RANZCOG questions

Question 1

State how cervical cancer is staged. (1 mark)

Answer 1

FIGO classification system Staging is clinical, using a combination of diagnostic tests to determine the local spread of cervical cancer during examination under anaesthesia

Question 2

Outline the diagnostic tests which may be used to determine the stage using the International Federation of Gynaecology and Obstetrics (FIGO) staging system. (5 marks) (cervical ca)

Answer 2

• Colposcopy and biopsy- to assess the size of macroscopic tumours • Cone biopsy- to assess depth of invasion and size of microscopic tumours • EUA- examination of vulva, vagina and cervix under anaesthesia, bimanual and PR • Sigmoidoscopy- examination of invasion into the sigmoid colon • Cystoscopy- examination of invasion into the bladder • CXR- distant spread into the lungs • IVP- IV contrast given to image the renal tract and review if obstruction is present • Other modes of imaging- CT, MRI, PET CT are not part of formal FIGO staging but assist in surgical planning as they can help define node involvement

Question 3

A 30 year old woman has a cervical biopsy showing cervical intraepithelial neoplasia Grade 3 (CIN 3). The histology of the large loop excision of the transformation zone (LLETZ) specimen is reported as “squamous cell carcinoma with invasion 6 mm deep and lateral spread of 9 mm”. Her last Pap smear, 5 years ago, was normal. She has no other significant past history and has one child delivered vaginally. After full assessment, diagnosis of cervical carcinoma Stage IB1 is made. b. Treatment options include surgery or chemoradiation. What factors influence the choice of treatment for this woman? (2 marks)

Answer 3

Patient factors • Current pregnancy status • Future plans for childbearing and fertility • Preference Tumour factors • If tumour is fully resectable • Any evidence of spread

Question 4

Comprehensively outline the late complications of radiotherapy that could occur in this woman. (7 marks) (1b1 cervical ca)

Answer 4

1. Vaginal stenosis: Can occur with external beam and brachytherapy, Dyspareunia and interference with coitus- dilator therapy can help 2. Infertility and early menopause: Prevents further childbearing and if falls pregnancy high-risk pregnancy. Radiation to the ovaries raises the risk of early menopause and thus menopausal symptoms (VVA, VMS etc) and risks associated with early menopause (CVD, osteoporosis) 3. Bladder: Urinary incontinence and recurrent UTI 4. Chronic proctitis: Symptoms include rectal and abdominal pain, tenesmus, diarrhoea, rectal bleeding and dyschezia 5. Ileitis: Symptoms include severe abdominal pain, chronic constipation or diarrhoea, chronic PR bleeding Can result in stricture formation and bowel perforation 6. Fistula: Vesico-vaginal, ureterovaginal and rectovaginal fistula can all occur and be dehibilitating for women 7. Lymphoedema: Causes constant aching, difficulty with mobilising, predisposes to leg infections 8. Femoral head necrosis: Hip pain and immobility, necessitates hip replacement when severe 9. Pelvic malignancy: Risk of spontaneous pelvic malignancy increase eg leiomyosarcoma 10. Psychological symptoms: Anxiety, depression

Question 5

In both Australia and New Zealand, indigenous populations have significantly higher incidence of, and mortality from, cervical malignancy than non-indigenous populations. a. Identify five (5) epidemiological factors which may account for this. (5 marks)

Answer 5

Increased incidence of smoking Lower age at commencing sexual intercourse Risker sexual behaviour ie multiple partners, less likely to use barrier contraception Barriers to cervical screening and colposcopy Barriers to appropriate medical care and follow up eg financial, psychosocial, transport issues Barriers to education regarding the importance of cervical screening, immunisation and treatment of CIN Lower Gardasil immunisation uptake Cultural barriers

Question 6

A 32 year old woman is diagnosed with cervical carcinoma Stage 1B. b. According to the FIGO Staging system, list all the examinations and investigations that could have been included to establish the diagnosis and the stage of her cancer. (4 marks)

Answer 6

Physical examination- speculum, bimanual and rectovaginal examination for palpation and inspection of the primary tumour, uterus, vagina and parametria. Palpation of groin and supraclavicular LN to exclude metastatic cancer. Cystoscopy, Proctosigmoidoscopy EUA, hysteroscopy, endocervical curettage Colposcopic assessment- Colposcopy with directed cervical biopsy for visible cervical abnormalities/ lesion +/-Endocervical curettage, cone biopsy CXR Intravenous pyelogram (IVP) – Evaluation for urinary tract obstruction Imaging not part of FIGO staging but to help guide management: CT or MRI abdomen and pelvis to evaluate extra peritoneal or lymph node spread PET Barium enema

Question 7

Her tumour is a squamous cell carcinoma which is 2cm in size. Imaging studies do not suggest lymph node involvement. (You may wish to use a table to answer the following.) c. i) List her three (3) treatment options. (1½ marks) ii) Outline one (1) indication for each treatment option. (1½ marks) iii) List two (2) long-term complications associated with each treatment option. (Use different complications for each treatment option). (3 marks)

Answer 7

1. Modified radical hysterectomy and lymph node dissection. Indication: Complete staging procedure- able to o provide histological specimen and information regarding lymph node spread Has completed famiy Other uterine pathology present- firboid Complication: Lymphadema, Chronic pelvic pain, Damage to bladder/ bowel, Pelvic floor prolapse, Sexual dysfuction, Haematoma 2. Trachelectomy Indication: Fertility sparing option for women in childbearing age Complication: Cervical incompetence Preterm birth Requires cervical suture and ELLSCS Stenosis/ impair fertility 3. Primary radiotherapy Indication: Reserved as primary treatment for women who are not candidates for surgery due to medical comorbidities or poor functional status/ declined surgery Complication: Ovarian failure, Vaginal stenosis, Radiation cystitis, Bladder dysfunction, Dyspareunia Bowel stenosis 4. Hysterectomy with ovarian conservation Indication: Provide treatment wihout casuing premature menopause Complication: Risk of disease spread to the adnexa or parametria Risk of lymphovascular invasion, 2-8%

Question 8

What is CIN? What is metaplasia?

Answer 8

Cancer precursors Lack the features of invasive cancer (no invasion of the basement membrane) Severity of lesion is in thirds from the bottom (basement membrane) upwards. How does metaplasia occur? - rise in oestrogen at puberty - promotes growth of lactobacilli - lowers pH - exposure of low pH to columnar epithelium stimulates metaplasia  squamous - metaplasia most active during adolescence and pregnancy

Question 9

Likelihood of developing cancer for CIN 1,2 and 3:

Answer 9

CIN1 = 1% proceed to cancer CIN2 = 5% CIN3 = 12+ % Only a third of CIN3 will regress.

Question 10

Sensitivity and specificity of smear?

Answer 10

• 55-80% sensitivity for a single test. However very high specificity (98%) - Never evaluated in an RCT however countries using it in screening programmes have noted a dramatic decline in cervical cancer

Question 11

How to take a smear?

Answer 11

Taking a pap smear - avoid menstruation or intercourse 48h prior. - Document date of last period, pregnancy or not, hormone use, IUCDs, menopausal status, past smear history Now Liquid based cytology used - put brush in a liquid transport medium - processed to produce a monolayer of cells on a glass slide. - Blood, mucus and cell overlap eliminated - Most of all collected cell material is available on glass slide - ? lower unsatisfactory smear rate however ? lower positive predictive value.

Question 12

Colposcopy

Answer 12

• magnification 3 to 40 fold - high intensity light - green (red free) filter - normal saline- helps remove mucus - acetic acid (clumps nuclear chromatin) - Lugol (iodine) normal oestrogenised cells stain dark due to glycogen content. Dysplastic cells have lower glycogen content Reid Colposcopic index (each scored 0-2) - peripheral margin (feathery, smooth, rolled) - colour to ACW (shiney translucent, duller white, dull white/ gray) - vascular patterns (fine, absent, coarsed) - lugol solution (positive, partial, negative) Other things to note about abnormal vessels - punctuation (fine  coarse) - mosaicism (mosaic tiles with central punctuation = CIS) - atypical vessels o graded on o vessel calibre o intercapillary distance o uniformity

Question 13

Advantages and disadvantages of LLETZ:

Answer 13

Advantages - can be done in clinic under local - ease of procedure - safety profile - low cost - good tissue specimen Disadvantages - training - bleeding - infection - cervical insufficiency - preterm birth - cervical stenosis

Question 14

Mary Smith is a 54-year old woman presenting with recent onset of vaginal bleeding. Her last period was four years earlier. Mary is obese (BMI 33). She is otherwise well and has not been taking any medication or over-the-counter therapies. Bimanual pelvic examination is normal. Her last Pap smear was 18 months ago and was normal. a) Mary is concerned that she has cancer. What are you going to advise her in the outpatient setting? (3 marks)

Answer 14

• Cancer is not the most common cause of her symptoms (only 10%) but it is an important one to investigate • Potential cancers could include endometrial, cervical, vaginal, vulvar or ovarian (atypical presentation but possible) • Other potential causes for her PMB are: o Atrophy o Polyps o Hyperplasia o Infection • Suggest further investigation with imaging (TV USS) and endometrial biopsy.

Question 15

Mary Smith is a 54-year old woman presenting with recent onset of vaginal bleeding. Her last period was four years earlier. Mary is obese (BMI 33). She is otherwise well and has not been taking any medication or over-the-counter therapies. Bimanual pelvic examination is normal. Her last Pap smear was 18 months ago and was normal. List and briefly compare three modalities of ultrasound evaluation of the endometrium in relation to this presentation. (3 marks)

Answer 15

• Grayscale USS – acceptable, cheap, TV gives best definition but TA may be more acceptable to some women • Sonohysterography – best for assessment of endometrial abnormalities e.g. polyps. More expensive • Doppler USS – helpful for assessing vascularity e.g. polyp. No additional cost (standard setting on most machines)

Question 16

Briefly compare and contrast the role of outpatient endometrial sampling (such as Pipelle) versus formal hysteroscopy, dilatation and curettage with reference to the differential diagnosis of endometrial carcinoma in this presentation. (4 marks)

Answer 16

• Pipelle*
• Hyst D&C*

Cost

Cheap

Expensive

Need for GA

No

Sometimes

Outpatient procedure

Yes

Sometimes office

Targetted biopsy

No

Yes

Visualise endometrial cavity

No

Yes

Can ‘see and treat’

No

Yes e.g. polyp, submucous fibroid

Risk perforation

No

Possible

Need for cervical dilation

No

Possible

Sensitivity for cancer

High

High

False negative

Possible (10%) – may miss polyp or may miss cancer if small area

Less likely

Question 17

The histopathology of the endometrium shows “complex atypical endometrial hyperplasia”.

d) Briefly discuss the significance of this report and the likely pathogenesis in this patient. (2 marks)

Answer 17

• Significance:
  
  • This is a premalignant condition with a high (30%) chance of coexistent endometrial cancer
• Pathogenesis:
  
  • Related to oestrogen exposure – obesity and/or other risk factors
  • Unopposed oestrogen leads to excessive stimulation/growth of endometrium
  • Progressive growth of endometrium can cause hyperplasia
  • Further progression can occur to atypia and malignancy

Question 18

List the management options available for Mary. (3 marks)

(obese, endometrial hyperplasia with atypia)

Answer 18

• Hysterectomy recommended with the presence of atypia
• Can offer BSO and peritoneal washings at the same time, as if coexistent endometrial cancer diagnosed on histology after hysterectomy, will be recommended for staging surgery (reduces risk of needing subsequent surgery)
• Progesterone therapy – PO or mirena and ongoing surveillance with repeat endometrial biopsy in 3-6 months
• Possible if not fit for surgery (unlikely in this case) or unwilling
• If persists try higher dose, if persists despite this recommend hysterectomy
• If normalizes continue progesterone for minimum of 1 year with annual TVUSS

Question 19

A 60 year old woman presented to you with a history of postmenopausal bleeding. An endometrial biopsy demonstrates endometrial adenocarcinoma.

a. Describe the main pre-operative and intra-operative management principles for staging and treatment in this case. (6 marks)

Answer 19

Pre-operative:

Explain and counsel patient re diagnosis, treatment and possible prognosis

Gynaeoncology MDT referral (gynaeoncologists, medical and radation oncologists, radiologists, pathologists, nurse specialists) - to assess likely stage and grade after review of endometrial biopsy histology and imaging and plan primary surgeon and surgical approach

FBC, G&S, U&E, LFT – for medical pre-op workup

CXR – metastases

MRI – assess depth of invasion of tumour for surgical planning (PLND if >50% DOI) and for cervical involvement, lymph node involvement and metastases

CT chest/abdo/pelvis if high grade or high risk cancer eg sarcoma

Assess comorbidities, fitness for surgery, optimize health e.g. correct anaemia. Anaesthetic review.

Intra-operative – staging laparotomy and treatment:

Consent

Midline laparotomy

TAH/BSO and washings for all women

PLND if - >50% DOI on MRI (stage 1b or above), high risk histological subtype or grade 3 tumour

Inspection/palpation of all peritoneal surfaces – include liver, omentum, pelvic sidewalls, para-arotic nodes, biopsy/cytoreduction of any suspicious lesions.

VTE prophylaxis

Question 20

List eight main prognostic factors associated with a poor outcome in this condition. (4 marks)

(Endometrial adenocarcinoma)

Answer 20

Disease factors:

• High stage disease
• High grade tumour
• High risk histological subtype e.g. papillary serous, clear cell
• Lymphovascular space invasion
• Tumour > 2cm
• Hormone receptor status
• Nuclear grade

Patient factors:

• Age > 60
• Medical comorbidities

Obesity

Question 21

Describe the situations where radiotherapy can be used in the management of endometrial carcinoma. (5 marks)

Answer 21

Adjuvant therapy:

• Stage 1b – 2
• High grade tumour – grade 3 (any stage)
• High risk histological subtype (any stage or grade)

2. Primary therapy for women not fit for surgery (but poor outcomes)
3. Palliation – advanced disease (usually in combination with chemotherapy)
4. Treatment of local recurrence if not had previous radiotherapy

Can be given in the form of external beam radiation and/or vault brachytherapy

Question 22

What are the problems associated with a population-based cervical screening?

Answer 22

• Acceptability – may not be acceptable to all patients to have a smear
• Reliability – some false positive and false negatives, some missed cases and some unnecessary colposcopy/treatment/anxiety
• Cost/facilities – advertising campaigns to increase participation, places to have smear taken, pathology labs to interpret results, colposcopy clinics to followup abnormalities, quality control and assessment/audit
• Some cultural/language barriers and lack of education regarding importance

Question 23

1. What is necessary for a successful population based cervical screening program?

Answer 23

• Nationwide program for recall and recording of results
• Availability of skilled smear takers in primary practice in close proximity for all women
• Low cost appointments for smear taking
• Education programme to emphasise the importance of smears and screening interval
• System of notification and explanation of smear results
• Clear guidelines for smear takers on when to refer for colposcopy
• Adequate colposcopy services in all regions
• Skilled and accredited colposcopists
• Skilled and accredited labs for cytology and histology
• MDT meetings for complex cases and discrepancies between smear/colposcopy/biopsy
• Clear guidelines on followup
• Gynaeoncology teams to refer patients with cervical cancer

Question 24

1. What are the indications for cone biopsy?

Answer 24

• Suspicion of early microinvasive SCC or known stage 1a1 SCC – fertility sparing option
• Inability to visualise upper limit of lesion or transformation zone with HSIL referral
• Suspicion of concurrent significant glandular abnormality

Question 25

1. **HPV testing for women with treated HSIL – what are the sensitivity and PPV for residual/recurrent disease?**

Answer 25

* Sens 96% * Spec 81% * PPV 46% * NPV 99%

Question 26

A 63 year old woman presents after one episode of light vaginal bleeding over the last week. She has a BMI of 42 kg/m2, chronic hypertension and Type 2 diabetes. a. List five (5) possible causes for her bleeding. Identify the most common cause. (3 marks)

Answer 26

* Vulvo-vaginal atrophy (most common cause) * Endometrial hyperplasia (endogenous or exogenous oestrogen) * Endometrial polyp * Cervical polyp * Cervical cancer * Trauma * Anti-coagulants * STIs

Question 27

A 63 year old woman presents after one episode of light vaginal bleeding over the last week. She has a BMI of 42 kg/m2, chronic hypertension and Type 2 diabetes. i) List four (4) risk factors for endometrial cancer not mentioned in the scenario that you should elicit from her .history. (2 marks)

Answer 27

* Early menarche and late menopause * Nulliparity * Unopposed oestrogen Rx * Tamoxifen * PCOS * Familial cancer syndrome

Question 28

ii) Justify your initial management of this patient to assess her risk for endometrial cancer. (3 marks)

Answer 28

Management Justification Pelvic examination Will reveal vulvovaginal atrophy, cervical polyps or masses Assess the size and mobility of the uterus Swabs + cervical smear Important for screening for cervical pre-malignancy, not sensitive for invasive cervical cancer, may see atypical endometrial cells on smear test Rule out STI as contributory to bleeding Pelvic ultrasound Review pelvic anatomy, endometrial thickness (ET \<5 usually excludes endometrial cancer and \>20 is highly suggestive), presence of polyps, fibroids, or other masses Endometrial sampling Office endometrial biopsy has a good sensitivity for endometrial cancer- 95-99% if the cancer/hyperplasia is \>50% of the cavity, in women with a high suspicion of cancer, formal sampling under GA will be needed Hysteroscopy Reviews the structure of the endometrium, can identify polyps and thickened endometrium. Can be done as an office procedure or under GA.

Question 29

This patient underwent hysteroscopy and endometrial biopsy. The histology report showed a grade 1 endometrial adenocarcinoma. c. Discuss the rationale for your management plan for this patient. (5 marks)

Answer 29

* Explain results to patient and counsel regarding surgery, next steps, ensure support person present, give written information * MRI pelvis: in New Zealand, surgery for superficial endometrial cancer is performed by general gynaecologists, rather than gynae-oncologists, so an MRI needs to be done before a gynae onc MDM referral to review degree of invasion and any obvious nodal metastases * CT chest/abdo/pelvis: used to exclude other masses or metastases * Consider PET scan where lymph node metastases are suspected * Refer to gynae onc MDM: review of imaging, pathology and decision about when/where/who will do the operation * TAH/TLH, BSO, and washings as recommended treatment * Intraoperative frozen section or inspection of the myometrium to determine the degree of myometrial invasion can be done at the same time * Pelvic LN dissection recommendation depends on the unit internationally- in low-risk women with small tumours it may not be necessary, should be done if \>50% myometrium involved * Explain further treatment may be needed if the intra-operative findings are different from the findings on imaging * Pre-op planning * Pre-operative bloods: group and hold, full blood count, renal function and electrolytes * Anaesthetic assessment: ensure anaesthetic is safe * Optimisation of medical conditions pre-op

Question 30

d. Discuss the aspects in her history that would alert you to the possibility of her having Lynch syndrome. (2 marks)

Answer 30

* Personal history of ovarian, urinary tract, or colorectal cancer (right colon) * Family history (Amsterdam criteria) (3, 2, 1 rule): * 3 affected family members with histologically verified lynch-syndrome associated cancers, one of whom is a first degree relative of the other two and in whom familial adenomatous polyposis has been excluded * 2 generations of affected family members * 1 or more cancers diagnosed before the age of 50

Question 31

a) Outline the association between HPV & cervical cancer. (5 marks)

Answer 31

Role of HPV in cervical cancer: * Overwhelming evidence that HPV infection is necessary, although not sufficient, for development of cervical ca. * HPV is central to the development of cervical cancer and can be detected in 99.7% of cervical cancers * Among the more than 40 genital mucosal HPV types identified, approximately 15 are known to be oncogenic. * The two most common ones, HPV 16 and 18, are found in over 70% of all cervical cancers. * HPV infection is extremely common, with \>50% of sexually active women acquiring HPV at least once by 50 yrs * Most HPV infections are transient and the virus alone is not sufficient to cause cervical cancer * Cervical cancer is a rare outcome of HPV infection, and progression of HPV infection to cancer is slow

Question 32

**b) ****Briefly describe the two vaccinations available in Australia & New Zealand. (2 marks)**

Answer 32

Gardasil * Quadrivalent HPV vaccine * Targets HPV genotypes 16 & 18, which cause approximately 70% of cervical cancers and precancerous lesions * Also targets HPV genotypes 6 and 11, which cause 90% of genital warts. * Also registered for the prevention of vulval and vaginal cancer, and their pre-cancerous or dysplastic lesions * Licensed for use in females aged 9 to 45 years * Also approved for use in males aged 9 to 15 years for the prevention of HPV infections * 3 dose schedule given at 0, 2, and 6 months * Efficacy in preventing HSIL = 90% in HPV-naïve populations * Efficacy in preventing HSIL = 40% in general population * Also provides 20% protection against CIN2 caused by non-vaccine HPV types Cervarix: * Bivalent vaccine * Targets HPV genotypes 16 & 18, which cause approximately 70% of cervical cancers and precancerous lesions * Licensed for use in females aged 10 to 45 years * 3 dose schedule given at 0, 1, and 6 months * Efficacy in preventing HSIL = 90% in HPV-naïve populations * Efficacy in preventing HSIL = 30% in general population * Also provides 50% protection against CIN2 caused by non-vaccine HPV types

Question 33

**c) ****Describe the National Immunisation Program in Australia or New Zealand. Whom is it given to & how is it given? (4 marks)**

Answer 33

Australian Immunisation Program: * Endorsed by RANZCOG * Funds free vaccination for all women aged 12 to 26 years * Catch up program for schoolgirls concluded at the end of 2008 * Program for young women concluded at the end of 2009 * On an on-going basis, 12 year old Australian girls will receive a free vaccination in the first year of high school * 3 dose injection schedule given at 0, 2, and 6 months

Question 34

**d) ****22 year old women with low grade intraepithelial lesion on Pap smear. What would you advise her? (4 marks)**

Answer 34

Obtain history: * Symptoms (IMB, PCB) * Pap smear history (previous pap smear frequency and results, including colposcopies and treatment) * Gynaecological history (LMP, contraception) * Obstetric history (pregnancies and deliveries) * Medical and surgical history (immunosupression, smoking) * Family history (cervical cancer) Advise: * Cease smoking * Notify if symptoms occur (IMB, PCB) * Offer HPV vaccination * Counsel that vaccine may be less effective for those women already exposed to HPV * Most likely benefit from protection against disease caused by HPV subtypes not already exposed to * Advise need for ongoing cervical cytology despite vaccine * Repeat pap smear in 12 months if asymptomatic * Refer for colposcopy if cervical dysplasia confirmed again * Need 2 normal pap smears 12 months apart before extending pap smear interval to 2 years * Advise colposcopy if symptomatic * Offer STI screen if appropriate and encourage safe sex practices

Question 35

1. **“Breast cancer pathology is similar in age-matched pregnant and non-pregnant women, but the prognosis for cancers diagnosed in pregnancy is worse.”** ## Footnote **Discuss this statement. (2 marks)**

Answer 35

Pregnancy itself does not appear to worsen the prognosis for women diagnosed with breast ca during the pregnancy (for age and stage matched women). However * Breast cancers diagnosed in pregnancy (i.e. in women of childbearing age) are often have more aggressive features (e.g high grade, ER negative) and therefore are more advanced at diagnosis, leading to inferior prognosis

Question 36

**A 36 year old woman presents at 14 weeks gestation with a persistent breast lump that she first noticed about one month ago.** **b. i) List the imaging and investigations that are appropriate to diagnose (not stage) breast cancer. (3 marks)** **ii) How does her pregnancy impact on the use and interpretation of each of these tests? (3 marks)** **[You may use a table to answer the question.]**

Answer 36

Investigation Impact from pregnancy Mammography High false negative rate (increased breast density) Radiation effect – use shielding USS First line in pregnancy High sens/spec Non-ionising Percutaneous core biopsy Gold standard Recommended for all breast lumps in pregnancy Alert pathologist that pt is pregnant – proliferative changes in the breast MRI - gadolinium Only if USS inconclusive Limited data FNA Often inconclusive due to proliferative changes in the breast

Question 37

**Investigations confirm the presence of a Stage II invasive ductal carcinoma.** 1. **Outline the effect of the cancer on her pregnancy management and subsequent postnatal care. (7 marks)**

Answer 37

Effects of pregnancy on disease – unlikely to be significant Management options in pregnancy: General * MDT involvement – obstetrician, MFM, breast surgeon, oncologist, midwifery, nurse specialist, counselor/psychologist * Consider: * Age * Tumour factors - stage/size/grade/type * Gestation * Desire for future fertility * Assessment of nodal status – axillary USS – if +ve – axillary clearance. If –ve, radioisotope scintigraphy. * Assessment of further metastases – only performed if it will alter management in pregnancy. Otherwise complete staging postpartum. Options: 1. Consider TOP and treat as per non-pregnant – careful discussion with couple 1. Surgery – may include WLE, mastectomy, axillary lymphadenectomy. * Advise safest in T2 – risk of miscarriage lowest * Effects on mother – infection, bleeding, VTE, anaesthetic comps * Effects on fetus – minimal * Usually delay reconstructive surgery until postpartum 1. Chemotherapy – not recommended in T1 during organogenesis * Can be given from 14/40 onwards * Current evidence suggests same regimen as non-preg but ?dilutional effects and effect of increased GFR * Maternal – haemopoetic suppression – in T3 avoid in last 3 weeks of pregnancy to minimise anaemia/neutropenia at delivery * Fetal – poor quality data. Possible effects: IUFD, PTL, haemopoetic suppression 1. Radiotherapy – likely to cause IUFD within 3 weeks * postpone until postpartum period 1. Tamoxifen/herceptin – not recommended (teratogenic) Other antenatal care: * Delivery – aim NVD after 37/40, usual indications for C/S apply * Postpartum – can complete planned adjuvant CTX/RTX. Reconstructive surgery can be performed now. * Breastfeeding: * Breast-conserving surgery – likely to be able to BF * CTX – allow washout period before BF * RTX – breast fibrosis – unlikely to be able to BF * Herceptin – no BF * Contraception * Individualise – non-hormonal ideal e.g. IUCD, sterilization if desired * Avoid oestrogen-containing, limited data on progesterone only methods * In general advise to avoid future pregnancy for 2 years after treatment – highest chance of relapse * Weigh risk of relapse vs risk of subfertility with age – consider fertility referral

Question 39

**A 63 year old woman presents with a 5 year history of vulval itching and soreness.** **a. What are the macroscopic features that would lead you to suspect lichen sclerosus? (4 marks)**

Answer 39

* Porcelain-white papules and plaques of inter-labial sulci, labia minora, clitoral hood, clitoris, perineal body and perineum * Decreased subcutaneous fat – vulval atrophy with small or absent labia minora * Obliteration of anatomical landmarks * Thin labia majora * Sometimes see phimosis of prepuce * Figure of 8 appearance

Question 40

**A biopsy confirms the lesion to be lichen sclerosus.** **b. What advice would you give her about this condition and its management? (6 marks)**

Answer 40

Counselling * Can occur in any age but most common postmenopausally * Autoimmune condition * Causes vulval itch and eventually reduced clitoral sensation * Can result in dyspareunia due to introital stenosis * 1-4% risk of developing vulval squamous cell carcinoma * If adequately managed, risk is reduced Management * Topical steroid therapy for symptoms and slowing of disease progression (which leads to anatomical distortion) * Start with potent topical steroids (e.g. mometasone) to gain relief and then change to a less potent steroid (e.g. hydrocortisone) for maintenance * Vulval care important * Gentle drying of vulva after washing * Avoid perfumes, deodorants, soaps, fabric softeners, tight clothing and excessive pad/panty liner use * No place for surgery – except occasionally adhesion division * **Regular follow-up every 6-12 months** to monitor disease progression and check for developing malignancy

Question 41

**After three years, she presents with a small (\<2cm) ulcerating lesion on one side of the vulva 2cm from the mid line at the level of the posterior fourchette. After examination and biopsy, this is thought to be a stage I b squamous cell carcinoma of the vulva.** **c. What are the management options for this patient? (5 marks)**

Answer 41

* Risk of inguinal node metastases \>8% * Multidisciplinary approach – gynaecologists, gynae-oncologists, cancer nurses, oncologists etc. * Needs referral to tertiary gynae-oncology team * Counsel patient and family – 85% five-year survival rate * Individualise treatment Surgery * Radical vulvectomy * Ipsilateral groin node dissection * If lymph node positive: * Contralateral node dissection, or * Radiotherapy * If two or more positive inguinal nodes, needs bilateral groin and pelvic radiation * Warn her of risks of potential postoperative complications, especially radiotherapy * Early: diarrhea, cystitis, skin reactions, pelvic bone marrow suppression * Late: Bowel obstruction / fistula formation, changes in bladder capacity, vaginal atrophy, vaginal narrowing * Generally minimal role for chemo- or radiotherapy * Sometimes joint chemotherapy used to reduce tumour volume if close to urethra or anus