Surgery and radiotherapy in vulvar cancer

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Abstract

The majority of patients with vulvar cancer have squamous cell carcinomas (SCC). The cornerstone of the treatment is surgery. Radical vulvectomy with “en bloc” inguinofemoral lymphadenectomy has led to a favorable prognosis but with impressive morbidity. Nowadays, treatment is more individualized with wide local excision with uni- or bilateral inguinofemoral lymphadenectomy via separate incisions as the standard treatment for early stage patients with SCC of the vulva with depth of invasion >1 mm without suspicious groins. In case of more than one intranodal lymph node metastasis and/or extranodal growth, postoperative radiotherapy on the groins and pelvis is warranted. Until now there is a limited role for primary radiotherapy on the vulva and/or groins in early stage disease.

The sentinel lymph node (SLN) procedure with the combined technique (preoperative lymphoscintigraphy with a radioactive tracer and intraoperative blue dye) is a promising staging technique for patients with early stage vulvar cancer. The safety of clinical implementation of the SLN procedure and the role of additional histopathological techniques of the SLNs need to be further investigated before its wide-scale application.

Patients with advanced vulvar cancer are difficult to treat. One of the problems in patients with locally advanced vulvar cancer is the high incidence of concomitant bulky lymph nodes in the groin(s). Ultraradical surgery in case of resectable disease will lead to impressive morbidity because of the exenterative-type procedure. (Chemo)radiation with or without surgery should be regarded as the first choice for patients with locally advanced vulvar cancer only when primary surgery will necessitate performance of a stoma. Further studies are needed to determine the optimal combined modality treatment in these patients.

Due to the fact that vulvar cancer is a rare disease, further clinical studies will only be possible, when international collaborative groups will join forces in order to perform clinical trials, in which different treatment options such as SLN procedure, primary radiotherapy on the groins and multimodality treatment for advanced disease will be investigated.

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

References (156)

  • M.J. Schulz et al.

    Recurrent vulvar carcinoma in the intervening tissue bridge in early invasive stage I disease treated by radical vulvectomy and bilateral groin dissection through separate incisions

    Gynecol Oncol

    (1989)
  • W. Christopherson et al.

    Radical vulvectomy and bilateral groin lymphadenectomy utilizing separate groin incisions: report of a case with recurrence in the intervening skin bridge

    Gynecol Oncol

    (1985)
  • M.P. Burger et al.

    The importance of the groin node status for the survival of T1 and T2 vulval carcinoma patients

    Gynecol Oncol

    (1995)
  • M. Arvas et al.

    Radical versus conservative surgery for vulvar carcinoma

    Int J Gynecol Obstet

    (2005)
  • T.W. Burke et al.

    Surgical therapy of T1 and T2 vulvar carcinoma: further experience with radical wide excision and selective inguinal lymphadenectomy

    Gynecol Oncol

    (1995)
  • R. Farias-Eisner et al.

    Conservative and individualized surgery for early squamous carcinoma of the vulva: the treatment of choice for stage I and II (T1–2N0–1M0) disease

    Gynecol Oncol

    (1994)
  • J.M. Heaps et al.

    Surgical–pathologic variables predictive of local recurrence in squamous cell carcinoma of the vulva

    Gynecol Oncol

    (1990)
  • J.A. de Hullu et al.

    Groin surgery and the sentinel lymph node

    Best Pract Res Clin Obstet Gynaecol

    (2003)
  • J.Y. Lin et al.

    Morbidity and recurrecne with modifications of radical vulvectomy and groin dissection

    Gynecol Oncol

    (1992)
  • P.J. Paley et al.

    The effect of sartorius transposition on wound morbidity following inguinal–femoral lymphadenectomy

    Gynecol Oncol

    (1997)
  • R. Rouzier et al.

    Inguinofemoral dissection for carcinoma of the vulva: effect of modifications of extent and technique on morbidity and survival

    J Am Coll Surg

    (2003)
  • P.L. Judson et al.

    A prospective, randomized study analyzing sartorius transposition following inguinal–femoral lymphadenectomy

    Gynecol Oncol

    (2004)
  • C. Levenback et al.

    Groin dissection practices among gynecologic oncologists treating early vulvar cancer

    Gynecol Oncol

    (1996)
  • D.P. Barton

    The prevention and management of treatment related morbidity in vulval cancer

    Best Pract Res Clin Obstet Gynaecol

    (2003)
  • M. Janda et al.

    The functional assessment of cancer-vulvar: reliability and validity

    Gynecol Oncol

    (2005)
  • N. Gould et al.

    Predictors of complications after inguinal lymphadenectomy

    Gynecol Oncol

    (2001)
  • C.W. Helm et al.

    A matched comparison of single and triple incision techniques for the surgical treatment of carcinoma of the vulva

    Gynecol Oncol

    (1992)
  • T.J. Selman et al.

    A systematic review of the accuracy of diagnostic tests for inguinal lymph node status in vulvar cancer

    Gynecol Oncol

    (2005)
  • D.K. Abang Mohammed et al.

    Inguinal node status by ultrasound in vulvar cancer

    Gynecol Oncol

    (2000)
  • D.E. Cohn et al.

    Prospective evaluation of positron emission tomography for the detection of groin node metastases from vulvar cancer

    Gynecol Oncol

    (2002)
  • S.A. Sohaib et al.

    Imaging in vulval cancer

    Best Pract Res Clin Obstet Gynaecol

    (2003)
  • J.F. Thompson et al.

    Cutaneous melanoma

    Lancet

    (2005)
  • J.M. Thomas et al.

    Selective lymphadenectomy in sentinel node-positive patients may increase the risk of local/in-transit recurrence in malignant melanoma

    Eur J Surg Oncol

    (2004)
  • U. Veronesi et al.

    Sentinel node biopsy in breast cancer: early results in 953 pateints with negative sentinel node biopsy and no axillary dissection

    Eur J Cancer

    (2005)
  • E.J.Th. Rutgers

    Guidelines to assure quality in breast cancer surgery

    EJSO

    (2005)
  • C. Levenback et al.

    Intraoperative lymphatic mapping and sentinel node identification with blue dye in patients with vulvar cancer

    Gynecol Oncol

    (2001)
  • Z. Radovanovic et al.

    Blue dye versus combined blue dye-radioactive tracer technique in detection of sentinel lymph node in breast cancer

    Eur J Surg Oncol

    (2004)
  • E.E. Sanidas et al.

    How many cases are enough for accreditation in sentinel lymph node biopsy in breast cancer?

    Am J Surg

    (2003)
  • E.E. Sanidas et al.

    Modifactions of the learning curve guidelines for breast cancer sentinel node biopsy

    EJSO

    (2005)
  • R.G. Moore et al.

    Sentinel node identification and the ability to detect metastastic tumor to inguinal lymph nodes in vulvar malignancies

    Gynecol Oncol

    (2003)
  • L.M. Puig-Tintoré et al.

    Further data on the usefulness of sentinel lymph node identification and ultrastaging in vulvar squamous cell carcinoma

    Gynecol Oncol

    (2003)
  • J. Chu et al.

    Femoral node metastases with negative superficial inguinal nodes in early vulvar cancer

    Am J Obstet Gynecol

    (1981)
  • C. Levenback et al.

    Potential applications of intraoperative lymphatic mapping in vulvar cancer

    Gynecol Oncol

    (1995)
  • S.L. DeCesare et al.

    A pilot study utilizing intraoperative lymphoscintigraphy for identification of the sentinel lymph nodes in vulvar cancer

    Gynecol Oncol

    (1997)
  • N.F. Hacker

    Vulvar cancer

  • O. Visser et al.

    Incidence of cancer in the Netherlands 1993

    (1996)
  • K.C. Podratz et al.

    Carcinoma of the vulva: analysis of treatment and survival

    Obstet Gynecol

    (1983)
  • J.H. Shepherd

    Staging announcement. FIGO staging of gynecologic cancers; cervical and vulva

    Int J Gynecol Cancer

    (1995)
  • B.L. Andersen et al.

    Psychosexual adjustment after vulvar surgery

    Obstet Gynecol

    (1983)
  • N.F. Hacker et al.

    Management of regional lymph nodes and their prognostic influence in vulvar cancer

    Obstet Gynecol

    (1983)
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    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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