Journal of the American Academy of Dermatology
Saiag et al. 23. Navin TR, Miller KD, Satriale RF, et a1. Adverse reactions
man immunodeficiency virus infection. Arch Dermatol
associated with pyrimethamine-sulfadoxine prophylaxis for Pneumocystis carinii infections in AIDS [Letter]. Lancet
1991;127:714-7. 28. Saiag P, Capesius C, Roujeau J-C, et a1. Abnormal phar-
ysis occurring during treatment with trimethoprim alone. Br Moo J 1988;296:970. 25. Lee BL, Medina I, Benowitz NL, et a1. Dapsone, trimethoprim, and sulfamethoxazole plasma levels during treatment of pneumocystis pneumonia in patients with the acquired immunodeficiencysyndrome (AIDS). Ann Intern
71. 30. Rieder MJ, Uetrecht J, Shear NH, et a1. Diagnosis of sul-
1985;1:1332. 24. Nwokolo C, Byrne L, Misch KJ. Toxic epidermal necrol-
Moo 1989;110:606-11. 26. Brunet lB, David G, Lantrade P, et a1. La prevalence de l'infection par Ie VIR en France en 1989. Bull Epidemiol Rebdomadaire 1990;37: 159-61. 27. Coopman SA, Stern RS. Cutaneous drug reactions in hu-
macokinetics of the culprit drug in toxic epidermal necrolysis [Abstract]. J Invest Dermatol 1988;91 :402. 29. Kovacs JA, Hiemenz JW, Macher AM, et a1. Pneumocystis carinil pneumonia: a comparison between patients with the acquired immunodeficiencysyndrome and patients with other immunodeficiencies. Ann Intern Med 1984; 100:663fonamide hypersensitivity reactions by in-vitro "rechallenge" with hydroxylamine metabolites. Ann Intern Med
1989;110:28 6-9 . 31. Torgovnick J. Desensitization to sulfonarnides in patients with HIV infection [Letter]. Am J Med 1990;88:548-9.
Basal cell carcinoma of the scrotum Report of three cases and review of the literature George T. Nahass, MD, Andrew Blauvelt, MD, Craig L. Leonardi, MD, and Neal S. Penneys, MD, PhD Miami, Florida Background: Although basal cell carcinoma (BCC) is the most common human malignancy, only 21 cases involving the scrotum have been previously reported. Objective: Our purpose is to describe three additional cases of scrotal BCC and review the literature summarizing the clinical features and identifying any predisposing factors. Methods: We retrospectively reviewed 21 cases ofscrotal BCC and described three new cases. Polymerase chain reaction (PCR) was used to detect human papillomavirus (HPV) DNA in our biopsy specimens. Results: Scrotal BCCs present as persistent ulcerations or plaques without identifiable predisposing factors. Lymphatic, pulmonary, or skin metastases were present in 3 of 24 cases (13%) resulting in death in one case. PCR did not detect HPV DNA in our three cases. Conclusion: Scrotal BCC rarely occurs and should be considered in the diagnosis of a persistent scrotal ulcer or plaque. Metastatic disease may be more common than with other BeCs and wide local excision or Mohs micrographic surgery may be the most appropriate initial therapeutic approach. (J AM ACAD DERMATOL 1992;26:574-8.)
Basal cell carcinoma (BCe) is the most common human malignancy. 1 The American Cancer Society estimated that more than 600,000 cases of skin cancer were diagnosed in 19902 and that 70% to 75% of From the Department of Dermatology and Cutaneous Surgery, University of Miami School of Medicine. Accepted for publication Nov. 12, 1991. Reprint requests: Neal S. Penneys, MD, PhD, Department of Dermatology and Cu taneous Surgery, University of Miami School of M edicine, P.O. Box 016940 (R-ll), Miami, FL 33101. 16/1/34926
574
these cases were BCCs. Although many different factors have been implicated in the pathogenesis of BCCs, I UV light exposure is the most common predisposing factor. Therefore it is not surprising that 85% of all BCes occur On the head and neck. 1 However, BCCs can also occur in sun-protected areas including the rare occurrence on the scrotum. Although squamous cell carcinoma (SCC) is the most common form of scrotal malignancy, there have been 21 previously reported cases of scrotal BCC, mostly in the urology literature. We report the
Volume 26 Number 4 Aprill992
Scrotal basal cell carcinoma 575
Fig. 1. Ulcerated scrotal plaque with well-defined pearly border just left of mid line.
clinical and histologic features of three additional cases of scrotal BCe and review the literature.
CASE REPORTS Case I.A 61-year-old white man had a 16-year history of a scrotal eruption. A biopsy specimen obtained 4.Years after the lesion was first noted showed a chronic dermatitis. He was treated with various topical corticosteroids and antibacterial products without significant improvement. One year before our evaluation, the involved area became more indurated and increased in size. He had no history of prior skin cancer. Physical examination revealed a 2.5 em ulcerated erythematous plaque with a well-defined raised pearly border just left of the midline of the scrotum (Fig. 1). No adenopathy was present and the remainder ofthe skin examination was unremarkable. A biopsy specimen showed superficial BCe. The cancer was excised by Mohs micrographic surgery. Case 2. An 82-year-old white man had left-sided scrotal pruritus for 8 months after a bilateral herniorrhaphy. This was treated with various topical over-the-counter products without benefit. Intermittently, the area would become irritated and bleed. He had one prior skin cancer of unknown type removed from the face. Physical examination revealed a 1.5 em pigmented plaque at the base of the left side of the scrotum. No adenopathy was appreciated and the remainder of the skin examination was unremarkable. An incisional biopsy specimen showed pigmented BCe. The cancer was excised. Examination of the surgical specimen revealed a multifocal BeC, which was completely excised. Case 3. An 80-year-old white man had an asymptomatic left-sided scrotal lesion for 6 months. Physical exam·
ination revealed a 7.5 mm ulcerated plaque on the left side of the scrotum. No adenopathy was present and the remainder of the skin examination was unremarkable. A biopsy specimen showed superficial Bee. The cancer was excised. In all cases there was no history of scrotal trauma, radiation therapy, or exposure to sun, chemicals, or arsenic. Polymerase chain reaction (peR) was performed on the formalin-fixed paraffin-embedded sections. The primer! probe system used is capable of detecting3 and typing4 multiple human papillomaviruses (HPVs). The primers used bracket a 450 base-pair region within the highly conserved L1 open reading frame of numerous HPVs. Ethidium-stained amplification products were detected on agarose gels and by dot blot hybridization as previously described. 5 PCR did not detect HPV DNA in the three biopsy specimens.
DISCUSSION Carcinoma of the scrotum was the first recognized occupational cancer described in 1775 by Percivall Pott. 6 He entitled this malignancy "chimney sweeper's cancer" because of the high incidence among those so employed. Subsequently, a variety of other occupations involving prolonged exposure to oil and its products (Le., cotton mule spinners, gas and tar workers, mill workers, and machinists), radiation therapy, arsenical compounds,? and chronic dermatitis 8 have been associated with of the scrotum. It is now recognized that the carcinogen responsible for cases of scrotal in those exposed to soot or oil is 3,4-benzpyrene. 9
sec
sec
Journal of the American Academy of Dermatology
576 Nahass et al. Table 1. Histologic characteristics of scrotal carcinoma Author(s)
Year
sec
BeC
Other
Total
Godan!5 Kickman and Dufresne l6 Ray and Whitmore? McDonald8 Gerber9 Lowe!? Total
1962 1967
8 26
2
6
Angiosarcoma (1)
15 28
1977 1982 1985 1985
18 9
3
7 3 71
The discovery and avoidance of these different etiologic factors for scrotal carcinoma have resulted in fewer cases, 8 but scrotal cancers still do rarely occur. Early reviews of scrotal carcinoma mainly reflect the occupationally related cases,IO-14 but recent series document a somewhat different experience with less influence from known carcinogens.7-9, 15-17 Similar to the earlier cases, the more current series still demonstrate that the vast majority of scrotal malignancies are SCC. The histologic characteristics of the cases presented in six recent reviews are summarized in Table I. Other neoplastic processes that also rarely involve the scrotum and may mimic BeC include extramammary Paget's disease,18 Bowen's disease, 9 and melanoma. 19 BCes were present in 13 of 86 cases (15%) summarized in Table I. The clinical and histologic features ofthe 21 previously reported cases ofBCC ofthe scrotum and our three new cases are summarized in Table II. The average age of patients with scrotal BCC was 65 years (range 42 to 82 years). There was a wide variation in the duration of the lesion before presentation (3 months to 16 years). Clinically the lesions presented as plaques (four) or ulcers (seven). The side of the lesion was reported in 8 of 24 cases and was found on the left (five), right (two), and bilateral (one). This suggests a tendency for BCC to occur on the left side, but the available data are limited. There is a definite tendency to left-sided lesions in occupationally related cases (60% to 84%) of scrotal SCc. However, if patients with occupational cancers are excluded, no predilection for either side is evident. 7 Surgical treatment was the most common sole therapeutic intervention (12 patients). Radiation therapy (four patients) was the only other solitary therapeutic modality reported. A combination of
1 1
Fibrosarcoma (1)
TI
2"
19 12 9 3
86
surgery and radiation therapy was used in three cases, and chemotherapy was added to these other modalities in two patients with metastatic disease. The follow-up period ranged from 5 months to 11 years. Lymphatic, pulmonary, or skin metastases occurred in 3 of 24 cases (13%)20,21,25; death occurred from progressive disease in one case. Because metastatic BCC is rare, with a reported incidence of 0.0028% to 0.1%, I the 13% incidence of metastatic disease with scrotal BCC suggests a higher rate of metastasis than BCC at other sites. However, given the small number of cases in this review and the fact that metastatic cases are more likely to be reported, the biologic behavior of scrotal BCC cannot be definitively determined. Thorough clinical evaluation, followed by wide local excision (Ray and Whitmore? recommended 2 to 3 em margins for scrotal carcinoma in general) or Mohs micrographic surgery ifthe primary lesion is large, may be the most appropriate initial therapeutic approach to these lesions. The cause of BCC on non-sun-exposed areas is unknown. Previous studies of immunosuppressed patients suggest that the immune system may be important in the prevention of BCC development.27 Depressed immunosurveillance from advancing age or UV light at distant sites may have an etiologic role in these cases. However, ifimmunosuppression is the only factor involved, more BCCs in non-sun-exposed areas would be expected with the widespread use of immunosuppressant medications today. Because this is not evident and none of the reported patients with scrotal BCC was overtly immunosuppressed, other factors are undoubtedly involved. In this review, no occupation, predisposing clinical condition, or exposure to chemicals was consistentlyobserved. However, in two patients there was
Volume 26 Number 4 April 1992
Scrotal basal cell carcinoma 577
Table II. Reported cases of scrotal basal cell carcinoma
Author(s)
Richter20
Duration of lesion before presentation
Oinical
description
Treatment
Superficial erosion NA NA NA NA NA NA
XRT, chemical debridement XRT Extirpation Extirpation, XRT XRT Extirpation, XRT XRT
Kickbam and Dufresne l6 NA NA Hughes 21 4 yr
NA NA Ulcer
Ray and Whitmore? McEl eney22
NA NA Local excision, XRT,chemo Local excision Local excision
NA Thickened scrotum, plaque, pearly border Nodular ulcer Local excision
Godan l5
Grossman and Sogani23
NA 5 mo 1 yr 4mo 4 yr 6mo 4mo
NA 15 yr 3 yr 8yr
Greider and Vernon 24 McDonald8
Cieplinski25
1 yr
NA NA
NA
NA
3 mo
Casal et al. 26
15 yr
Gerber9
NA
Our cases
16 yr 8 mo
6 mo
5 yr died wi th residual BCC NA NA NA NA NA NA NA NA NA 4 yr died of progressive BCC NA NA
8 mo without recurrence 11 yr without Local excision recurrence Local excision 5 mo without recurrence Local excision NA Local excision 4 yr without recurrence Local excision 10 yr without recurrence 3.5 yr with Local excision, BCG llrununotherapy, cherno residual disease Local excision, 1 yr without XRT recurrence 6 mo without NA recurrence Mohs surgery 10 mo without recurrence 1 yr without Local excision recurrence 5 mo without Local excision recurrence
Superficial ulcerating lesion Flat, erythematous
NA NA
Follow-up
Multiple indurated ulcers Indurated tumor NA Ulcerated plaque Pigmented plaque Ulcer
Cherno, Chemotherapy; NA, information not available; XRT, radiation therapy.
a long history of dermatitis preceding the development of BCe. Several authors have previously suggested that scrotal carcinoma may be a result of nonspecific factors such as poor hygiene and chronic irritation.8, 18 PCR did not detect HPV DNA in the BCC tissue of our three cases and we believe it is unlikely
that HPV infection played any role in the development of these BCCs. These results are not surprising because Eliezri et a1.28 found HPV DNA in only I of26 BCes. However, in genital SCC, HPV DNA has been detected in 68% of cases and certain HPV types are believed to have an etiologic role in cutaneous SCC at various sites. 28
578
Nahass et at.
REFERENCES 1. Miller 81. Biology of basal cell carcinoma (Part I). J AM ACAD DERMATOL 1991;24:1-13. 2. Cancer facts and figures-1990. CA 1990:2-31. 3. Bauer HM, Greer CE, Chamber lC, et a1. Genital human papillomavirus infection in female university students as determined by a PCR-based method. JAMA 1991;265: 472-7. 4. Manos MM, Ting Y, Wright DK, et a1. Use of polymerase chain reaction amplification for the detection of genital human papillomaviruses. Cancer Cells 1989;7:209-14. 5. Leonardi CL, Zhu WY, Kinsey WH, et a1. Trichilemmomas are not associated with human papillomavirus DNA. 1 Cutan Patho1 1990;18:193-7. 6. Pott P. Cancer scroti. Chir Works 1775;5:63. 7. Ray B, Whitmore WF. Experience with carcinoma of the scrotum. J Ur011977;117:741-5. 8. McDonald MW. Carcinoma of scrotum. Urology 1982; 19:269-74. 9. Gerber WL. Scrotal malignancies: The University of Iowa experience and a review of the literature. Urology 1985; 26:337-42. 10. Southam AH, Wilson SR. Cancer of the scrotum: the etiology, clinicalfeatures and treatment ofthe disease. Br Med 1 1922;2:971-3. 11. Graves RC, Flo S. Carcinoma of the scrotum. J Urol 1940;43:309-32. 12. Chase PP. Cancer scroti. Rhode Island Med 1 1942;25: 104-6. 13. DeanAL. Epithelioma of scrotum. 1Uro11948;61:508-18. 14. Higgins CC, Warden JG. Cancer of the scrotum. 1 Urol 1949;62:250-6. 15. Godan F. The treatment of tumors of the scrotum. Br 1 Radio1 1962;35:861-5.
Journal of the American Academy of Dermatology
16. Kickham Cl, Dufresne M. An assessment of carcinoma of the scrotum. J Uro1 1967;98:108-10. 17. Lowe FC. Squamous cell carcinoma of the scrotum. Urology 1985;25:63-5. 18. Hach WHo Adenocarcinoma of the scrotum (extramammary Paget's disease): case report and review of the literature. 1 Uro11984;132:137-9. 19. Abeshouse BS. Primary and secondary melanoma of the genitourinary tract. South Med J 1958;51:994-1005. 20. Richter VG. Subpleura1e lungenmetastasen bei sog. Basalzellencarcinom. Hautarzt 1957;8:215-9. 21. Hughes JM. Metastatic basal cell carcinoma: a report of two cases and a review of the literature. Clin Radiol 1973;24:392-3. 22. McEleney DA. Basal cell carcinoma of the scrotum. Cutis 1976;18:227. 23. Grossman HB, Sogani PC. Basal cell carcinoma of the scrotum. Urology 1981; 17:241-2. 24. Greider HD, Vernon SE. Basal cell carcinoma of the scrotum: a case report and literature review. JUral 1982; 127:145-6. 25. Cieplioski W. Combination chemotherapy for the treatment of metastatic basal cell carcinoma of the scrotum: a case report. Clio Oncol 1984;10:267-72. 26. Casal J, Solari JJ, Monserrat 1M. Epitelioma del escroto. Rev Argent Urol Nefro11965;35:661-5. 27. Miller Sl. Biology of basal cell carcinoma (Part II). 1 AM ACAD DERMATOL 1991;24:161-75. 28. Eliezri YD, Silverstein SJ, Nuovo Gl. Occurrence of human papillomavirus type 16DNAin cutaneoussquamous and basal cell neoplasms. J AM ACAD DERMATOL 1990;23:836-42.
EDITOR'S NOTE The JOURNAL OF THE AMERICAN ACADEMY OF DERMATOLOGY introduces a new format for abstracts accompanying articles included in the Clinical and Laboratory Studies, Therapy, Dermatopathology, and Dermatologic Surgery sections. Authors submitting manuscripts for these sections to the JOURNAL should provide an abstract of no more than 150 words structured according to the following headings: Background, Objective, Methods, Results, and Conclusion. Please consult the Information for Authors for details.
Saiag et al. 23. Navin TR, Miller KD, Satriale RF, et a1. Adverse reactions
man immunodeficiency virus infection. Arch Dermatol
associated with pyrimethamine-sulfadoxine prophylaxis for Pneumocystis carinii infections in AIDS [Letter]. Lancet
1991;127:714-7. 28. Saiag P, Capesius C, Roujeau J-C, et a1. Abnormal phar-
ysis occurring during treatment with trimethoprim alone. Br Moo J 1988;296:970. 25. Lee BL, Medina I, Benowitz NL, et a1. Dapsone, trimethoprim, and sulfamethoxazole plasma levels during treatment of pneumocystis pneumonia in patients with the acquired immunodeficiencysyndrome (AIDS). Ann Intern
71. 30. Rieder MJ, Uetrecht J, Shear NH, et a1. Diagnosis of sul-
1985;1:1332. 24. Nwokolo C, Byrne L, Misch KJ. Toxic epidermal necrol-
Moo 1989;110:606-11. 26. Brunet lB, David G, Lantrade P, et a1. La prevalence de l'infection par Ie VIR en France en 1989. Bull Epidemiol Rebdomadaire 1990;37: 159-61. 27. Coopman SA, Stern RS. Cutaneous drug reactions in hu-
macokinetics of the culprit drug in toxic epidermal necrolysis [Abstract]. J Invest Dermatol 1988;91 :402. 29. Kovacs JA, Hiemenz JW, Macher AM, et a1. Pneumocystis carinil pneumonia: a comparison between patients with the acquired immunodeficiencysyndrome and patients with other immunodeficiencies. Ann Intern Med 1984; 100:663fonamide hypersensitivity reactions by in-vitro "rechallenge" with hydroxylamine metabolites. Ann Intern Med
1989;110:28 6-9 . 31. Torgovnick J. Desensitization to sulfonarnides in patients with HIV infection [Letter]. Am J Med 1990;88:548-9.
Basal cell carcinoma of the scrotum Report of three cases and review of the literature George T. Nahass, MD, Andrew Blauvelt, MD, Craig L. Leonardi, MD, and Neal S. Penneys, MD, PhD Miami, Florida Background: Although basal cell carcinoma (BCC) is the most common human malignancy, only 21 cases involving the scrotum have been previously reported. Objective: Our purpose is to describe three additional cases of scrotal BCC and review the literature summarizing the clinical features and identifying any predisposing factors. Methods: We retrospectively reviewed 21 cases ofscrotal BCC and described three new cases. Polymerase chain reaction (PCR) was used to detect human papillomavirus (HPV) DNA in our biopsy specimens. Results: Scrotal BCCs present as persistent ulcerations or plaques without identifiable predisposing factors. Lymphatic, pulmonary, or skin metastases were present in 3 of 24 cases (13%) resulting in death in one case. PCR did not detect HPV DNA in our three cases. Conclusion: Scrotal BCC rarely occurs and should be considered in the diagnosis of a persistent scrotal ulcer or plaque. Metastatic disease may be more common than with other BeCs and wide local excision or Mohs micrographic surgery may be the most appropriate initial therapeutic approach. (J AM ACAD DERMATOL 1992;26:574-8.)
Basal cell carcinoma (BCe) is the most common human malignancy. 1 The American Cancer Society estimated that more than 600,000 cases of skin cancer were diagnosed in 19902 and that 70% to 75% of From the Department of Dermatology and Cutaneous Surgery, University of Miami School of Medicine. Accepted for publication Nov. 12, 1991. Reprint requests: Neal S. Penneys, MD, PhD, Department of Dermatology and Cu taneous Surgery, University of Miami School of M edicine, P.O. Box 016940 (R-ll), Miami, FL 33101. 16/1/34926
574
these cases were BCCs. Although many different factors have been implicated in the pathogenesis of BCCs, I UV light exposure is the most common predisposing factor. Therefore it is not surprising that 85% of all BCes occur On the head and neck. 1 However, BCCs can also occur in sun-protected areas including the rare occurrence on the scrotum. Although squamous cell carcinoma (SCC) is the most common form of scrotal malignancy, there have been 21 previously reported cases of scrotal BCC, mostly in the urology literature. We report the
Volume 26 Number 4 Aprill992
Scrotal basal cell carcinoma 575
Fig. 1. Ulcerated scrotal plaque with well-defined pearly border just left of mid line.
clinical and histologic features of three additional cases of scrotal BCe and review the literature.
CASE REPORTS Case I.A 61-year-old white man had a 16-year history of a scrotal eruption. A biopsy specimen obtained 4.Years after the lesion was first noted showed a chronic dermatitis. He was treated with various topical corticosteroids and antibacterial products without significant improvement. One year before our evaluation, the involved area became more indurated and increased in size. He had no history of prior skin cancer. Physical examination revealed a 2.5 em ulcerated erythematous plaque with a well-defined raised pearly border just left of the midline of the scrotum (Fig. 1). No adenopathy was present and the remainder ofthe skin examination was unremarkable. A biopsy specimen showed superficial BCe. The cancer was excised by Mohs micrographic surgery. Case 2. An 82-year-old white man had left-sided scrotal pruritus for 8 months after a bilateral herniorrhaphy. This was treated with various topical over-the-counter products without benefit. Intermittently, the area would become irritated and bleed. He had one prior skin cancer of unknown type removed from the face. Physical examination revealed a 1.5 em pigmented plaque at the base of the left side of the scrotum. No adenopathy was appreciated and the remainder of the skin examination was unremarkable. An incisional biopsy specimen showed pigmented BCe. The cancer was excised. Examination of the surgical specimen revealed a multifocal BeC, which was completely excised. Case 3. An 80-year-old white man had an asymptomatic left-sided scrotal lesion for 6 months. Physical exam·
ination revealed a 7.5 mm ulcerated plaque on the left side of the scrotum. No adenopathy was present and the remainder of the skin examination was unremarkable. A biopsy specimen showed superficial Bee. The cancer was excised. In all cases there was no history of scrotal trauma, radiation therapy, or exposure to sun, chemicals, or arsenic. Polymerase chain reaction (peR) was performed on the formalin-fixed paraffin-embedded sections. The primer! probe system used is capable of detecting3 and typing4 multiple human papillomaviruses (HPVs). The primers used bracket a 450 base-pair region within the highly conserved L1 open reading frame of numerous HPVs. Ethidium-stained amplification products were detected on agarose gels and by dot blot hybridization as previously described. 5 PCR did not detect HPV DNA in the three biopsy specimens.
DISCUSSION Carcinoma of the scrotum was the first recognized occupational cancer described in 1775 by Percivall Pott. 6 He entitled this malignancy "chimney sweeper's cancer" because of the high incidence among those so employed. Subsequently, a variety of other occupations involving prolonged exposure to oil and its products (Le., cotton mule spinners, gas and tar workers, mill workers, and machinists), radiation therapy, arsenical compounds,? and chronic dermatitis 8 have been associated with of the scrotum. It is now recognized that the carcinogen responsible for cases of scrotal in those exposed to soot or oil is 3,4-benzpyrene. 9
sec
sec
Journal of the American Academy of Dermatology
576 Nahass et al. Table 1. Histologic characteristics of scrotal carcinoma Author(s)
Year
sec
BeC
Other
Total
Godan!5 Kickman and Dufresne l6 Ray and Whitmore? McDonald8 Gerber9 Lowe!? Total
1962 1967
8 26
2
6
Angiosarcoma (1)
15 28
1977 1982 1985 1985
18 9
3
7 3 71
The discovery and avoidance of these different etiologic factors for scrotal carcinoma have resulted in fewer cases, 8 but scrotal cancers still do rarely occur. Early reviews of scrotal carcinoma mainly reflect the occupationally related cases,IO-14 but recent series document a somewhat different experience with less influence from known carcinogens.7-9, 15-17 Similar to the earlier cases, the more current series still demonstrate that the vast majority of scrotal malignancies are SCC. The histologic characteristics of the cases presented in six recent reviews are summarized in Table I. Other neoplastic processes that also rarely involve the scrotum and may mimic BeC include extramammary Paget's disease,18 Bowen's disease, 9 and melanoma. 19 BCes were present in 13 of 86 cases (15%) summarized in Table I. The clinical and histologic features ofthe 21 previously reported cases ofBCC ofthe scrotum and our three new cases are summarized in Table II. The average age of patients with scrotal BCC was 65 years (range 42 to 82 years). There was a wide variation in the duration of the lesion before presentation (3 months to 16 years). Clinically the lesions presented as plaques (four) or ulcers (seven). The side of the lesion was reported in 8 of 24 cases and was found on the left (five), right (two), and bilateral (one). This suggests a tendency for BCC to occur on the left side, but the available data are limited. There is a definite tendency to left-sided lesions in occupationally related cases (60% to 84%) of scrotal SCc. However, if patients with occupational cancers are excluded, no predilection for either side is evident. 7 Surgical treatment was the most common sole therapeutic intervention (12 patients). Radiation therapy (four patients) was the only other solitary therapeutic modality reported. A combination of
1 1
Fibrosarcoma (1)
TI
2"
19 12 9 3
86
surgery and radiation therapy was used in three cases, and chemotherapy was added to these other modalities in two patients with metastatic disease. The follow-up period ranged from 5 months to 11 years. Lymphatic, pulmonary, or skin metastases occurred in 3 of 24 cases (13%)20,21,25; death occurred from progressive disease in one case. Because metastatic BCC is rare, with a reported incidence of 0.0028% to 0.1%, I the 13% incidence of metastatic disease with scrotal BCC suggests a higher rate of metastasis than BCC at other sites. However, given the small number of cases in this review and the fact that metastatic cases are more likely to be reported, the biologic behavior of scrotal BCC cannot be definitively determined. Thorough clinical evaluation, followed by wide local excision (Ray and Whitmore? recommended 2 to 3 em margins for scrotal carcinoma in general) or Mohs micrographic surgery ifthe primary lesion is large, may be the most appropriate initial therapeutic approach to these lesions. The cause of BCC on non-sun-exposed areas is unknown. Previous studies of immunosuppressed patients suggest that the immune system may be important in the prevention of BCC development.27 Depressed immunosurveillance from advancing age or UV light at distant sites may have an etiologic role in these cases. However, ifimmunosuppression is the only factor involved, more BCCs in non-sun-exposed areas would be expected with the widespread use of immunosuppressant medications today. Because this is not evident and none of the reported patients with scrotal BCC was overtly immunosuppressed, other factors are undoubtedly involved. In this review, no occupation, predisposing clinical condition, or exposure to chemicals was consistentlyobserved. However, in two patients there was
Volume 26 Number 4 April 1992
Scrotal basal cell carcinoma 577
Table II. Reported cases of scrotal basal cell carcinoma
Author(s)
Richter20
Duration of lesion before presentation
Oinical
description
Treatment
Superficial erosion NA NA NA NA NA NA
XRT, chemical debridement XRT Extirpation Extirpation, XRT XRT Extirpation, XRT XRT
Kickbam and Dufresne l6 NA NA Hughes 21 4 yr
NA NA Ulcer
Ray and Whitmore? McEl eney22
NA NA Local excision, XRT,chemo Local excision Local excision
NA Thickened scrotum, plaque, pearly border Nodular ulcer Local excision
Godan l5
Grossman and Sogani23
NA 5 mo 1 yr 4mo 4 yr 6mo 4mo
NA 15 yr 3 yr 8yr
Greider and Vernon 24 McDonald8
Cieplinski25
1 yr
NA NA
NA
NA
3 mo
Casal et al. 26
15 yr
Gerber9
NA
Our cases
16 yr 8 mo
6 mo
5 yr died wi th residual BCC NA NA NA NA NA NA NA NA NA 4 yr died of progressive BCC NA NA
8 mo without recurrence 11 yr without Local excision recurrence Local excision 5 mo without recurrence Local excision NA Local excision 4 yr without recurrence Local excision 10 yr without recurrence 3.5 yr with Local excision, BCG llrununotherapy, cherno residual disease Local excision, 1 yr without XRT recurrence 6 mo without NA recurrence Mohs surgery 10 mo without recurrence 1 yr without Local excision recurrence 5 mo without Local excision recurrence
Superficial ulcerating lesion Flat, erythematous
NA NA
Follow-up
Multiple indurated ulcers Indurated tumor NA Ulcerated plaque Pigmented plaque Ulcer
Cherno, Chemotherapy; NA, information not available; XRT, radiation therapy.
a long history of dermatitis preceding the development of BCe. Several authors have previously suggested that scrotal carcinoma may be a result of nonspecific factors such as poor hygiene and chronic irritation.8, 18 PCR did not detect HPV DNA in the BCC tissue of our three cases and we believe it is unlikely
that HPV infection played any role in the development of these BCCs. These results are not surprising because Eliezri et a1.28 found HPV DNA in only I of26 BCes. However, in genital SCC, HPV DNA has been detected in 68% of cases and certain HPV types are believed to have an etiologic role in cutaneous SCC at various sites. 28
578
Nahass et at.
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EDITOR'S NOTE The JOURNAL OF THE AMERICAN ACADEMY OF DERMATOLOGY introduces a new format for abstracts accompanying articles included in the Clinical and Laboratory Studies, Therapy, Dermatopathology, and Dermatologic Surgery sections. Authors submitting manuscripts for these sections to the JOURNAL should provide an abstract of no more than 150 words structured according to the following headings: Background, Objective, Methods, Results, and Conclusion. Please consult the Information for Authors for details.
