Giant Condylomata Acuminata of Buschke and Lowenstein
- Author: Catharine Lisa Kauffman, MD, FACP; Chief Editor: Dirk M Elston, MD more...
Background
First described by Buschke and Löwenstein in 1925, the giant condyloma of Buschke and Löwenstein (GCBL) is a slow-growing, locally destructive verrucous plaque that typically appears on the penis but may occur elsewhere in the anogenital region. It most commonly is considered to be a regional variant of verrucous carcinoma, together with oral florid papillomatosis and epithelioma cuniculatum.
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Pathophysiology
GCBL is slow growing, highly destructive to contiguous tissue, and seldom metastasizes. Most commonly located on the glans penis, GCBL can be found on any anogenital mucosal surface, including the vulva, vagina, rectum, scrotum, and bladder.[1] Co-localization with human papillomavirus (HPV) types 6 and 11[2] ; occasionally HPV types 16 and 18; and, on one occasion, HPV 54 has been shown. The E6 protein of HPV-6 and HPV-11 binds p53 tumor suppressor protein less efficiently than that of HPV-16 and HPV-18 but, theoretically, could lead to accelerated degradation of the p53 protein. The E6 protein also inhibits p53 transcription.
Alternatively, a mutation may occur in the p53 protein, leading to clonal proliferation. Several reports have shown some overexpression of p53 in genital warts and squamous cell carcinomas (SCCs), but one study concluded that despite the overexpression, p53 mutations were not present.[3] Other implicated agents are chronic chemical exposure, chronic irritation, and poor hygiene.
Epidemiology
Frequency
United States
GCBL is rare. Estimates of incidence show that GCBL accounts for 5-24% of penile cancers, which, in turn, are 0.3-0.5% of male malignancies. Another review assessed that verrucous carcinoma accounted for approximately 50% of all low-grade SCCs of the penis. GCBL located outside the penis is much more infrequent. Fewer than 50 cases of perianal tumors and only 20-30 cases of vulvar or bladder GCBL have been reported. The bladder lesions have been associated with schistosomiasis (ie, Schistosoma haematobium).
International
SCC of the penis is much more common elsewhere in the world compared with the United States. No specific data are available in the English literature regarding international incidence.
Mortality/Morbidity
If untreated, GCBL can be locally very destructive, extending into the pelvic organs and bony structures. Even with treatment, morbidity rates can be high because recurrences are very common with all treatment modalities. One case report noted recurrent lesions in the ischial tuberosities that required pelvic exenteration. Malignant transformation is reported in 30-56% of patients.
Race
No racial predilection is reported.
Sex
Most cases of GCBL occur in males on the glans penis. This condition is more common in males who are uncircumcised. The male-to-female ratio is 3.5:1.
Age
Two thirds of cases of GCBL occur in persons younger than 50 years. It is rarely reported in children. A recent trend toward a younger reported age at presentation is recognized.
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