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A woman in her early 40s, HIV-positive, non-compliant to highly active antiretroviral therapy (HAART) and in stage C3 of AIDS. There were no other active sexually transmitted infections, except vulvar condylomatosis known for 12 years and unsuccessfully treated with oral isotretinoin, interferon and cryotherapy. She presented a ‘cauliflower-like’ mass in the vulva and perianal region with 35×11 cm, which had worsened in the last year (figure 1). Multiple full-thickness biopsies were performed. Histological analysis revealed epidermal hyperplasia, hyperkeratosis and koilocytosis with no evidence of dysplasia or malignant transformation. Colposcopic and proctoscopic examinations were normal and pelvic magnetic resonance showed no signs of deep infiltration. The case was evaluated by a multidisciplinary team of gynaecologists, plastic surgeons and dermatologists. Since previous topical treatments were unsuccessful and imiquimod would be too expensive for the patient considering the large extension of the lesion, surgical approach was decided. The patient was submitted to surgical excision of the mass with electrosurgery and curettage, and the wound was intentionally left open to heal by secondary intention. Histological examination confirmed no evidence of malignant transformation. At 1 year follow-up, the patient showed no signs of local recurrence, except for the presence of perianal condylomas, later treated with cryotherapy. With appropriate compliance to antiretroviral medication, the patient is currently in stage C2 of AIDs and disease free at 7-year follow-up (figure 2).
Buschke-Löwenstein condylomas are extremely rare benign tumours and reported cases in developed countries are even scarcer.1 2 Most commonly, they affect the penis and perianal regions, but also the vulva and the vagina.1 These tumours are associated with human papilloma virus and are more frequent in immunocompromised patients.3–5 Even if their histology is benign and there is no distant metastisation, they may be locally destructive with propensity for fistulisation, ulceration, abscesses and haemorrhage and, eventually, may prove fatal.6 7 The recurrence rate is high (60%–70%) and malignant transformation may occur in about 30% to 56% of patients.6 8 9 HIV-positive patients present higher susceptibility to rapid progression, recurrence and malignant transformation and may present a clinical challenge in achieving optimal therapeutic management.8 10 11 Screening for other sexually transmitted infections is essential. Due to its rarity, consensus on management and treatment is challenging and many treatment options are mentioned in literature, such as podophyllin, 5-fluorouracil, methotrexate, bleomycin, interferon, oral retinoid therapy and even paiteling.2 9 12–14 Minimally-invasive techniques (cryotherapy, photodynamic therapy, CO2 laser ablation, electrosurgery or curettage) and radiotherapy may also be used in some smaller lesions.2 9 14 Wide surgical excision with tumour-free margins seems to be the most accepted approach for large lesions.15 After the excision, the wound may be left open to granulate or reconstructive surgery with flaps or grafts may be performed, taking into account the size and location of the lesion.14 16–19 Recently, immunotherapy with imiquimod has had success in treating large lesions.20 21
Buschke-Löwenstein tumours are extremely rare but have an enormous impact on patients’ physical, mental and social aspects of life. Education of immunocompromised patients is an essential tool to detect this disease in earlier stages and prevent its progression.
Though surgical excision with tumour-free margins seems to be the most accepted approach, other minimally invasive techniques (cryotherapy, photodynamic therapy, electrosurgery) may prove adequate for some patients.
Promising results with some non-surgical options such as imiquimod or paiteling may become a more common option with less residual scaring and lower recurrence rates.
Regular follow-up is mandatory in order to detect early-stage recurrences, especially in immunocompromised patients.
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Contributors JS and MR were directly responsible for the initial diagnosis; JS, MR and DM were responsible for the surgical intervention. AMV, JS and DM were all involved in the follow-up of the patient. All authors were major contributors in writing the manuscript. All authors read and approved the final manuscript.
Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
Competing interests None declared.
Provenance and peer review Not commissioned; externally peer reviewed.