Warning: fopen(/home/virtual/colon/journal/upload/ip_log/ip_log_2024-11.txt): failed to open stream: Permission denied in /home/virtual/lib/view_data.php on line 95 Warning: fwrite() expects parameter 1 to be resource, boolean given in /home/virtual/lib/view_data.php on line 96 Perianal Paget's Disease
Skip Navigation
Skip to contents

Ann Coloproctol : Annals of Coloproctology

OPEN ACCESS
SEARCH
Search

Articles

Page Path
HOME > Ann Coloproctol > Volume 30(5); 2014 > Article
Case Report
Perianal Paget's Disease
Chang Woo Kim, Yon Hee Kim1, Min Soo Cho, Byung Soh Min, Seung Hyuk Baik, Nam Kyu Kim
Annals of Coloproctology 2014;30(5):241-244.
DOI: https://doi.org/10.3393/ac.2014.30.5.241
Published online: October 28, 2014

Division of Colon and Rectal Surgery, Department of Surgery, Severance Hospital, Yonsei University College of Medicine, Seoul, Korea.

1Department of Pathology, Severance Hospital, Yonsei University College of Medicine, Seoul, Korea.

Correspondence to: Seung Hyuk Baik, M.D. Division of Colon and Rectal Surgery, Department of Surgery, Severance Hospital, Yonsei University College of Medicine, 50 Yonsei-ro, Seodaemun-gu, Seoul 120-752, Korea. Tel: +82-2-2228-2100, Fax: +82-2-313-8289, whitenoja@yuhs.ac
• Received: March 31, 2014   • Accepted: June 2, 2014

© 2014 The Korean Society of Coloproctology

This is an open-access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

prev
  • 3,882 Views
  • 69 Download
  • 5 Web of Science
  • 9 Crossref
  • 6 Scopus
  • The incidence of extramammary Paget's disease (EMPD) is very low. An 84-year-old Korean man was treated with topical and oral medications at a local dermatologic clinic for a year, but the symptoms did not improve. He visited Severance Hospital and underwent a perianal skin biopsy and was finally diagnosed with EMPD. The authors performed a wide local excision according to a 1-cm margin around the lesion. For the skin and the soft tissue defects, bilateral inferior gluteal artery perforator flap transpositions were performed. The size of the lesion was 14 cm2 × 9 cm2, and the lateral and the basal margins were all disease free.
Perianal Paget's disease (PPD) is a subgroup of extramammary Paget's disease (EMPD), which is an uncommon intraepithelial neoplasm. The rate of PPD associated with malignancy ranges from 33% to 86% [1]. The true incidence of the disease is difficult to estimate due to its rarity, but it is known to represent less than 1% of all anal diseases and 6.5% of all cases of Paget's disease [2]. The authors report a case of a patient with PPD.
Patient
An 84-year-old Korean man with hypertension and a history of appendectomy presented with perianal pruritus and a rash. He was treated with topical and oral medications at a local dermatologic clinic for a year, but his clinical symptoms did not improve. He visited Severance Hospital and underwent a perianal skin biopsy, and was finally diagnosed with EMPD. The 6.5 cm2 × 8 cm2-sized skin lesion was oval shaped and located from under the coccygeal area to the perineal body. The lesion abutted the anoderm 1 cm from the anal verge, but had not invaded the penis, testes, or both thighs. Abdomino-pelvic computed tomography and rectal magnetic resonance imaging were performed to evaluate preoperative staging and to detect other diseases. They showed no abnormalities, including no regional and inguinal lymph-node enlargement. A colonoscopy showed no intraluminal abnormalities. Thus, the authors decided to perform a wide local excision and to make flaps for the large area of skin and the soft tissue defects.
Surgery
The patient received a bowel preparation with polyethylene glycol one day before surgery because of the possibility of conversion to an abdominoperineal excision. He was placed in a jack-knife position on the table (Fig. 1A). Surgical margins 1 cm around the main lesion were demarcated based on a preoperative multiple-punch biopsy at eight locations which were all proven to be basal pigmentation or eczematous change. A wide local excision was performed (Fig. 1B). The size of the lesion was 6.5 cm2 × 8 cm2. However, we carefully resected the lesion over the demarcation because the patient was too old to tolerate the time required for the frozen biopsy to confirm the proper margins. As a result, the final size of the specimen was 9 cm × 14 cm (Fig. 1C). For the skin and the soft tissue defects, bilateral inferior gluteal artery perforator flap transpositions were performed (Fig. 1D). Two closed suction drains were inserted at both flap sites. Repair and reinforcement of the dentate line with the skin were performed. A 28-Fr rectal tube was inserted into the anal canal. Aseptic dressing was done, and the patient was sent to the recovery room without any events. The total operation time was 182 minutes, and the estimated blood loss was 20 mL.
Postoperative course
The patient recovered without any complications. He sustained the prone position for the integrity of flaps and was kept on nil per os (nothing by mouth) with intravenous nutritional support alone for 12 days after surgery to prevent wound infection from the stool. The right drain was removed at postoperative day (POD) #6 whereas the left drain was removed at POD #12. The rectal tube was removed at POD #7. The patient started his diet at POD #13 without any problems. The pathology was proven as EMPD. The immunohistochemistry was positive for cytokeratin 7 (CK7), carcinoembryonic antigen, and C-erb B2 while it was negative for HMB-45 (Fig. 2). The lateral and the basal margins were all disease free (0.7 cm and 0.1 cm, respectively). The patient was discharged at POD #20.
Darier and Couillaud reported the first case of PPD 19 years after the first report of Paget's disease by Sir James Paget in 1874 [3]. Fewer than 200 cases of PPD have been reported in the literature, and the true incidence is difficult to estimate. PPD has also rarely been reported in Korea. Fewer than 20 articles on EMPD have been in Korea; moreover, no article on PPD has been found until now. A multicenter study on EMPD in Korea revealed only seven cases (3.6%) of PPD during 5 years [4].
The diagnosis of PPD can be obtained by using a histological examination showing the presence of Paget cells. In addition, CK7, CK19, and C-erb B2 are favorable immunohistochemical markers for the diagnosis of EMPD [5, 6]. However, it can be misdiagnosed easily without a histological confirmation due to its rarity and nonspecific clinical manifestation, which include exfoliative, exudative, verrucous, or hypopigmented patches that are accompanied by pruritus or burning sensations, which ultimately mimic treatment-refractory eczema [4]. In the present case, the first impression at a local clinic was eczema and dermatitis. A treatment of topical steroid and oral antihistamine was applied, but was ineffective for a year. If a conventional treatment for general dermatitis is not effective for a certain period, PPD should be suspected, and a biopsy of the lesion should be considered.
Reaching a consensus on the treatment for PPD is difficult because PPD is rare, and most of the reports in the literature are case reports. Some authors have suggested nonsurgical treatment first. Kobayashi et al. [7] reported the effect of intralesional interferon. Cohen et al. [8] reported the results of the topical imiquimod in 9 cases of EMPD. Guerrieri and Back [9] suggested radiotherapy to control locally the disease for selected cases. However, many authors have recommended surgical treatment, including a wide local excision through abdominoperineal excision, for PPD. Shutze and Gleysteen [10] established a staging classification of PPD and recommended several treatment options. The present case corresponds to stage I according to their classification: Paget's cells found in the perianal epidermis and adnexae without a primary carcinoma. The recommendation for stage I and IIA is a wide local excision.
A wide local excision was performed safely after the demarcation of a margin of 1 cm based on a skin-punch biopsy. A safe resection margin of 2 cm is often recommended, although a clear margin of 1 cm could be safe for EMPD [1, 11]. The final pathologic report confirmed disease-free lateral and basal margins after the wide local excision. Skin or a muscle flap is usually used for skin and soft tissue defects. Shutze and Gleysteen [10] described the defect as being covered with skin flaps for the skin and the defects in their two cases of PPD.
Long-term follow-up of patients with EMPD is important because of the possibility of recurrence or development of an associated cancer. Our patient recovered and was discharged without any complications. We are following up on the patient, and he has been alive for 3 months without relapse. A multicenter study on EMPD in Korea demonstrated that the clinical features of EMPD were consistent with reports from other Asian population-based studies, but the study showed that the rate of association with malignancy (14.4%) in the Korean population was higher than that observed in other Asian, such as 3%-5% in China [4].

No potential conflict of interest relevant to this article was reported.

  • 1. Kanitakis J. Mammary and extramammary Paget's disease. J Eur Acad Dermatol Venereol 2007;21:581–590. ArticlePubMed
  • 2. Kyriazanos ID, Stamos NP, Miliadis L, Noussis G, Stoidis CN. Extra-mammary Paget's disease of the perianal region: a review of the literature emphasizing the operative management technique. Surg Oncol 2011;20:e61–e71. ArticlePubMed
  • 3. Classic articles in colonic and rectal surgery: Sir James Paget, 1814-1899, on disease of the mammary areola preceeding cancer of the mammary gland. Dis Colon Rectum 1980;23:280–281. ArticlePubMed
  • 4. Lee SJ, Choe YS, Jung HD, Ahn SK, Cha YC, Cho KH, et al. A multicenter study on extramammary Paget's disease in Korea. Int J Dermatol 2011;50:508–515. ArticlePubMed
  • 5. Miyamoto A, Akasaka K, Oikawa H, Akasaka T, Masuda T, Maesawa C. Immunohistochemical study of HER2 and TUBB3 proteins in extramammary Paget disease. Am J Dermatopathol 2010;32:578–585. ArticlePubMed
  • 6. Hikita T, Ohtsuki Y, Maeda T, Furihata M. Immunohistochemical and fluorescence in situ hybridization studies on noninvasive and invasive extramammary Paget's disease. Int J Surg Pathol 2012;20:441–448. ArticlePubMed
  • 7. Kobayashi H, Someda Y, Furukawa M, Chanoki M, Hamada T. Intralesional interferon in the treatment of extramammary Paget's disease. Nihon Hifuka Gakkai Zasshi 1987;97:1–7. PubMed
  • 8. Cohen PR, Schulze KE, Tschen JA, Hetherington GW, Nelson BR. Treatment of extramammary Paget disease with topical imiquimod cream: case report and literature review. South Med J 2006;99:396–402. ArticlePubMed
  • 9. Guerrieri M, Back MF. Extramammary Paget's disease: role of radiation therapy. Australas Radiol 2002;46:204–208. ArticlePubMed
  • 10. Shutze WP, Gleysteen JJ. Perianal Paget's disease. Classification and review of management: report of two cases. Dis Colon Rectum 1990;33:502–507. ArticlePubMed
  • 11. Murata Y, Kumano K. Extramammary Paget's disease of the genitalia with clinically clear margins can be adequately resected with 1 cm margin. Eur J Dermatol 2005;15:168–170. PubMed
Fig. 1
Position of the patient (A), after the wide local excision (B), the surgical specimen (C), and after the skin-flap transpositions (D).
ac-30-241-g001.jpg
Fig. 2
Immunohistochemistry examinations: (A) H&E (×100), (B) cytokeratin 7 (×100), (C) carcinoembryonic antigen (×100), (D) C-erb B2 (×100), (E) HMB-45 (×100), and (F) mucicarmine (×100).
ac-30-241-g002.jpg

Figure & Data

References

    Citations

    Citations to this article as recorded by  
    • Rectal Adenocarcinoma With Pagetoid Spread: A Novel Entity
      Lefika Bathobakae, Pasha Shenasan, Aakash Trivedi, Ruhin Yuridullah, Sohail Qayyum, Abraham El-Sedfy, Hirotada Akiho
      Case Reports in Gastrointestinal Medicine.2024;[Epub]     CrossRef
    • Perianal Paget’s Disease; Surgery and Non-surgical Interventions, Untreated Patient: a Report of Two Cases
      Ümit Özdemir, Mustafa Salış, İlter Özer, Bülent Ünal
      Indian Journal of Surgery.2022;[Epub]     CrossRef
    • Perianal paget’s disease: A case report
      Hernández Pilar Navajas, Delgado Teresa Valdés, Aguado Jesús Machuca, González-Cámpora Ricardo, Arias Federico Argüelles
      Archives of Clinical Gastroenterology.2022; 8(3): 050.     CrossRef
    • Perianal Paget's disease
      Pilar Navajas Hernández, Teresa Valdés Delgado , Jesús Machuca Aguado, Ricardo González-Cámpora, Federico Argüelles Arias
      Revista Española de Enfermedades Digestivas.2022;[Epub]     CrossRef
    • Enfermedad de Paget perianal
      Mireia Merichal Resina, Carlos Cerdan Santacruz, Enrique Sierra Grañón, Jordi Antoni Tarragona Foradada, Jorge Juan Olsina Kissler
      Cirugía Española.2019; 97(3): 179.     CrossRef
    • Perianal Paget Disease
      Mireia Merichal Resina, Carlos Cerdan Santacruz, Enrique Sierra Grañón, Jordi Antoni Tarragona Foradada, Jorge Juan Olsina Kissler
      Cirugía Española (English Edition).2019; 97(3): 179.     CrossRef
    • Enfermedad de Paget extramamaria de la región perianal, reporte de caso
      Lucía Giraldo Mordecay, Joanna Cajamarca Rueda, Edgar Olmos Olmos, Jenny Gonzalez Arboleda
      Piel.2017; 32(3): 178.     CrossRef
    • Bilateral V-Y flap for a perianal basal cell carcinoma: A case report
      José P. Rivera-Chavarría, Francisco Vargas-Villalobos, Silvia Riggioni-Víquez
      International Journal of Surgery Case Reports.2016; 24: 153.     CrossRef
    • Tumours and inflammatory lesions of the anal canal and perianal skin revisited: an update and practical approach
      Heather Dawson, Stefano Serra
      Journal of Clinical Pathology.2015; 68(12): 971.     CrossRef

    • PubReader PubReader
    • Cite this Article
      Cite this Article
      export Copy Download
      Close
      Download Citation
      Download a citation file in RIS format that can be imported by all major citation management software, including EndNote, ProCite, RefWorks, and Reference Manager.

      Format:
      • RIS — For EndNote, ProCite, RefWorks, and most other reference management software
      • BibTeX — For JabRef, BibDesk, and other BibTeX-specific software
      Include:
      • Citation for the content below
      Perianal Paget's Disease
      Ann Coloproctol. 2014;30(5):241-244.   Published online October 28, 2014
      Close
    • XML DownloadXML Download
    Figure
    • 0
    • 1
    Perianal Paget's Disease
    Image Image
    Fig. 1 Position of the patient (A), after the wide local excision (B), the surgical specimen (C), and after the skin-flap transpositions (D).
    Fig. 2 Immunohistochemistry examinations: (A) H&E (×100), (B) cytokeratin 7 (×100), (C) carcinoembryonic antigen (×100), (D) C-erb B2 (×100), (E) HMB-45 (×100), and (F) mucicarmine (×100).
    Perianal Paget's Disease

    Ann Coloproctol : Annals of Coloproctology Twitter Facebook
    TOP