Perianal Paget disease treated with wide excision and thigh skin flap reconstruction

Abstract

Rationale:Extramammary Peget disease (EMPD) is a rare tumor, which typically occurs in the perianal regions. Perianal Paget disease (PPD) was first reported in 1893, and which has only 180 cases that have been reported in literature. The rarity of the disease means that no large studies have been made, and so the optimal treatment for this disease still remains controversial.Patient concerns:In this case, we reported a 65-years-old female patient with PPD. The patient suffered intermittent pruritus in the perianal region for 1 year. She had neither genitourinary nor gastrointestinal symptoms. Local examination revealed a whitish gray skin lesion in the left perianal area with a 3 × 3 cm size.Diagnoses:The perianal skin biopsy was consistent with EMPD. Then the patient underwent a screening colonoscopy, gynecological ultrasonography, and whole-body computed tomography to exclude underlying malignancy.Interventions:The patient underwent wide local excision with margin control by frozen section examination and posterior thigh flap reconstruction. Subsequently follow-up remains 6 years.Outcomes:The operation was successful. The total operation time was 296 minutes, and the estimated blood loss was 120 mL. The patient recovered without any complication and discharged home on the sixth postoperative day. After 6 years’ follow-up, there was no evidence of recurrence and no influence in the anal bowel control function.Lessons:PPD is a rare disorder; the current knowledge on diagnose and treatment is based on small case series. Thus, it is complicated to elaborate a consensus on diagnostic and treatment guidelines. Wide local excision remains the treatment of choice with a variety of adjuvant therapies. Our method has an advantage which is the posterior thigh flap could be designed in accordance with the defect of the perianal. It is mandatory that the patient must accept a long-term follow-up to detect local recurrence and to distant carcinoma.

Extramammary Paget’s disease of the perianal area: a case report and the summarizing review of last 5-year literature.

“…Secondly, the usually affected age is considered a 6th-7th decade [1][2][3][4][5][6]. As a witness, we found only 5 cases (10,2%) with age less than 60: mean 67,68±1,64; median 67 years.…”

“…EMPD is one of the rarest malignant tumors without accurate epidemiological data. There are some studies with numbers of cases from 100 up to 200 [2,8], but a predominance of published data is devoted just case report. We analyzed and summarized reported information about all published in PubMed (https://www.ncbi.…”

“…Despite the official fact of discovering and coming out EMPD like separate disease took place more than one century ago (in 1889 by Radcliff Crocker) [1], accurate its accidence is unknown owing to this tumor rarity. Up to 200 cases just have been reported in the literature to refer to Shen K et al [2], and only several countries have looked into this problem, for instance, the USA and Japan [3,4].…”

“…However, in the last few years, this theme is generally sensational in the scientist publishing sphere. But it should be mentioned that in most articles only single or pair of rare cases in different locations is performed and there is a lack of summarizing data [2,5,6]. On the other hand, given that __________________________________________________________________________________ MORPHOLOGIA • 2019 • Том 13 • № 3…”

Perianal Paget’s disease: one century later and still a challenge

“…The diagnosis is established by skin biopsy and histological identification of Paget cells in the epidermis,2 7 essential to differentiate from other mimicking diseases like Bowen disease, cutaneous superficial spreading melanoma and dermatitis. Surgical treatment remains the best available option, ranging from wide local excision to abdominoperineal resection, depending on invasiveness 8.…”