Buschke-Löwenstein tumour: a rare and challenging entity

Abstract

Buschke-Löwenstein tumour (BLT) is rare and locally aggressive, and malignant transformation is a possibility. Because there is no consensus on the best treatment approach, the authors present a treatment algorithm based on several case reports. A 57-year-old male patient resorted to surgical consultation with a giant perianal cauliflower-like mass. A BLT was diagnosed. Due to the involvement of the anal sphincter, a wide local excision saving the rectum failed. Abdominoperineal resection was performed. Malignant transformation was diagnosed, and adjuvant radiotherapy was delivered. Clinical evolution was uneventful. Aggressive behaviour despite the absence of malignancy is the hallmark of BLT. The common presentation is an anal mass with a cauliflower-like appearance. Anal verrucous carcinoma and squamous cell carcinoma are the major differential diagnoses. BLT treatment is challenging. Surgery is the first-line treatment, raging from wide local excision to abdominoperineal resection. To improve outcomes, chemoradiation can be used in combination with surgery. Long-term follow-up is mandatory.

Keywords: dermatology; general surgery; human papilloma virus.

Figure 1

Buschke-Löwenstein tumour. (A) Large perianal exophytic cauliflower-like mass. (B) Foley catheter is inside the rectum for reference during wide local excision.

Figure 2

Pelvic MRI showing deep infiltration of the subcutaneous fat by the Buschke-Löwenstein, with focal involvement of the external sphincter without clear invasion of the internal sphincter, with suspicions of a trans-sphincteric fistula. (A) Axial plane. (B) Sagittal plane.

Figure 3

Wide local excision. (A) Final aspect after local excision. (B) Terminal colostomy was performed by laparoscopy.

Figure 4

Abdominoperineal resection with negative margins. (A) The large perineal defect following surgery. It was closed with a V-Y miocutaneous advancement flap. (B) Specimen with macroscopic negative margins. (C) Final aspect at 18 months of follow-up.

Figure 5

Pathology findings. (A) Area of the superficial tumour with dyskeratosis. (B) High magnification image with intense mitotic activity and abnormal mitotic figures. (C) Deep rectal invasion and neutrophilic microabscesses. (D) Positive p16 immunohistochemistry (staining in more than 70% of tumour cells).

Figure 6

Proposed algorithm for BLT treatment. R0 resection is the first-line treatment. Although a wide local excision should be preferable, sometimes abdominoperineal resection is necessary. If a negative-margin resection is not possible, neoadjuvant chemoradiation should be offered to aim at downsizing. Chemoradiation alone is indicated for patients unfit for surgery or in cases of downsizing failure. When malignant transformation towards an SCC is present, adjuvant radiotherapy should be considered. A long-time follow-up is mandatory. ¹Multiple fistulae; invasion into the anal sphincter, anal canal or rectum; deep penetration. BLT, Buschke-Löwenstein tumour; SCC, squamous cell carcinoma.