Problems of the female genital tract

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7 Problems of the female genital tract

Chapter Contents

Benign conditions of the vulva

Bartholin’s cyst
Vulvodynia
Dermatoses
Vulvar vestibulitis
Vulvar intraepithelial neoplasia

Carcinoma of the vulva

Clinical features
FIGO staging
Investigations
Management
Prognosis

Benign conditions of the cervix

Cervical ectropion
Cervical polyps
Cervical neoplasia

Carcinoma of the cervix

Presentation
Investigations
Management
Prognosis
Adenocarcinoma of the cervix

Benign tumours of the uterus

Polyps
Uterine fibroids
Adenomyosis
Congenital abnormalities of the uterus

Endometrial carcinoma

FIGO staging of endometrial cancer (updated 2009)
Microscopic appearance
Management
Prognosis
Follow-up

Ovarian cysts

Ovarian cysts in postmenopausal women

Tumours of the ovary

Epithelial tumours
Germ cell tumours
Sex cord tumours

Ovarian carcinoma

Aetiology
Microscopic appearance
Presentation
Management

Gynaecological cancer in pregnancy

Carcinoma of the cervix
Carcinoma of the ovary
Carcinoma of the vulva and endometrium

Summary

Benign conditions of the vulva

Bartholin’s cyst

Bartholin’s glands are located on the posterolateral aspects of the introitus. Cysts can often be asymptomatic, but can become infected and result in pain, dyspareunia and often discomfort on sitting. If unresolved after a course of antibiotics, the infected cyst may need to be opened, drained and marsupialized under anaesthetic. Marsupialization involves a series of sutures between the cyst edge and vagina so that the cyst is left open to heal by secondary intention. Rarely, a recurrent cyst may need to be excised completely, although this is best done when the infection has resolved.

Vulvodynia

Definition

Vulvodynia is the term used to describe a specific type of vulval pain characterized by burning, stinging, irritation or rawness. However, the majority of women presenting with vulval symptoms will complain of pruritus.

Assessment

Management is aimed at identifying the cause and treating appropriately. A full history must include information about previous treatments, other skin or systemic disorders, hygiene measures, drugs taken and allergies known. Examination should include inspection of skin surfaces, the mouth, hands and nails, and palpation of the inguinal nodes. The vulva, including the mons pubis and the perianal skin, the vagina, urethral meatus and cervix should be inspected. Colposcopy of the vulva can define lesions that are not otherwise visible and a biopsy should be performed if epithelial abnormalities are suspected.

Causes of vulvodynia include vulvar dermatoses and vulvar vestibulitis.

Dermatoses

The dermatoses are classified as non-neoplastic epithelial disorders of the skin and mucosa, and they are outlined below.

Eczema

Allergic responses to substances such as perfume or washing powders can result in an acutely red, swollen, sharply demarcated area that is not only extremely itchy, but also may cause a burning raw sensation. Secondary infection leads to a wet, painful, often offensive-smelling lesion. Treatment is by removing the irritant or allergen and controlling the symptoms with soothing applications, bland emollients, local corticosteroid creams and appropriate therapy for secondary infection.

Lichen planus

This is usually idiopathic, but may be drug-related. Lesions occur in any keratinized skin and are flat, papular and purplish in colour. If the condition is erosive, the inner aspects of the vulva and vagina are affected: the vagina becomes friable with risk of adhesions and stenosis developing. Treatment is with potent steroids topically, or systemically with the addition of azathioprine.

Lichen sclerosus

The symptoms and signs are variable and may affect any age group and should always be considered in young women with dyspareunia. There is usually a long history of vulval irritation or pain and the affected area is usually thin and parchment-like. The affected areas may fuse together with associated genital atrophy. The treatment is with topical steroids or testosterone cream, often requiring maintenance therapy. If not resolving after first-line treatment, then a biopsy should be performed. There is a risk of progression to vulval carcinoma in 5–10% of cases and so patients should be followed up regularly with repeated biopsy of suspicious changes.

Vulvar vestibulitis

This is a very difficult condition to treat and is a diagnosis of exclusion. There are three cardinal criteria for this diagnosis: pain on entry or touch, vestibular erythema and tenderness to pressure.

Management of acute vulvar vestibulitis is medical, with the removal of any allergen or irritant, treatment of infection and local corticosteroid application. Treatment with a topical steroid preparation containing antifungal and antibacterial components (Trimovate) may relieve symptoms.

Vulvar intraepithelial neoplasia

Vulvar intraepithelial neoplasia (VIN) is a condition in which atypical or neoplastic cells are present within the boundaries of the surface epithelium of the vulva. Like its cervical counterpart (cervical intraepithelial neoplasia (CIN)), VIN has been divided into three categories depending on the extent of epithelial involvement and is also associated with CIN in around one-quarter of cases. VIN is uncommon, although it is believed that its incidence is increasing, particularly in young women. As well as the association with CIN there is also an association with human papillomavirus and lichen sclerosus. There are also strong associations between sexually transmitted diseases and VIN, in particular condyloma, herpes simplex, gonorrhoea, syphilis, trichomoniasis and Gardnerella vaginalis.

Clinical features

Symptoms:

• Pruritus, present in 38–73% of cases

• Vulval pain/soreness

• Occasionally it can present with a vulval lump or lesions, often in the perianal region

• It can be asymptomatic, with many lesions detected at routine gynaecological examination.

Physical signs

Clinical manifestations of VIN are highly variable, from papular (wart-like) or macular with irregular borders. Brown or black pigmentation of these lesions is common.

White lesions reflect hyperkeratosis and are commonly seen in the keratinized portion of the vulva.

Diagnosis

Diagnosis of VIN is made on microscopy and, therefore, a biopsy, which can be performed under local anaesthesia, is always required. Thorough assessment of the whole lower genital tract, including cervical cytology and colposcopy, is mandatory in patients with VIN.

Circumstances where excisional biopsies should be considered, rather than limited sampling, are:

• Elevated lesions, irregular surfaces

• Areas with distinct vascular patterns, e.g. mosaic, abnormal vessels

• Excessive hyperkeratosis in hair-bearing areas

• Pigmented lesions

• Elderly women with unifocal lesions.

Management

Management of VIN should be individualized, taking into consideration the presence or absence of symptoms and whether risk factors are present, i.e. postmenopausal presentation, immunosuppressed/immunodeficient patients, women with histologically progressive lesions on serial biopsy or excessively hyperkeratotic lesions.

If the risk of invasion is thought to be low and malignancy is excluded, then major vulval surgery can be avoided. Options include topical 5 fluorouracil, dinitrochlorobenzene, cryosurgery, carbon dioxide laser ablation, vulvectomy and wide local excision.

Small lesions can be dealt with in the outpatient clinic using local anaesthesia. Large areas of VIN require general anaesthesia for laser treatment.

Carcinoma of the vulva

This accounts for approximately 5% of genital tract cancers, with just over 1000 cases diagnosed in the UK each year. It is most common (over 80% of cases) after the menopause in the 60–70-year age group, although it can occur in younger women with a history of premalignant VIN.

The majority are squamous cell in origin, with the remainder including the rarer lesions of melanoma, basal cell carcinoma, sarcoma and adenocarcinomas of the Bartholin’s gland.

Clinical features

Patients will usually experience symptoms of pruritus, bleeding or discharge or may present with a vulval mass. Examination will reveal an ulcer or mass, usually on the labia majora or clitoris, and often palpable inguinal lymph nodes.

FIGO staging

Staging is performed following surgical removal and histological assessment. Approximately one-half present with stage 1 disease and spread is via the lymphatic drainage of the vulva to the superficial and then deep inguinal nodes, and finally to the external iliac nodes. Pelvic lymph node spread is slow and will, therefore, indicate advanced disease with poor prognosis.

• Stage 0 – carcinoma in situ (preinvasive)

• Stage 1 – tumour confined to vulva or vulva and perineum < 2 cm in diameter with no nodal involvement

• stage 1a – stromal invasion < 1 mm
• stage 1b – stromal invasion > 1 mm with negative nodes

• Stage 2 – tumour > 2 cm in diameter with no nodal involvement

• Stage 3 – spread of tumour to urethra, vagina or anus or unilateral involvement of nodes. This stage has been subdivided into A, B or C according to number and nature of lymph node metastases. These changes reflect the fact that the number of nodes involved is a more significant prognostic factor than fully resected local disease

• Stage 4 – tumour within rectum, bladder, bone or distant metastases and bilateral nodal involvement.

Investigations

A biopsy is taken for histological diagnosis. Following diagnosis, the patient is prepared for surgery with routine bloods (including full blood count, urea and electrolytes, group and crossmatch) and chest X-ray and electrocardiograph. For tumours > 2 cm, computed tomography (CT) of chest, abdomen and pelvis can detect disease above the inguinal ligament.

Management

In stage 1a carcinoma, a wide local excision is appropriate with regular follow-up. In all other stages, a wide local excision and lymphadenectomy should be carried out. Sentinel lymph node mapping (the sentinel node receives the primary lymphatic drainage from the tumour) can be performed peroperatively as part of the staging procedure in order to reduce morbidity of radical lymphadenectomy. If the lesion is greater than 2 cm from the midline, then a unilateral inguinal lymphadenectomy is appropriate, but if < 2 cm from the midline, a bilateral inguinal lymphadenectomy should be performed. This type of surgery can cause postoperative problems with wound breakdown, infection and lymphoedema.

Radiotherapy can be considered in cases of local recurrence or nodal disease but chemotherapy is rarely used, except in younger patients with positive nodes.

Prognosis

For stage 1 carcinoma the 5-year survival rate is 80%; it is 50% for stage 2, 30% for stage 3 and 10–15% for stage 4.

Benign conditions of the cervix

The outer surface of the cervix within the vagina is covered with stratified squamous epithelium, which becomes columnar within the cervical canal at the squamocolumnar junction. With the onset of puberty, the ovarian hormones cause eversion of the lower cervical canal so that this junction approaches the external cervical os. Squamous metaplasia occurs when the columnar epithelium at this junction is replaced by squamous epithelium.

Cervical ectropion

This is the florid appearance of the lower cervical canal that is often mistakenly described as an ‘erosion’ and is caused by the hormonal changes during puberty, pregnancy and by the oral contraceptive pill. It is usually asymptomatic, but may cause a persistent vaginal discharge or postcoital bleeding. In the presence of a normal smear test, this can be treated by diathermy or cryocautery in the office.

Cervical polyps

These are usually found protruding through the external cervical os and can either be asymptomatic or cause chronic discharge, postcoital or intermenstrual bleeding. In this situation they can simply be avulsed and the base cauterized with diathermy.

Cervical neoplasia

Role of human papillomavirus

Over 60 genotypes of human papillomavirus (HPV) have been identified; in particular, HPV 16, 18 and 33 have been shown to be associated with cervical neoplasia and with increasing grades of CIN.

Up to 10% of women will have HPV on cervical cytology at some stage and of these, 35–40% will have CIN. However, there is no increased risk of cancer in those patients with HPV without CIN. Most women will contract HPV at some point in their lives but will build up immunity and expel the virus before dysplasia can develop.

Cervical cytology

Definitions:

• Inflammatory – this means that there are increased polymorphs and this may occur during the premenstrual or menstrual phases of the cycle.

• Mild squamous changes – mainly due to inflammation or an artefact of poor fixation, which will require a repeat smear in 6 months.

• Hyperkeratosis – may be due to HPV or uterovaginal prolapse, but CIN may be present in less than 5%.

• Dyskaryosis – this is the term used for cells that come from a dysplastic or neoplastic lesion.

• Transformation zone – this is the area between the original and existing squamocolumnar junction and is where the columnar epithelium undergoes metaplasia. This zone may extend up the cervical canal, especially in the postmenopausal patient.

Cervical screening

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