Giant Condylomata Acuminata of Buschke and Lowenstein

Updated: Oct 16, 2018
  • Author: Catharine Lisa Kauffman, MD, FACP; Chief Editor: Dirk M Elston, MD  more...
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Overview

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.

Related Medscape Drugs & Diseases articles of possible interest include Head and Neck Cutaneous Squamous Cell Carcinoma and Human Papillomavirus.

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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, 3, 4] ; occasionally HPV types 16 and 18; and, on one occasion, HPV-54 has been shown. In a study of various types of verrucous carcinomas, the presence of low-risk HPV serotypes 6 and 11 was confirmed in all three giant condylomas analyzed. Two anal lesions showed HPV-6, while a vulvar giant condyloma demonstrated the presence of HPV-11. Although a causal role of these HPV strains in carcinogenesis was not directly supported, the authors recommend the testing for HPV-6 and HPV-11 as a tool to improve the differential diagnosis of giant condylomas from other verrucous carcinomas. [4]

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 it appears that p53 mutations were not present. Other implicated agents are chronic chemical exposure, chronic irritation, and poor hygiene.

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Etiology

Chronic phimosis and poor penile hygiene have been postulated as inciting or contributing events. This may account for the higher incidence in males who are uncircumcised. Populations with a higher incidence of circumcision have a lower rate of GCBL. In general, newborn circumcision has been estimated to be 99.9% effective in eliminating cancer of the penis. Chronic irritation, produced by a perianal fistula and ulcerative colitis, has been implicated as a causative factor. Immunosuppression secondary to HIV disease or due to immunosuppressive medication may be a predisposing factor.

Other risk factors for GCBL are low socioeconomic status, drug abuse, use of oral contraceptives, presence of other sexually transmitted diseases, diabetes, smoking, and, possibly, pregnancy, [5] which may be associated with an impaired immune response. [6] The dependency of Buschke-Löwenstein tumor growth to a state of decreased immune responsiveness is exemplified by the development of new penile and perianal lesions in a male with chronic lymphocytic leukemia at the time he received chemotherapy. [7]

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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.

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, with a mean age of presentation of 44 years.

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Prognosis

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.

Inadequately treated GCBL has a relentless progression and is fatal by direct spread to pelvic organs. By definition, adequately treated GCBL has a low recurrence rate and, therefore, an excellent prognosis. However, one study of perianal/anogenital GCBL, with treatments ranging variously from podophyllin to pelvic exenteration, showed a 68% recurrence rate with a 21% mortality rate.

In another series of 42 patients, the median number of recurrences of giant condyloma acuminatum was 2 years (range, 1-7 y), and the median time to first recurrence was 10 months. A high rate of recurrence correlates with a long duration of disease. [8]

One case report noted recurrent lesions in the ischial tuberosities that required pelvic exenteration.

Malignant transformation is reported in 30-56% of patients.

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Patient Education

For excellent patient education resources, visit eMedicineHealth's Men’s Health Center and Cancer Center. Also, see eMedicineHealth's patient education article Cancer: What You Need to Know.

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