- Email: [email protected]
Basal cell carcinoma of the vulva: Clinical features and treatment results in 28 patients
Basal Cell Carcinoma of the Vulva: Clinical Features and Treatment Results in 28 Patients J. L. BENEDET, MD, D. M. MILLER,
MD, T. G. EHLEN, MD, AND M. A. BERTRAND,
Objective: To review our experience and that in the recent literature regarding basal cell carcinoma of the vulva to see whether current management guidelines are appropriate. Methods: Twenty-eight women with basal cell carcinoma of the vulva were seen over 25 years at the BC Cancer Agency. The clinical-pathologic features were tabulated and the outcome was analyzed. Results: The mean age was 74 years, and almost two-thirds were over the age of 70 at diagnosis. Patients typically presented with an irritation or soreness, with a symptom duration ranging from a few months to several years. Most lesions were confined to the anterior half of the vulva, and 23 of the 28 patients had Tl lesions. Wide local excision was the treatment method used most commonly. Only one patient was known to have died from disease metastasis. Ten women had other basal cell carcinomas, either before or after the diagnosis of their vulvar lesions, and in ten patients 11 other malignancies were diagnosed. Conclusion: Basal cell carcinoma of the vulva is an extremely uncommon tumor that rarely metastasizes or spreads. Primary treatment should consist of wide local excision and continued follow-up. (Obstet Gynecol 1997;90: 765-S. 0 1997 by The American College of Obstetricians and Gynecologists.)
conservative surgical ported by these data.
approach
to treatrnent
MD is sup-
Materials and Methods Records at the BC Cancer Agency, Vancouver Cancer Centre between 1970 and 1996 were examined to provide a listing of all patients seen with basal cell carcinoma of the vulva. There were 28 patients,. all of whom were assessed and managed by the medical staff of the Division of Gynecologic Oncology. PathLology specimens for all patients were reviewed by the Cancer Agency pathologists. The records were -reviewed for demographic, clinical, pathologic, treatment, and outcome information for analysis. A MEDLIVE literature search was performed for reports on basal cell carcinoma of the vulva between the years 1976 and 1997 to see if there were any substantive differences in the clinical features, methods of management, and frequency of metastasis from the material in this series.
Results Basal cell carcinoma of the vulva is an uncommon vulvar tumor accounting for approximately 2-3s of all vulvar cancers, in contrast to 60-65s of all cutaneous malignancies that are basal cell cancers. Temesvaryi is generally credited as being the first to describe basal cell carcinoma of the vulva in 1926. Most of the literature on this topic consists of sporadic case reports2-h documenting some particular feature or unusual occurrence, with few reports7-lo containing sizable numbers of cases from a single institution. The purpose of this study is to report our experience with the clinical features and management of this uncommon tumor over a 25-year period and to see whether the currently recommended From the Depurtment qf Obstetrics and Gynecology, University of British Columbia, and the Division of Gynecologic Oncology, BC Cnncer Agency, Vancower, British Columbia, Canada
VOL.90,NO.5,NOVEMBER
1997
The ages of the patients in this study ranged from 44 to 95 years, with a mean of 73.9. Eighteen of the 28 women were over age 70 at the time of diagnosis, ,and only two were 60 or younger. Fourteen patients were gravida 2 or less, and six others were gravida 6 or greater. Similarly, eight patients were nulliparous and 11 patients were either para 1 or 2. Five patients were known to be grand multiparas. An irritation or “soreness” was the most commonly documented presenting complaint, reported by 12 women. Pruritis was noted in seven cases and was the next most common symptom. In four elderly women, specific information regarding s’ymptomatology and duration was not available because of the confused mental state of the patients when first seen. The duration of symptoms varied tremendously and ranged from a few months to several years. Seven patients had symptoms for less than 1 year and 14 had
0029.7844/97/$17.00 PI1 50029.7844(~'7)00416-X
765
complaints of 1 year or longer; in seven instances an accurate assessment of symptom duration was not possible. Twenty-one women were nonsmokers and four gave a history of smoking; smoking status was not available for three patients. The records did not provide information regarding possible prior use of arsenicals and no patient had a history of syphilis, two conditions that were mentioned in the older literature as possible etiologic agents for basal cell carcinoma of the vulva. One patient had had irradiation to the pelvis for a cervical carcinoma many years before the diagnosis of basal cell cancer of the vulva. An additional patient had had irradiation to both legs because of a problem of excessive hair growth. These were the only two women who had had some form of ionizing radiatioln that may have affected the vulvar area. Most lesions were on the anterior half of the vulva, with each side being involved equally. Five patients had lesions involving the skin of the upper mons remote from the clitoral or labial attachments. Non-hairbearing skin or epithelium of the central vulvar structures was the site least commonly involved, with only three patients affected. Twenty-three women had Tl lesions measuring 2 cm or less and five patients had T2 lesions larger than 2 cm. No patient had clinically suspicious groin nodes when first seen. Various treatment methods were used. One patient, aged 95 years, was confused and infirm when first seen and had a small lesion that was biopsied only. She received no further treatment but survived 6 more years; she died at age 101 of other causes, with vulvar disease still present. Observation of this patient revealed a slow enlargement of her lesion, which gradually doubled in size over the 6 years. Twenty-one women had wide local excision as their primary treatment, with disease extending to a resection margin on microscopic evaluation in 10 cases. Two of these patients with positive margins had re-excision, and neither had residual disease found. Seven women with involved surgical margins have been followed for 2-24 years, with no recurrence to date. Three patients were treated with simple vulvectomy, and another three patients had a radical vulvectomy and node dissections. The initial biopsies of two of these women revealed invasive squamous cell carcinoma, and after radical vulvectomy and node dissection, pathologic examination revealed concomitant distinct basal cell cancers as well. In one woman, further review suggested that the initial diagnosis of squamous cell carcinoma was incorrect and that only a basal cell carcinoma was present. The third of the group was a woman whose initial biopsy revealed a basosquamous cancer with an aggressive histologic pattern.
766
Benedet et al
Vuln7r Basal Cell Cancer
Three women in this series developed a recurrence, two at 2 years and one at 6 years. All had been treated initially with wide local excision in which the margins were positive. Two of these patients were retreated with another wide local excision, with negative margins, and both are alive and well. Rtbview of the histologic sections in these cases failed to reveal any histologic features that might have predicted recurrence other than the positive margins. Lymph-vascular space invasion by basal cell carcinoma was not evident in these patients. The third patient was treated initially with wide local excision and had immediate re-excision because of a positive margin. This second specimen had negative resection margins. She was well for 2 years, then appeared with a suspicious node in the left supraclavicular fossa. Histologic examination of this neck node showed metastatic disease with a histologic picture similar to that of her original vulvar lesion. At that time there was no evidence of any nodal disease either in the groin or on the vulva itself, but 2 ye,ars later she developed a lesion on the opposite side of the vulva, which biopsy showed to be basal cell carcinoma. The supraclavicular nodal metastasis was treated with irradiation, with apparently complete resolution. This woman died of renal failure 2 years later; her death was thought to be due to pelvic and abdominal disease, but no autopsy was done. This is the only death attributable to disease in this series. Currently, 14 patients are alive and well without evidence of disease. Thirteen others died of other causes; one of these had disease present at the time of her death (the patient who received no :specific treatment). No patients have been lost to follow-up. Twentyone of the 28 patients were followed for more than 5 years and in six women, the length of follow-up was 11-26 years. In only seven patients was the length of follow-up less than 5 years. Ten patients had other basal cell carcinomas on the face, trunk, or extremities. In four patients these lesions preceded the diagnosis of their vulvar can’cer, and in six women these lesions were diagnosed after the vulvar basal cell cancers. In ten patients, 11 other malignancies were diagnosed: One patient each had ovary, cervix, lung, breast, lymphoma, and squamous carcinoma of the vulva; three patients had colon cancers; and one patient had both colon and breast cancers. In all, 11 patients (39.3%) had another lesion, either basal cell or some other tumor.
Discussion This series confirms vulva is uncommon
that basal cell carcinoma of the and predominantly a tumor of
Obstetrii-s
& Chjnecolo~~y
Table
1. Metastatic
Basal
Cell
Carcinoma
of the Vulva
Size of primary (cm)
Year of report
Age (y)
Jimenez7 Perrone”
1975 1987
41 86
5x5 “Large”
HOffnIan’3
1988
74
6X4
Sworn” Winkelmanns
1979 1990
71 71
8 x 4.5 6 X 15
1994 1997
87 80
Principal
author
Gleesor? Benedet (current
study)
2.5 X 4 2.0 x 1
Pathologic
features
Site of metastasis
Invasion to subcutis Invasion to vagina, urethra, subcutis Invasion of subcutis; two discrete primary lesions Invasion of subcutis Necrosis to depth of symphysis pubis Shallow ulcer only Deep invasion to subcutis; oerineural invasion
Groin Bilateral inguinal 5 left 2/12 left inguinal 5/1.5 inguinal Groin Groin Supraclavicular
nodes:
Outconle 3 right,
nodes
NS Alive
and well,
14 mo
Alive .and well Died at 2 y
at 1 y
NS
nodes
NS NS
node
NS = not stated
elderly women. As with many vulvar lesions, patients most commonly present with an irritation or soreness or palpation of a nodule or a mass on the vulva, which then leads to the diagnosis. In this group of patients, as has been noted in other reports,‘,*,” an appreciable proportion had a substantial delay in presenting for assessment and diagnosis. The information available does not provide any insights or explanations as to the possible etiology of these lesions. Arsenicals, previous irradiation, syphilis, and chronic irritation have all been suggested as possible etiologic or precipitating causes. Although basal cell carcinoma of the face and extremities often has been linked to ultraviolet irradiation and sunlight, this does not appear to be a plausible explanation for vulvar lesions. It is interesting to note, however, that a large proportion of individuals in this series had basal cell carcinoma involving the face, trunk, or extremities. For the most part, these lesions behave in an indolent fashion and have a low propensity for lymphatic or distant spread. A MEDLINE search identified only six reports of metastatic basal cell carcinoma of the vulva. Jiminez et al” reported the first case of vulvar basal cell
Table
carcinoma metastatic to groin nodes in 1975. Since then, five additional cases have been reported.s,“,‘‘-‘a Table 1 summarizes the reported cases of metastatic basal cell carcinoma of the vulva. Six patients had deep invasion to the subcutaneous layer and a similar number had large tumors more than 2 cm in diameter. All of these patients had at least one of these adverse features, and five had both. It appears that lesions that do metastasize generally are larger in size and volume and often have aggressive-appearing or poorly differentiated histologic patterns. Unfortunately, outcome data for most of these cases are lacking. Although 11 other malignancies were noted in this group of patients, this frequency is probably not greater than what might be expected from a similar group of essentially elderly women. Wide local excision is the treatment generally recommended for this disease and appears to be sufficient for the vast majority of these individuals, particularly given their age and general medical condition. Although clear surgical margins should be attempted in all cases, this may not always be possible, particularly if functional or cosmetic factors are important. The results of this review suggest that these patients can be followed safely,
2. Series of Vulvar Basal Cell Carcinomas
outcome
Principal
author
Palladino7 Cruz-JimenezX Breen’ Simonsen”’ Perrone” Benedet (current
Year of report
study)
1969 1975 1975 1985 1987 1997
Summary
Age (y) Range
Mean
Other cancers
Recurrence
Died of disease
65 11 17 21 11 28
34-90 45-75 34-83 55-90 62-86 44-95
65 59 58 76 74 74
13/65 NS 3/17 7/21 3/11 11/28
13/65 o/11 o/17 2/21 o/11 3/28
0 0 0 0 0 1
153
34-95
67.7
37/142
18/153
No. of patients
Died of other disease 19 3 2 2 1 13
Length of follow-up Survived
Lost
(Y)
39 8 14 19 10 14
7 0 1 0 0 0
4-19 l-25 l-7 2-19 l-10 l-25
NS = not stated.
VOL.
90, NO.
5, NOVEMBER
1997
Benedet
et al
Vuivar Basal Cdl Cancer
767
as the propensity for spread is extremely low and the majority do not develop further disease. Metastasis from sites other than the vulva usually has a poor prognosis, with very short survival intervals once the diagnosis is made.‘” Similar evaluations with regard to metastatic basal cell carcinoma from the vulva cannot be made from the literature currently available. Table 2 summarizes the outcome data from the larger series of vulvar basal cell cancer in the literature, indicating the generally favorable outcome for this group of elderly women. These lesions do not seem to metastasize readily to regional nodes, so routine lymphadenectomy is not warranted. Nonetheless, individuals with larger tumors or tumors showing deep invasion with lymphatic involvement should be considered for selective lymphadenectomy until more information is available regarding the behavior of this group of lesions.
R$erences 1. Temesvary Vulva, mit 50:1575-82.
N. Uber ein multiples Krompechersches ausgendehnter Elephantiasis. Zentralbl
Karzinom Gynakol
der 1926;
3. Jimenez HT, Fenoglio noma with metastasis: 1975;121:285-6.
CM, A
Richart RM. case report.
Vulvar basal Am J Obstet
Benedet
et al
vulva
with
groin
Vuhar Basal Cell Cancer
node
basal
of the vulva.
cell
carcinoma
E, Gregori CA. 1975;46:122-9.
of
Basal
cell
10. Simonsen E, Johnsson J-E, Trope C, Alm I’. Basal (cell carcinoma of the vulva. Acta Obstet Gynecol Stand 1985;64:231-4. 11. Sworn MJ, Hammond GT, Buchanan R. Metastatic basal cell carcinoma of the vulva case report. Br J Obstet Gynaecol 1979;86: 332-3. 12. Perrone T, Twiggs LB, Adcock LL, Dehner LP. Vulvar basal cell carcinoma: An infrequently Pathol 1987;6:152-65. 13. Hoffman MS, Roberts WAS,
metastasizing
vulva with inguinal 29:113-9. 14. Farmer ER, Helwig
node
pathologic
study
lymph
Ruffolo
EH. Basal metastasis.
EB. Metastatic of seventeen
neoplasm.
basal cases.
cell
Cancer
cell
Int J Gynecol carcinoma
Gynecol
of the
Oncol
carcinoma:
1988;
A clinico-
1986;46:748-57.
Address reprint requests to: J. L. Benedet, MD Departmenf of Gynecologic Oncology BC Cancer Agency 600 West 10th Avenue BC V5Z
4E6
Canada E-mail: [email protected]
cell carciGynecol
4. Simkin RJ, Fisher BK. Basal cell epithelioma of the vulva. Obstet Gynecol 1977;49:617-9. 5. Winkelmann SE, Llorens AS. Case report: Metastatic basal cell carcinoma of the vulva. Gynecol Oncol 1990;38:138--40. 6. Gleeson NC, Ruffolo EH, Hoffman MS, Cavanagh D. Case report: Basal cell carcinoma of the Gynecol Oncol 1994;53:366-8.
cell carcinoma
vulva. Cancer 1975;36:1860-8. 9. Breen JL, Neubecker RD, Greenwald carcinoma of the vulva. Obstet Gynecol
Vancouver,
2. Goldstein AI, Kent DR. All vulvar lesions should be biopsied: Basal cell carcinoma-an example of the futility of diagnosis by gross appearance. Am J Obstet Gynecol 1975;121:173-4.
768
7. Palladino VS, Duffy JL, Bures GJ. Basal Cancer 1969;24:460-70. 8. Crun-Jimenez PR, Abel1 MR. Cutaneous
metastasis.
Received May 1, 1997. Received in rezrised form ]une Accepted July 10, 1997.
Copyright Gynecologists.
0
1997 by Published
23, 1997
The American by Elsevier
College of Science Inc.
Obstetricians
and
Obstetrics & (;ynecology
MD, T. G. EHLEN, MD, AND M. A. BERTRAND,
Objective: To review our experience and that in the recent literature regarding basal cell carcinoma of the vulva to see whether current management guidelines are appropriate. Methods: Twenty-eight women with basal cell carcinoma of the vulva were seen over 25 years at the BC Cancer Agency. The clinical-pathologic features were tabulated and the outcome was analyzed. Results: The mean age was 74 years, and almost two-thirds were over the age of 70 at diagnosis. Patients typically presented with an irritation or soreness, with a symptom duration ranging from a few months to several years. Most lesions were confined to the anterior half of the vulva, and 23 of the 28 patients had Tl lesions. Wide local excision was the treatment method used most commonly. Only one patient was known to have died from disease metastasis. Ten women had other basal cell carcinomas, either before or after the diagnosis of their vulvar lesions, and in ten patients 11 other malignancies were diagnosed. Conclusion: Basal cell carcinoma of the vulva is an extremely uncommon tumor that rarely metastasizes or spreads. Primary treatment should consist of wide local excision and continued follow-up. (Obstet Gynecol 1997;90: 765-S. 0 1997 by The American College of Obstetricians and Gynecologists.)
conservative surgical ported by these data.
approach
to treatrnent
MD is sup-
Materials and Methods Records at the BC Cancer Agency, Vancouver Cancer Centre between 1970 and 1996 were examined to provide a listing of all patients seen with basal cell carcinoma of the vulva. There were 28 patients,. all of whom were assessed and managed by the medical staff of the Division of Gynecologic Oncology. PathLology specimens for all patients were reviewed by the Cancer Agency pathologists. The records were -reviewed for demographic, clinical, pathologic, treatment, and outcome information for analysis. A MEDLIVE literature search was performed for reports on basal cell carcinoma of the vulva between the years 1976 and 1997 to see if there were any substantive differences in the clinical features, methods of management, and frequency of metastasis from the material in this series.
Results Basal cell carcinoma of the vulva is an uncommon vulvar tumor accounting for approximately 2-3s of all vulvar cancers, in contrast to 60-65s of all cutaneous malignancies that are basal cell cancers. Temesvaryi is generally credited as being the first to describe basal cell carcinoma of the vulva in 1926. Most of the literature on this topic consists of sporadic case reports2-h documenting some particular feature or unusual occurrence, with few reports7-lo containing sizable numbers of cases from a single institution. The purpose of this study is to report our experience with the clinical features and management of this uncommon tumor over a 25-year period and to see whether the currently recommended From the Depurtment qf Obstetrics and Gynecology, University of British Columbia, and the Division of Gynecologic Oncology, BC Cnncer Agency, Vancower, British Columbia, Canada
VOL.90,NO.5,NOVEMBER
1997
The ages of the patients in this study ranged from 44 to 95 years, with a mean of 73.9. Eighteen of the 28 women were over age 70 at the time of diagnosis, ,and only two were 60 or younger. Fourteen patients were gravida 2 or less, and six others were gravida 6 or greater. Similarly, eight patients were nulliparous and 11 patients were either para 1 or 2. Five patients were known to be grand multiparas. An irritation or “soreness” was the most commonly documented presenting complaint, reported by 12 women. Pruritis was noted in seven cases and was the next most common symptom. In four elderly women, specific information regarding s’ymptomatology and duration was not available because of the confused mental state of the patients when first seen. The duration of symptoms varied tremendously and ranged from a few months to several years. Seven patients had symptoms for less than 1 year and 14 had
0029.7844/97/$17.00 PI1 50029.7844(~'7)00416-X
765
complaints of 1 year or longer; in seven instances an accurate assessment of symptom duration was not possible. Twenty-one women were nonsmokers and four gave a history of smoking; smoking status was not available for three patients. The records did not provide information regarding possible prior use of arsenicals and no patient had a history of syphilis, two conditions that were mentioned in the older literature as possible etiologic agents for basal cell carcinoma of the vulva. One patient had had irradiation to the pelvis for a cervical carcinoma many years before the diagnosis of basal cell cancer of the vulva. An additional patient had had irradiation to both legs because of a problem of excessive hair growth. These were the only two women who had had some form of ionizing radiatioln that may have affected the vulvar area. Most lesions were on the anterior half of the vulva, with each side being involved equally. Five patients had lesions involving the skin of the upper mons remote from the clitoral or labial attachments. Non-hairbearing skin or epithelium of the central vulvar structures was the site least commonly involved, with only three patients affected. Twenty-three women had Tl lesions measuring 2 cm or less and five patients had T2 lesions larger than 2 cm. No patient had clinically suspicious groin nodes when first seen. Various treatment methods were used. One patient, aged 95 years, was confused and infirm when first seen and had a small lesion that was biopsied only. She received no further treatment but survived 6 more years; she died at age 101 of other causes, with vulvar disease still present. Observation of this patient revealed a slow enlargement of her lesion, which gradually doubled in size over the 6 years. Twenty-one women had wide local excision as their primary treatment, with disease extending to a resection margin on microscopic evaluation in 10 cases. Two of these patients with positive margins had re-excision, and neither had residual disease found. Seven women with involved surgical margins have been followed for 2-24 years, with no recurrence to date. Three patients were treated with simple vulvectomy, and another three patients had a radical vulvectomy and node dissections. The initial biopsies of two of these women revealed invasive squamous cell carcinoma, and after radical vulvectomy and node dissection, pathologic examination revealed concomitant distinct basal cell cancers as well. In one woman, further review suggested that the initial diagnosis of squamous cell carcinoma was incorrect and that only a basal cell carcinoma was present. The third of the group was a woman whose initial biopsy revealed a basosquamous cancer with an aggressive histologic pattern.
766
Benedet et al
Vuln7r Basal Cell Cancer
Three women in this series developed a recurrence, two at 2 years and one at 6 years. All had been treated initially with wide local excision in which the margins were positive. Two of these patients were retreated with another wide local excision, with negative margins, and both are alive and well. Rtbview of the histologic sections in these cases failed to reveal any histologic features that might have predicted recurrence other than the positive margins. Lymph-vascular space invasion by basal cell carcinoma was not evident in these patients. The third patient was treated initially with wide local excision and had immediate re-excision because of a positive margin. This second specimen had negative resection margins. She was well for 2 years, then appeared with a suspicious node in the left supraclavicular fossa. Histologic examination of this neck node showed metastatic disease with a histologic picture similar to that of her original vulvar lesion. At that time there was no evidence of any nodal disease either in the groin or on the vulva itself, but 2 ye,ars later she developed a lesion on the opposite side of the vulva, which biopsy showed to be basal cell carcinoma. The supraclavicular nodal metastasis was treated with irradiation, with apparently complete resolution. This woman died of renal failure 2 years later; her death was thought to be due to pelvic and abdominal disease, but no autopsy was done. This is the only death attributable to disease in this series. Currently, 14 patients are alive and well without evidence of disease. Thirteen others died of other causes; one of these had disease present at the time of her death (the patient who received no :specific treatment). No patients have been lost to follow-up. Twentyone of the 28 patients were followed for more than 5 years and in six women, the length of follow-up was 11-26 years. In only seven patients was the length of follow-up less than 5 years. Ten patients had other basal cell carcinomas on the face, trunk, or extremities. In four patients these lesions preceded the diagnosis of their vulvar can’cer, and in six women these lesions were diagnosed after the vulvar basal cell cancers. In ten patients, 11 other malignancies were diagnosed: One patient each had ovary, cervix, lung, breast, lymphoma, and squamous carcinoma of the vulva; three patients had colon cancers; and one patient had both colon and breast cancers. In all, 11 patients (39.3%) had another lesion, either basal cell or some other tumor.
Discussion This series confirms vulva is uncommon
that basal cell carcinoma of the and predominantly a tumor of
Obstetrii-s
& Chjnecolo~~y
Table
1. Metastatic
Basal
Cell
Carcinoma
of the Vulva
Size of primary (cm)
Year of report
Age (y)
Jimenez7 Perrone”
1975 1987
41 86
5x5 “Large”
HOffnIan’3
1988
74
6X4
Sworn” Winkelmanns
1979 1990
71 71
8 x 4.5 6 X 15
1994 1997
87 80
Principal
author
Gleesor? Benedet (current
study)
2.5 X 4 2.0 x 1
Pathologic
features
Site of metastasis
Invasion to subcutis Invasion to vagina, urethra, subcutis Invasion of subcutis; two discrete primary lesions Invasion of subcutis Necrosis to depth of symphysis pubis Shallow ulcer only Deep invasion to subcutis; oerineural invasion
Groin Bilateral inguinal 5 left 2/12 left inguinal 5/1.5 inguinal Groin Groin Supraclavicular
nodes:
Outconle 3 right,
nodes
NS Alive
and well,
14 mo
Alive .and well Died at 2 y
at 1 y
NS
nodes
NS NS
node
NS = not stated
elderly women. As with many vulvar lesions, patients most commonly present with an irritation or soreness or palpation of a nodule or a mass on the vulva, which then leads to the diagnosis. In this group of patients, as has been noted in other reports,‘,*,” an appreciable proportion had a substantial delay in presenting for assessment and diagnosis. The information available does not provide any insights or explanations as to the possible etiology of these lesions. Arsenicals, previous irradiation, syphilis, and chronic irritation have all been suggested as possible etiologic or precipitating causes. Although basal cell carcinoma of the face and extremities often has been linked to ultraviolet irradiation and sunlight, this does not appear to be a plausible explanation for vulvar lesions. It is interesting to note, however, that a large proportion of individuals in this series had basal cell carcinoma involving the face, trunk, or extremities. For the most part, these lesions behave in an indolent fashion and have a low propensity for lymphatic or distant spread. A MEDLINE search identified only six reports of metastatic basal cell carcinoma of the vulva. Jiminez et al” reported the first case of vulvar basal cell
Table
carcinoma metastatic to groin nodes in 1975. Since then, five additional cases have been reported.s,“,‘‘-‘a Table 1 summarizes the reported cases of metastatic basal cell carcinoma of the vulva. Six patients had deep invasion to the subcutaneous layer and a similar number had large tumors more than 2 cm in diameter. All of these patients had at least one of these adverse features, and five had both. It appears that lesions that do metastasize generally are larger in size and volume and often have aggressive-appearing or poorly differentiated histologic patterns. Unfortunately, outcome data for most of these cases are lacking. Although 11 other malignancies were noted in this group of patients, this frequency is probably not greater than what might be expected from a similar group of essentially elderly women. Wide local excision is the treatment generally recommended for this disease and appears to be sufficient for the vast majority of these individuals, particularly given their age and general medical condition. Although clear surgical margins should be attempted in all cases, this may not always be possible, particularly if functional or cosmetic factors are important. The results of this review suggest that these patients can be followed safely,
2. Series of Vulvar Basal Cell Carcinomas
outcome
Principal
author
Palladino7 Cruz-JimenezX Breen’ Simonsen”’ Perrone” Benedet (current
Year of report
study)
1969 1975 1975 1985 1987 1997
Summary
Age (y) Range
Mean
Other cancers
Recurrence
Died of disease
65 11 17 21 11 28
34-90 45-75 34-83 55-90 62-86 44-95
65 59 58 76 74 74
13/65 NS 3/17 7/21 3/11 11/28
13/65 o/11 o/17 2/21 o/11 3/28
0 0 0 0 0 1
153
34-95
67.7
37/142
18/153
No. of patients
Died of other disease 19 3 2 2 1 13
Length of follow-up Survived
Lost
(Y)
39 8 14 19 10 14
7 0 1 0 0 0
4-19 l-25 l-7 2-19 l-10 l-25
NS = not stated.
VOL.
90, NO.
5, NOVEMBER
1997
Benedet
et al
Vuivar Basal Cdl Cancer
767
as the propensity for spread is extremely low and the majority do not develop further disease. Metastasis from sites other than the vulva usually has a poor prognosis, with very short survival intervals once the diagnosis is made.‘” Similar evaluations with regard to metastatic basal cell carcinoma from the vulva cannot be made from the literature currently available. Table 2 summarizes the outcome data from the larger series of vulvar basal cell cancer in the literature, indicating the generally favorable outcome for this group of elderly women. These lesions do not seem to metastasize readily to regional nodes, so routine lymphadenectomy is not warranted. Nonetheless, individuals with larger tumors or tumors showing deep invasion with lymphatic involvement should be considered for selective lymphadenectomy until more information is available regarding the behavior of this group of lesions.
R$erences 1. Temesvary Vulva, mit 50:1575-82.
N. Uber ein multiples Krompechersches ausgendehnter Elephantiasis. Zentralbl
Karzinom Gynakol
der 1926;
3. Jimenez HT, Fenoglio noma with metastasis: 1975;121:285-6.
CM, A
Richart RM. case report.
Vulvar basal Am J Obstet
Benedet
et al
vulva
with
groin
Vuhar Basal Cell Cancer
node
basal
of the vulva.
cell
carcinoma
E, Gregori CA. 1975;46:122-9.
of
Basal
cell
10. Simonsen E, Johnsson J-E, Trope C, Alm I’. Basal (cell carcinoma of the vulva. Acta Obstet Gynecol Stand 1985;64:231-4. 11. Sworn MJ, Hammond GT, Buchanan R. Metastatic basal cell carcinoma of the vulva case report. Br J Obstet Gynaecol 1979;86: 332-3. 12. Perrone T, Twiggs LB, Adcock LL, Dehner LP. Vulvar basal cell carcinoma: An infrequently Pathol 1987;6:152-65. 13. Hoffman MS, Roberts WAS,
metastasizing
vulva with inguinal 29:113-9. 14. Farmer ER, Helwig
node
pathologic
study
lymph
Ruffolo
EH. Basal metastasis.
EB. Metastatic of seventeen
neoplasm.
basal cases.
cell
Cancer
cell
Int J Gynecol carcinoma
Gynecol
of the
Oncol
carcinoma:
1988;
A clinico-
1986;46:748-57.
Address reprint requests to: J. L. Benedet, MD Departmenf of Gynecologic Oncology BC Cancer Agency 600 West 10th Avenue BC V5Z
4E6
Canada E-mail: [email protected]
cell carciGynecol
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Received May 1, 1997. Received in rezrised form ]une Accepted July 10, 1997.
Copyright Gynecologists.
0
1997 by Published
23, 1997
The American by Elsevier
College of Science Inc.
Obstetricians
and
Obstetrics & (;ynecology