The broad grouping of vulval SCC is:
- Stage I-II (early, manage with surgery)
- Stage III-IVA (locally advanced, manage with chemoradiotherapy possibly followed by surgery)
- Stage IVB (metastatic disease, control if possible and manage palliatively)
Early Stage Disease (I-II)
The primary treatment for women in this stage is surgical resection. Current practice includes:
- Local excision of the primary tumour with 1 cm margin
- Ipsilateral inguinal lymph node dissection
- Contralateral inguinal lymph node dissection if the lesion is within 1 cm of midline
Clinical assessment of inguinal nodes is unreliable. Inguinal lymph node sampling is required in all cases except stage IA (< 1 mm invasion). Sentinel node techniques are an area of current study but dissection remains the 'gold standard' treatment for patients with early disease. Lymph node dissection is associated with 33% risk of wound breakdown and 50% risk of lymph oedema.
Adjuvant treatment consists of radiation only. Indications for radiotherapy are controversial:
- Treatment of the tumour bed should be considered for tumours with a resection margin of < 8 mm as they are at higher risk of local recurrence
- Treatment of inguinal nodes should be considered if there are over 1 node involved, if there is extranodal extension or if surgery was inadequate. There is debate as to the benefit of nodal irradiation if only one node is involved.
- Adjuvant doses are usually 45 Gy in 25 fractions. Residual macroscopic disease normally requires re-resection.
- Adjuvant radiotherapy improves nodal control from 20% to 5%; it increases the rate of lymphoedema from 10% to 20%. It also improves overall survival by a modest amount.
Areas of Controversy
Primary Inguinal Node Radiation
The use of inguinal nodal irradiation has been examined by the Cochrane Collaboration. Only one study meets acceptable criteria for analysis (Stehman 1992) and the radiotherapy techniques in this study have been criticised. This study demonstrated that although radiotherapy reduced the risk of lymphoedmea in bilateral legs there was an increased risk of inguinal node failure and reduced overall survival.
Locally Advanced Disease (III-IVA)
Surgical management of these patients is highly morbid, often requiring exenteration and almost always requiring bladder and bowel diversion, the only exception being minimal local disease with palpable inguinal node disease.
Neoadjuvant chemoradiotherapy has been explored as a method of reducing the morbidity of extensive surgery in patients with advanced disease. This is the gold standard treatment in patients who are able to tolerate both agents; radiotherapy alone may be acceptable in patients unable to receive systemic cytotoxic therapy.
- Neoadjuvant radiotherapy dose is usually 45 Gy in 25#
- Chemotherapy regimens normally include 5-fluorouracil with the addition of cisplatin or mitomycin C
- Surgery is possible in 97% of patients after this treatment; 50% of patients will have no residual disease.
In the event that surgery is not possible, the chemoradiation course is extended (total dose 60 Gy to primary and equivalent 50 Gy to microscopic disease) which is considered a radical dose.
Metastatic Disease (IVB)
Metastatic disease is uncommon but has a terrible prognosis. There is limited activity of cytotoxic agents in this disease. Most regimens are similar to adjuvant therapies (5-fluorouracil, cisplatin, mitomycin C).
