in ovarian cancer?

Answer 38

Symptom Control  Pain o Analgesic ladder o Co-analgesics = antidepressants, steroids and cytotoxics o Opioid analgesia can be patient-controlled and usually accompanied by anti-emetics o Alternative therapies e.g. acupuncture also used  Nausea and vomiting o 60% patients with advanced carcinoma o Due to opiates, metabolic causes (uraemia), vagal stimulation or psychological factors o Antiemetics include anticholinergics, antihistamines, dopamine antagonists of 5HT-3 antagonists  Heavy vaginal bleeding o Advanced cervical and endometrial cancer o High dose progestogens o Radiotherapy if not already used  Bowel obstruction and ascites o Particularly in OCa o Ascites – drained by repeated paracentesis o Mx of obstruction at home – 1/3 spontaneous resolution o Metoclopramide used if partial obstruction o Can use dexamethasone for tissue oedema o Complete obstruction: cyclizine and ondansetron for N&V, with hyoscine for spasm o Surgical palliation indicated with acute, single-site obstruction o Can insert stents in sigmoid or rectum  Terminal distress o Last 24h o Good symptom control with anxiolytics and analgesics without overlying sedating

Question 39

Define ovarian torsion.

Answer 39

Ovarian torsion is a condition where the ovary twists in relation to the surrounding connective tissue, fallopian tube and blood supply (the ***adnexa***).

Question 40

Who usually gets ovarian torsion?

Answer 40

Ovarian torsion is usually due to an ***ovarian mass*** larger than 5cm, such as a ***cyst*** or a ***tumour***. It is more likely to occur with ***benign tumours***. It is also more likely to occur during pregnancy. Ovarian torsion can also happen with normal ovaries in younger girls ***before menarche*** (the first period), when girls have longer ***infundibulopelvic ligaments*** that can twist more easily.

Question 41

What are the consequences of ovarian torsion?

Answer 41

Twisting of the adnexa and blood supply to the ovary leads to ***ischaemia***. If the torsion persists, ***necrosis*** will occur, and the function of that ovary will be lost. Therefore, ovarian torsion is an ***emergency***, where a delay in treatment can have significant consequences. Prompt diagnosis and management is essential. ## Footnote A delay in treating ovarian torsion can result in loss of function of that ovary. The other ovary can usually compensate, so fertility is not typically affected. Where this is the only functioning ovary, loss of function leads to infertility and menopause. Where a necrotic ovary is not removed, it may become ***infected***, develop an ***abscess*** and lead to ***sepsis***. Additionally it may ***rupture***, resulting in ***peritonitis*** and ***adhesions***.

Question 42

How does ovarian torsion present?

Answer 42

* Sudden pelvic or abdominal pain, fluctuating, radiating to loin or thigh * Nausea + Vomiting * Pyrexia * Tachycardia * Generalised abdominal tenderness, localised guarding, rebound * Cervical excitation, adnexal tenderness, adnexal mass

Question 43

How do we diagnose ovarian torsion?

Answer 43

***Pelvic ultrasound*** is the initial investigation of choice. Transvaginal is ideal, but transabdominal can be used where transvaginal is not possible. It may show “***whirlpool sign***”, ***free fluid*** in pelvis and ***oedema*** of the ovary. Doppler studies may show a lack of blood flow. The definitive diagnosis is made with ***laparoscopic surgery***.

Question 44

How do we manage ovarian torsion?

Answer 44

Patients need emergency admission under gynaecology for urgent investigation and management. Depending on the duration and severity of the illness they require ***laparoscopic surgery*** to either: * ***Un-twist*** the ovary and fix it in place (***detorsion***) * ***Remove*** the affected ovary (***oophorectomy***) The decision whether to save the ovary or remove it is made during the surgery, based on a visual inspection of the ovary. Laparotomy may be required where there is a large ovarian mass or malignancy is suspected.