Giant Condyloma Acuminata of the Anorectum Treated by Abdominoperineal Resection And Gracilis Flap Reconstruction.
G Hirst, WD Beasley and J Beynon
Abstract
Anorectal giant condyloma acuminata (GCA) should be considered a fatal disease and aggressive multidisciplinary management is necessary. We present a case that illustrates the difficulties encountered and strategies employed in managing these rare tumours. We also provide a review of the pathology and management of GCA and of the plastic surgical strategies available for reconstruction following radical perineal surgery.
Introduction
Giant Condyloma Acuminata (GCA) of the anorectum are rare lesions. They predominately occur in patients who are immunocompromised. Histologically they are benign tumours, though they behave in a malignant fashion by invading adjacent tissue. They have a tendency to transform into invasive squamous cell carcinoma. They should, therefore, be considered as a potentially fatal condition.
Case Report
A 54 year old man presented with a one week history of passing blood and mucus per rectum associated with weight loss of 10 kg over a period of four weeks. He made no mention of a perineal mass. He had no history of immunodeficiency diseases and took no medication. He was a heavy smoker and drinker and did not appear to look after himself very well. He denied having anoreceptive intercourse. Rectal examination revealed giant anal condylomata (figure 1).
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A biopsy confirmed a diagnosis of condylomata acuminata with areas of severe dysplasia with carcinoma-in-situ. Radiological staging using endorectal ultrasound (figure 2) showed involvement of the internal and external anal sphincters and abdominal and pelvic CT scanning (figure 3) confirmed a large tumour involving the whole length of the anal canal and lower part of the rectum with extension into the ischiorectal fossae.
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Bilateral inguinal lymphadenopathy was present but there were no distant metastases. An examination under anaesthesia revealed a large necrotising tumour with concurrent local sepsis. Discussion at a multidisciplinary team meeting involving colorectal and plastic surgeons and oncologists felt neoadjuvant chemoradiotherapy would be inappropriate due to the presence of local sepsis. An extensive abdominoperineal resection was performed leaving a huge perineal defect (figure 4) which was reconstructed using bilateral myocutaneous gracilis flaps. Histology showed a moderately differentiated squamous cell carcinoma with clearance margins of 11mm distally and 0.2mm circumferentially. As there was a likelihood of remaining tumour, he was referred for consideration of post-operative radiotherapy. Unfortunately this was unavoidably delayed in starting because of prolonged healing of the perineal wound. Five months after the operation, in the week before he was finally due to start radiotherapy, he represented with bleeding from the perineal wound. Subsequent biopsy confirmed persistence of the tumour and a CT scan showed a 6cm tumour mass in the right side of the pelvis involving the prostate, pelvic floor and sidewalls and encasing the internal iliac arteries (figure 5).
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There was no evidence of distant metastases. He received palliative radiotherapy but unfortunately his condition deteriorated rapidly and died shortly afterwards.
Discussion
GCA are rare lesions tending to present in the 5th decade and are two to three times more common in men than women. They are associated with human papilloma virus types 6 and 111,2 and risk factors for their development include anoreceptive intercourse, HIV and immunosuppression. Anorectal GCA may present as an anal mass, with local symptoms such as bleeding, mucus discharge, sepsis, pruritis and altered bowel habit, or with systemic symptoms such as weight loss and poor appetite.3,4 Macroscopically they appear as large, cauliflower-like lesions arising in the perineum. Although microscopically benign, they behave in a malignant fashion, compressing adjacent tissues causing necrosis and giving the impression of invasion2-4 Histologically, GCA is characterized by undulating papillomatosis of well-differentiated keratinised epithelium with lymphocytic infiltration. However, these large lesions may progress to show areas of carcinoma-in-situ or invasive squamous cell carcinoma. As such, they should be considered to represent a spectrum between simple condyloma acuminatum and squamous cell carcinoma.3 Their associated mortality is high, ranging from 20-25% for benign disease, increasing to 50% in the presence of malignant transformation.1,4 Although mortality appears to be declining with time, probably due to increased understanding and medical and surgical advances, the incidence of malignant transformation seems to be increasing 1,3,4 and has been reported from 0.75 to 1.8 per cent.5
Treatment of GCA is problematic due to their rarity and hence lack of clinical experience.6 There are no randomised controlled trials published on its treatment. Prior to starting any treatment, the tumour needs accurate staging using endorectal ultrasound and CT scanning of the abdomen and pelvis. Treatments described range from topical podophyllin, primary chemotherapy or radiotherapy, local excision, or radical surgical procedures such as abdominoperineal resection and pelvic exenteration, with or without neoadjuvant or adjuvant chemoradiotherapy.1,4,6,7 However, best results have been obtained from early, aggressive surgery. Radical surgery of the kind indicated by these lesions inevitably results in the need to reconstruct the perineum, a situation more commonly encountered following surgery for recurrent pelvic malignancy. Small skin wounds may be closed primarily or covered with mesh skin grafts8 whilst larger defects may require myocutaneous flaps. The choices available include the rectus abdominis, gluteal and gracilis myocutaneous flaps. All three provide adequate replacement of excised perineal tissue and excellent healing.9,10 Gracilis flaps are favoured as they are less technically demanding, provide immediate perineal reconstruction in single stage operations and allow good rehabilitation with minimum morbidity and functional deficit.9-13 They have the added benefit of leaving the abdominal wall intact allowing secure stoma sighting.
Most authors agree that chemoradiotherapy should be given preoperatively, as the incidence of early recurrence would appear to be lower than with postoperative chemoradiotherapy.3,6,7,14 Fears of the putative carcinogenic effects of neoadjuvant chemoradiotherapy are not borne out,6 neither are those concerning the disruption of tissue planes, as the lesions themselves distort and destroy the local anatomy. Vigilant surveillance is important post-operatively, as the incidence of early recurrence is high. 1,2
This case illustrates the difficulties and challenges encountered by the surgeon in managing GCA of the anorectum. The tumour was locally advanced at presentation. Pre-operative chemoradiotherapy was deemed inappropriate by the multidisciplinary team in the presence of local sepsis and the negative effect on wound healing. Due to its size, radical surgery was unable to completely excise the tumour and created a large perineal defect which was closed with a gracilis flap. Perineal wound healing was impaired because of tumour persistence, the patient's smoking and drinking habits and poor nutritional status. This led to a delay in post-operative radiotherapy during which time the tumour regrew rapidly and fatally.
Conclusion
GCA should be considered a potentially fatal disease. A multidisciplinary team approach involving colorectal and plastic surgeons, oncologists and stoma therapists is essential for management. Despite aggressive and radical surgical treatment, disease control may still prove difficult or, as in our case, impossible. Palliative treatment may sometimes be the most appropriate course.
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