Abstract
Perianal Paget’s disease (PPD) is a rare intraepithelial adenocarcinoma. The clinical appearance of PPD may be confused with dermatitis and fungal infections of the skin, resulting in delayed diagnosis, sometimes for years. It may also be associated with secondary malignancies. Surgery and non-surgical methods such as radiotherapy, immunomodulating drugs, and chemotherapy are used in the treatment of PPD. In this case report, two patients with PPD are presented; the first was treated with surgical excision and radiotherapy and the second did not accept surgical or medical therapy. Although the lesion deepened and expanded towards adjacent structures, no secondary malignancy or metastasis was observed.
Similar content being viewed by others
References
Paget J (1971) On disease of the mammary areola preceding cancer of the mammary gland. CA: A Cancer J for Clin 21:303–304
Darier J (1893) Sur un cas de maladie de Paget de la region perineo-anale et scrotale. Ann Dermatol Syph 4:33–39
Isik O, Aytac E, Brainard J et al (2016) Perianal Paget’s disease: three decades experience of a single institution. Int J Colorectal Dis 31:29–34
Kanitakis J (2007) Mammary and extramammary Paget’s disease. J Eur Acad Dermatol Venereol 21:581–590
Apikotoa S, Stein J (2022) Stage 3 perianal extra mammary Paget’s disease: a case report of a non-operative approach. Int J Surg Case Rep 91:106796
Cassol OS, Pastorello J, Oliveira Bd et al (2019) Perianal Paget’s disease. J Coloproctology (Rio de Janeiro) 39:262–264
Coldiron BM, Goldsmith BA, Robinson JK (1991) Surgical treatment of extramammary Paget’s disease. A report of six cases and a reexamination of Mohs micrographic surgery compared with conventional surgical excision. Cancer 67:933–938
Lam C, Funaro D (2010) Extramammary Paget’s disease: summary of current knowledge. Dermatol Clin 28:807–826
Morris CR, Hurst EA (2020) Extramammary Paget disease: a review of the literature—part I: history, epidemiology, pathogenesis, presentation, histopathology, and diagnostic work-up. Dermatol Surg 46:151–158
Jones RE Jr, Austin C, Ackerman AB (1979) Extramammary Paget’s disease. A critical reexamination. Am J Dermatopathol 1:101–132
Kang Z, Zhang Q, Zhang Q et al (2015) Clinical and pathological characteristics of extramammary Paget’s disease: report of 246 Chinese male patients. Int J Clin Exp Pathol 8:13233
Devaji Rao S, Govindarajan M (2017) Extramammary Paget’s disease of the perianal region. Indian J Surg 79:360–362
Lloyd J, Flanagan A (2000) Mammary and extramammary Paget’s disease. J Clin Pathol 53:742–749
Smith KJ, Tuur S, Corvette D et al (1997) Cytokeratin 7 staining in mammary and extramammary Paget’s disease Modern pathology an official. J United States Canadian Acad Pathol Inc 10:1069–1074
Kyriazanos ID, Stamos NP, Miliadis L et al (2011) Extra-mammary Paget’s disease of the perianal region: a review of the literature emphasizing the operative management technique. Surg Oncol 20:e61–e71
Godbole C, Mehta J, Methil B et al (2017) Perianal Paget’s disease—a case report and a review of current diagnosis and management. Indian J Surg Oncol 8:619–621
Sarmiento JM, Wolff BG, Burgart LJ et al (1997) Paget’s disease of the perianal region—an aggressive disease? Dis Colon Rectum 40:1187–1194
Kim CW, Kim YH, Cho MS et al (2014) Perianal Paget’s disease. Ann coloproctology 30:241
Tsutsumida A, Yamamoto Y, Minakawa H et al (2003) Indications for lymph node dissection in the treatment of extramammary Paget’s disease. Dermatol Surg 29:21–24
Hendi A, Brodland DG, Zitelli JA (2004) Extramammary Paget’s disease: surgical treatment with Mohs micrographic surgery. J Am Acad Dermatol 51:767–773
Lam DT, Batista O, Weiss EG et al (2001) Staged excision and split-thickness skin graft for circumferential perianal Paget’s disease. Dis Colon Rectum 44:868–870
Shutze WP, Gleysteen JJ (1990) Perianal Paget’s disease. Dis Colon Rectum 33:502–507
Guidi GJ, de Sousa JP, Marques R et al (2021) Perianal Paget’s disease: one century later and still a challenge. BMJ Case Reports CP 14:e240435
Grossi U, Santoro GA, Antoniazzi E et al (2021) Bilateral lotus petal flap reconstruction for perianal Paget’s disease. Tech Coloproctol 25:141–142
Cohen PR, Schulze KE, Tschen JA et al (2006) Treatment of extramammary Paget disease with topical imiquimod cream: case report and literature review. South Med J 99:396–403
Simonds RM, Segal RJ, Sharma A (2019) Extramammary Paget’s disease: a review of the literature. Int J Dermatol 58:871–879
Hirai I, Funakoshi T (2017) Modified weekly regimen of cisplatin, epirubicin and paclitaxel induced a durable response in two cases of metastatic extramammary Paget’s disease. J Dermatol 44:1148–1151
Kato J, Hida T, Yamashita T et al (2018) Successful TS-1 monotherapy as the second-line treatment for advanced extramammary Paget’s disease: a report of two cases. J Dermatol 45:80–82
Shim PJ, Zeitouni NC (2020) Photodynamic therapy for extramammary Paget’s disease: a systematic review of the literature. Photodiagnosis Photodyn Ther 31:101911
Besa P, Rich TA, Delclos L et al (1992) Extramammary Paget’s disease of the perineal skin: role of radiotherapy. Int J Rad Oncology *Bio* Physics 24:73–78
Demiral S, Sager O, Dincoglan F et al (2013) Radiotherapy for perianal extramammary Paget’s disease: a case report. Gulhane Med J 55:310–313
Karam A, Dorigo O (2014) Increased risk and pattern of secondary malignancies in patients with invasive extramammary Paget disease. Br J Dermatol 170:661–671
Author information
Authors and Affiliations
Corresponding author
Ethics declarations
Competing Interests
The authors declare no competing interests.
Additional information
Publisher's Note
Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.
Rights and permissions
About this article
Cite this article
Özdemir, Ü., Salış, M., Özer, İ. et al. Perianal Paget’s Disease; Surgery and Non-surgical Interventions, Untreated Patient: a Report of Two Cases. Indian J Surg 85, 169–174 (2023). https://doi.org/10.1007/s12262-022-03376-6
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s12262-022-03376-6