Surgery and radiotherapy in vulvar cancer
Introduction
Vulvar cancer accounts for approximately 3–5% of all gynaecological malignancies and 1% of all cancers in women, with in incidence rate of 1–2/100,000 [1]. In the Netherlands (16 million inhabitants) about 230 new patients with vulvar cancer are diagnosed [2]. Typically these cancers occur in the seventh decade when comorbidity is common [3]. A rise in absolute numbers of vulvar cancer is expected because of the proportional increase in the average age of the population.
The most prominent presenting symptom of vulvar cancer is localized pruritus. Other common symptoms are a vulvar mass, bleeding, pain, discharge or urinary tract symptoms [4], [5]. Vulvar complaints are often noted many years before malignant changes are documented and often investigated only after trials of various medical regimens have been unsuccessful. In more than 50% of patients there is a (patient's and doctor's) delay of more than a year before the diagnosis is established [4], [6].
About 90% of vulvar cancers are squamous cell carcinomas (SCC) and the remaining 10% comprise an interesting variety of tumours ranging from malignant melanoma to adenocarcinoma of Bartholin's gland and Paget's disease [7]. The literature published on these tumours consists of case reports, retrospective series and review articles. Therefore, in this paper we will focus on SCC of the vulva. See Fig. 1 for examples of SCC of the vulva.
The pattern of dissemination of SCC of the vulva is predominantly lymphogenic, while spread by direct extension may occur but is less frequent compared to inguinofemoral lymph node metastases. Hematogenous spread is very rare, especially in absence of lymph node metastases [1]. The most important prognostic factor is the inguinofemoral lymph node status. Therefore, the lymph node status is an important factor in the surgicopathological staging according to The International Federation of Gynecology and Obstetrics (FIGO), which has been introduced in 1995 [8] (see Table 1). Five-year survival decreases from 90% with uninvolved lymph nodes to 75% with one or two lymph node metastases to 24% when five or six lymph nodes are involved [9].
In this review an outline is given on the surgical standard of care in SCC of the vulva and new developments with emphasis on the sentinel lymph node (SLN) procedure. Moreover, the role of radiotherapy and the treatment of advanced vulvar cancer will be discussed. The source of information that was used is MESH/pubmed with different combinations of keywords vulvar cancer, lymph node, sentinel, radiotherapy, advanced, morbidity, CT, MRI, PET, ultrasound, learning curve, breast cancer, cutaneous melanoma, ultrastaging. Gynaecologic oncology handbooks were used and manual checking of reference lists for relevant publications was performed.
Section snippets
Surgical treatment
Surgery is the cornerstone in the treatment of most patients with vulvar cancer.
Morbidity of surgery
Morbidity of the current standard surgical treatment (wide local excision and uni- or bilateral inguinofemoral lymphadenectomy via separate incisions) has decreased compared to the “en bloc” technique but remains significant: frequent wound breakdown, wound infections, lymphocysts, lymphoedema and impressive psychosexual consequences, especially in younger patients [4], [13].
Why?
At present there are no accurate non-invasive techniques available for the detection of inguinofemoral lymph node metastases [56], [57]. Palpation of the groins is notoriously inaccurate [4], while the results of ultrasound [58], [59] and positron emission tomography [60] in predicting the lymph node status in patients with vulvar cancer are disappointing. No literature is available on the diagnostic value of CT. MRI has some advantages for evaluation of the lymph nodes. The use of intravenous
Radiotherapy
There are different indications for radiotherapy in vulvar cancer patients. The role of radiotherapy in advanced vulvar cancer will be discussed in Section 6.
Advanced vulvar cancer
Patients with T3/T4 tumours are characterized by local extension resulting in serious local problems such as pain while sitting, discharge, bleeding from necrotic tumour and a foul odor. Positive lymph nodes are found in 50–60% of patients with locally advanced disease. Frequently these nodes are ulcerating and or fixed to the femoral vessels in the groin. See Fig. 12, Fig. 13 for an advanced vulvar cancer with ulcerating fixed lymph nodes in the groin. The 30% of vulvar cancer patients
Conclusion
In general, patients with squamous cell carcinomas of the vulva have a good prognosis. Standard treatment for early stage SCC is wide local excision with uni- and bilateral lymphadenectomy via separate incisions. Despite an increase in individualization of treatment, the morbidity of this surgery is still impressive. There is growing interest in the sentinel lymph node procedure and primary radiotherapy of the groins as attractive alternatives for elective inguinofemoral lymphadenectomy.
Reviewers
Dr. J. van der Velden, Department of Gynaecology, Academic Medical Center, Meibergdreef 9, NL-1105 AZ Amsterdam, The Netherlands.
Prof. Neville F. Hacker, Gynaecological Cancer Center, Royal Hospital for Women, Locked Bag 2000, Barker Street, Randwick, NSW 2031, Australia.
Joanne A. de Hullu attended Medical School at the State University in Groningen, The Netherlands. After her training as gynaecologist in 1998, she followed a fellowship in gynecologic oncology at the Department of Gynecologic Oncology of the University Medical Centre Groningen, the Netherlands and the Royal Adelaide Hospital, Australia (Dr. M. Davy). In 2002 she succesfully defended her thesis “Innovations in treatment of vulvar cancer”. Until 2003 she worked as a gynecologic oncologist at the
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2019, Journal of the American Academy of Dermatology
Joanne A. de Hullu attended Medical School at the State University in Groningen, The Netherlands. After her training as gynaecologist in 1998, she followed a fellowship in gynecologic oncology at the Department of Gynecologic Oncology of the University Medical Centre Groningen, the Netherlands and the Royal Adelaide Hospital, Australia (Dr. M. Davy). In 2002 she succesfully defended her thesis “Innovations in treatment of vulvar cancer”. Until 2003 she worked as a gynecologic oncologist at the Department of Gynecologic Oncology of the University Medical Centre Groningen. From 2003 onwards, she is working as a gynecologic oncologist at the Department of Gynecologic Oncology of the Radboud University, Nijmegen Medical Centre. She has published over 20 articles in peer-reviewed journals. Her fields of interest are vulvar cancer, vulvar premalignancies and heriditary cancer.
Ate G.J. van der Zee attended Medical School at the State University of Groningen, The Netherlands (cum laude). After his training as gynaecologist he worked as a scientist in the Beatson Laboratories, Glasgow, UK, (1992) and successfully defended his thesis “Translational research in ovarian carcinoma: cell biological aspects of drug resistance and tumor aggressiveness” (cum laude) in 1994. He was trained as gynecologic oncologist in the Department of Gynecologic Oncology (Dr. A. Covens) in Toronto Sunnybrook Regional Cancer Center, Toronto, Canada (1995). He currently is Head of the Department of Obstetrics and Gynecology, University Medical Center Groningen and professor in gynecologic oncology at the State University of Groningen, The Netherlands. He also is Chairman of the Dutch Society of Gynecologic Oncology and council member of the European Society of Gynecologic Oncology. van der Zee published over 120 articles in peer-reviewed journals.
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