review Wien Med Wochenschr DOI 10.1007/s10354-015-0354-9
Vulvar cancer: a review for dermatologists Anastasiya Atanasova Chokoeva · Georgi Tchernev · Elena Castelli · Elisabetta Orlando · Shyam B. Verma · Markus Grebe · Uwe Wollina
Received: 18 March 2015 / Accepted: 7 April 2015 © Springer-Verlag Wien 2015
Summary Vulvar malignancies are important tumors of the female reproductive system. They represent a serious health issue with an incidence between 2 and 7 per 100,000 and year. We provide a review about most important cancer entities, i.e., melanoma, squamous cell carcinoma, basal cell carcinoma, neuroendocrine cancer, and skin adnexal malignancies. Squamous cell carcinoma is the most common vulvar malignancy that can develop from vulvar intraepithelial neoplasia or de novo. Basal cell carcinoma represents only 2 % of all vulvar cancers. Melanoma of the vulva exists in two major types—superficial spreading and acral lentiginous. A special feature is the occurrence of multiple vulvar melanomas. Of the adnexal cancer types Paget’s disease and carcinoma are seen more frequently than other adnexal malignancies. The dermatologist
Prof U. Wollina, MD () Department of Dermatology and Allergology, Academic Teaching Hospital Dresden-Friedrichstadt, Friedrichstrasse 41, 01067 Dresden, Germany e-mail: [email protected] A. A. Chokoeva, MD Onkoderma - Policlinic for Dermatology and Dermatologic Surgery, General Skobelev Nr 26, 1407 Sofia, Bulgaria e-mail: [email protected] Prof G. Tchernev, MD Medical Faculty, University Hospital Lozenetz, Policlinic for Dermatology and Venerology, Saint Kliment Ohridski University, Koziak street 1, 1407 Sofia, Bulgaria e-mail: [email protected] E. Castelli, MD Department of Biomedicine, Internal Medicine,and Specialized Medicin (DIBIMIS), Section Dermatology, University of Palermo/
13
should be aware of this problem, since he might be the first to be consulted by patients for vulvar disease. Treatment should be interdisciplinary in close association to gynecologists, oncologists, and radiologists. Keywords Vulva · Squamous cell carcinoma · Basal cell carcinoma · Melanoma · Paget’s disease · Adnexal carcinomas
Maligne Tumore der Vulva: eine Übersicht für den Dermatologen Zusammenfassung Maligne Tumore der Vulva stellen wichtige Neoplasien des weiblichen Reproduktionssystems dar. Sie stellen ein erhebliches Gesundheitsprob-
AOUP Paolo Giaccone, Via del Vespro 131, 90127 Palermo, PR, Italy e-mail: [email protected] E. Orlando, MD Department of Sciences for Health Promotion and Mother and Child Care “Giuseppe D’Alessandro” – Section of Pathology, AOUP Paolo Giaccone, Via del vespro 129, 90127 Palermo, PR, Italy e-mail: [email protected] S. B. Verma, MBBS, DV & D 18 Amee Society, Near Rajnigandha Apartments, Diwalipura, Vadodara Gujarat, 390 015, India e-mail: [email protected] M. Grebe, MD Department of Gynecology, Academic Teaching Hospital DresdenFriedrichstadt, Friedrichstrasse 41, 01067 Dresden, Germany e-mail: [email protected]
Vulvar cancer: a review for dermatologists
1
review
lem dar bei einer Inzidenz zwischen 2 und 7 pro 100,000 und Jahr. Wir berichten in dieser Übersicht über die wichtigsten Krebsentitäten: das Melanom, das Plattenepithelkarzinom, das Basalzellkarzinom, neuroendokrine Malignome und Tumoren der Hautadnexe. Das Plattenepithelkarzinom stellt die häufigste VulvaNeoplasie dar. Es kann sich aus einer vulvären intraepithelialen Neoplasie oder de novo entwickeln. Basalzellkarzinome repräsentieren nur 2 % aller Vulvakarzinome. Das Melanom der Vulva tritt in zwei Haupttypen auf - dem superfiziell spreitenden und dem akrolentiginösen Typ. Eine Besonderheit ist das Vorkommen multipler Vulva-Melanome. Unter den adnexalen Tumoren sind der Morbus Paget und das pagekt-Karzinom häufiger als andere Tumore. Der Dermatologe sollte über diesen Problemkreis Bescheid wissen, da er der erste vom Patienten wegen Vulva-Erkrankungen angesprochene Arzt sein kann. Die Behandlung sollte interdisziplinär in enger Kooperation mit Gynäkologen, Onkologen und Radiologen erfolgen. Schlüsselwörter Vulva · Plattenepithelkarzinom · Basalzellkarzinom · Melanom · Morbus Paget · Hautadnextumore
Introduction Malignant tumors of the female reproductive system are a serious health issue as they are the second cause of death among women, after breast cancer [1]. Their incidence has increased dramatically during the past few years, with significant variability in different geographic areas, reaching close to 32 % in Africa and 13 % in North America [1, 2] This is likely due to differences in socioeconomic status of the countries, health care, and the integration of screening programs, sexual habits, and the degree of HIV/AIDS and human papilloma virus (HPV) carriers [1, 3]. Vulvar tumors constitute only 4 % of all gynecological neoplasms [3]. The overall incidence of the tumors with vulvar location is between 2 and 7 cases per 100,000 women, but it increases with age [2, 4]. Changes of classification of disease also influence their incidence as for instance in vulvar intraepithelial neoplasia (VIN), whose incidence has increased dramatically since 1970 [3, 4]. In US National Cancer Institute, vulvar carcinoma is among the 12 malignant tumors, whose incidence has increased rapidly in the last few years [1]. Incidence of both carcinoma of the vulva and VIN is increasing on average by 2.4 % every year. The mortality rate in Germany has been estimated as 0.8 per 100,000 women for 2010 [2]. The most common vulvar cancer is squamous cell carcinoma (SCC) (90 %) followed by malignant melanoma (MM) ( 1.0 mm, confined to the vulva or perineum, with negative nodes
Stage II
Tumor of any size with extension to adjacent perineal structures (1/3 lower urethra, 1/3 lower vagina, anus) with negative nodes
Stage III
Tumor of any size with or without extension to adjacent perineal structures (1/3 lower urethra, 1/3 lower vagina, anus) with positive inguinofemoral lymph nodes
IIIA
(i) With 1 lymph node metastasis (≥ 5 mm), or (ii) 1–2 lymph node metastasis(es) (
Vulvar cancer: a review for dermatologists Anastasiya Atanasova Chokoeva · Georgi Tchernev · Elena Castelli · Elisabetta Orlando · Shyam B. Verma · Markus Grebe · Uwe Wollina
Received: 18 March 2015 / Accepted: 7 April 2015 © Springer-Verlag Wien 2015
Summary Vulvar malignancies are important tumors of the female reproductive system. They represent a serious health issue with an incidence between 2 and 7 per 100,000 and year. We provide a review about most important cancer entities, i.e., melanoma, squamous cell carcinoma, basal cell carcinoma, neuroendocrine cancer, and skin adnexal malignancies. Squamous cell carcinoma is the most common vulvar malignancy that can develop from vulvar intraepithelial neoplasia or de novo. Basal cell carcinoma represents only 2 % of all vulvar cancers. Melanoma of the vulva exists in two major types—superficial spreading and acral lentiginous. A special feature is the occurrence of multiple vulvar melanomas. Of the adnexal cancer types Paget’s disease and carcinoma are seen more frequently than other adnexal malignancies. The dermatologist
Prof U. Wollina, MD () Department of Dermatology and Allergology, Academic Teaching Hospital Dresden-Friedrichstadt, Friedrichstrasse 41, 01067 Dresden, Germany e-mail: [email protected] A. A. Chokoeva, MD Onkoderma - Policlinic for Dermatology and Dermatologic Surgery, General Skobelev Nr 26, 1407 Sofia, Bulgaria e-mail: [email protected] Prof G. Tchernev, MD Medical Faculty, University Hospital Lozenetz, Policlinic for Dermatology and Venerology, Saint Kliment Ohridski University, Koziak street 1, 1407 Sofia, Bulgaria e-mail: [email protected] E. Castelli, MD Department of Biomedicine, Internal Medicine,and Specialized Medicin (DIBIMIS), Section Dermatology, University of Palermo/
13
should be aware of this problem, since he might be the first to be consulted by patients for vulvar disease. Treatment should be interdisciplinary in close association to gynecologists, oncologists, and radiologists. Keywords Vulva · Squamous cell carcinoma · Basal cell carcinoma · Melanoma · Paget’s disease · Adnexal carcinomas
Maligne Tumore der Vulva: eine Übersicht für den Dermatologen Zusammenfassung Maligne Tumore der Vulva stellen wichtige Neoplasien des weiblichen Reproduktionssystems dar. Sie stellen ein erhebliches Gesundheitsprob-
AOUP Paolo Giaccone, Via del Vespro 131, 90127 Palermo, PR, Italy e-mail: [email protected] E. Orlando, MD Department of Sciences for Health Promotion and Mother and Child Care “Giuseppe D’Alessandro” – Section of Pathology, AOUP Paolo Giaccone, Via del vespro 129, 90127 Palermo, PR, Italy e-mail: [email protected] S. B. Verma, MBBS, DV & D 18 Amee Society, Near Rajnigandha Apartments, Diwalipura, Vadodara Gujarat, 390 015, India e-mail: [email protected] M. Grebe, MD Department of Gynecology, Academic Teaching Hospital DresdenFriedrichstadt, Friedrichstrasse 41, 01067 Dresden, Germany e-mail: [email protected]
Vulvar cancer: a review for dermatologists
1
review
lem dar bei einer Inzidenz zwischen 2 und 7 pro 100,000 und Jahr. Wir berichten in dieser Übersicht über die wichtigsten Krebsentitäten: das Melanom, das Plattenepithelkarzinom, das Basalzellkarzinom, neuroendokrine Malignome und Tumoren der Hautadnexe. Das Plattenepithelkarzinom stellt die häufigste VulvaNeoplasie dar. Es kann sich aus einer vulvären intraepithelialen Neoplasie oder de novo entwickeln. Basalzellkarzinome repräsentieren nur 2 % aller Vulvakarzinome. Das Melanom der Vulva tritt in zwei Haupttypen auf - dem superfiziell spreitenden und dem akrolentiginösen Typ. Eine Besonderheit ist das Vorkommen multipler Vulva-Melanome. Unter den adnexalen Tumoren sind der Morbus Paget und das pagekt-Karzinom häufiger als andere Tumore. Der Dermatologe sollte über diesen Problemkreis Bescheid wissen, da er der erste vom Patienten wegen Vulva-Erkrankungen angesprochene Arzt sein kann. Die Behandlung sollte interdisziplinär in enger Kooperation mit Gynäkologen, Onkologen und Radiologen erfolgen. Schlüsselwörter Vulva · Plattenepithelkarzinom · Basalzellkarzinom · Melanom · Morbus Paget · Hautadnextumore
Introduction Malignant tumors of the female reproductive system are a serious health issue as they are the second cause of death among women, after breast cancer [1]. Their incidence has increased dramatically during the past few years, with significant variability in different geographic areas, reaching close to 32 % in Africa and 13 % in North America [1, 2] This is likely due to differences in socioeconomic status of the countries, health care, and the integration of screening programs, sexual habits, and the degree of HIV/AIDS and human papilloma virus (HPV) carriers [1, 3]. Vulvar tumors constitute only 4 % of all gynecological neoplasms [3]. The overall incidence of the tumors with vulvar location is between 2 and 7 cases per 100,000 women, but it increases with age [2, 4]. Changes of classification of disease also influence their incidence as for instance in vulvar intraepithelial neoplasia (VIN), whose incidence has increased dramatically since 1970 [3, 4]. In US National Cancer Institute, vulvar carcinoma is among the 12 malignant tumors, whose incidence has increased rapidly in the last few years [1]. Incidence of both carcinoma of the vulva and VIN is increasing on average by 2.4 % every year. The mortality rate in Germany has been estimated as 0.8 per 100,000 women for 2010 [2]. The most common vulvar cancer is squamous cell carcinoma (SCC) (90 %) followed by malignant melanoma (MM) ( 1.0 mm, confined to the vulva or perineum, with negative nodes
Stage II
Tumor of any size with extension to adjacent perineal structures (1/3 lower urethra, 1/3 lower vagina, anus) with negative nodes
Stage III
Tumor of any size with or without extension to adjacent perineal structures (1/3 lower urethra, 1/3 lower vagina, anus) with positive inguinofemoral lymph nodes
IIIA
(i) With 1 lymph node metastasis (≥ 5 mm), or (ii) 1–2 lymph node metastasis(es) (
