Yuichi Yoshida1, Hiroyuki Goto1, Reiko Tsutsumi1, Osamu Yamamoto1. 1. Department of Medicine of Sensory and Motor Organs, Division of Dermatology, Faculty of Medicine, Tottori University, Yonago, Japan. E-mail: yxyhifu1@grape.med.tottori-u.ac.jp.
Sir,Extramammary Paget's disease is a rare malignant skin neoplasm arising in areas rich in apocrine glands, e. g., vulva, scrotum, perianal area, and axilla.[1] Surgery is the first-line treatment for extramammary Paget's disease.[2] However, it is not easy to excise a tumor that has developed in the perianal region. Herein, we report three cases of perianal Paget's disease that were successfully treated with an anal retractor device.Case 1 – A 67-year-old male was referred to us for evaluation of a 2-year history of an erythematous plaque in his left anal region [Figure 1a]. A skin biopsy revealed proliferation of Paget's cells within the epidermis [Figure 1b]. Since there was no evidence of colorectal cancer by colonoscopy, a diagnosis of primary extramammary Paget's disease was made. No distant metastasis was found by a computed tomography scan. We set a plastic retractor around the tumor and then secured a field of view over a deep surgical site by putting elastic stays into the slot of the retractor [Figure 1c]. The lesion was excised and reconstruction was performed with a split-thickness skin graft [Figure 1d and e].
Figure 1
(a) An erythematous plaque in the left anal region (case 1). (b) Proliferation of Paget's cells within the epidermis (H and E, ×200). (c) Use of an anal retractor device with elastic stays at the time of surgery. (d) The lesion after excision. (e) Reconstruction with a split-thickness skin graft
(a) An erythematous plaque in the left anal region (case 1). (b) Proliferation of Paget's cells within the epidermis (H and E, ×200). (c) Use of an anal retractor device with elastic stays at the time of surgery. (d) The lesion after excision. (e) Reconstruction with a split-thickness skin graftCase 2 – A 66-year-old male was referred to us for evaluation of a 1-year history of a perianal erythematous lesion [Figure 2a]. Colonoscopy and a computed tomography scan showed no evidence of colorectal cancer. Based on the histopathological examination, a diagnosis of primary extramammary Paget's disease was made. Using the device and elastic hooks, we could pull out and excise the lesion in the anal canal with a 1-cm margin under general anesthesia [Figure 2b]. Reconstruction was then performed with a split-thickness skin graft. The resection margins were free of tumor cells.
Figure 2
(a) A perianal erythematous lesion (case 2). (b) Tumor resection with the use of an anal retractor device
(a) A perianal erythematous lesion (case 2). (b) Tumor resection with the use of an anal retractor deviceCase 3 – A 72-year-old male was referred to us for evaluation of an erythematous plaque with erosion around the anus [Figure 3a]. A diagnosis of primary extramammary Paget's disease was made. The lesion was also surgically excised with the device, and reconstruction was performed with a split-thickness skin graft. There was no recurrence or distant metastasis for 3 years after surgery [Figure 3b].
Figure 3
(a) An erythematous plaque with erosion around the anus (case 3). (b) Result of reconstruction with a split-thickness skin graft 3 years after surgery
(a) An erythematous plaque with erosion around the anus (case 3). (b) Result of reconstruction with a split-thickness skin graft 3 years after surgeryExtramammary Paget's disease accounts for <1% of all anal diseases.[3] Surgical excision is a gold standard treatment for extramammary Paget's disease,[4] but a wide local excision of a perianal lesion is a therapeutic challenge. Although an anal retractor device is commonly used for perianal surgery, there has been no report of its use in perianal Paget's disease. The device enables excellent viewing of the mucosal lesion by adjusting tension. By the device, it was easier for a surgeon to accurately evaluate the tumor border in mucosal sites without the help of assistants in the prone position.However, care is needed when hooking elastic stays, since it has been reported that colorectal cancer occasionally recurred at the insertion site of elastic stays.[5]In conclusion, an anal retractor device is helpful for achieving complete removal of perianal Paget's disease by securing an operation field in mucosal sites at the time of surgery
Authors: Giuseppe Carbotta; Pierluca Sallustio; Antonio Prestera; Rita Laforgia; Pierluigi Lobascio; Nicola Palasciano Journal: Ann Med Surg (Lond) Date: 2016-06-21