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Extramammary Paget Disease Workup

  • Author: Neil Sandhu, MD; Chief Editor: Dirk M Elston, MD  more...
 
Updated: Oct 03, 2014
 

Approach Considerations

The diagnosis of extramammary Paget disease (EMPD) requires a high degree of clinical suspicion followed by skin biopsy with pathologic correlation. Initially, a detailed review of systems and physical examination should be performed in all patients. The examination should include the following:

  • Full skin examination
  • Palpation of all lymph nodes
  • Rectal examination
  • Sigmoidoscopy
  • Cystoscopy

Additionally, women require pelvic examination with a Papanicolaou test, breast examination, and colposcopy.

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Imaging Studies

Imaging studies in EMPD should be directed by the anatomic location of the involved skin and the sex of the patient. Imaging studies should be used to augment physical and endoscopic examination in assessing possible undetected internal malignancy.[2]

Positron emission tomography (PET) may be helpful in assessing regional lymph nodes and locating distal disease, especially in patients with dermal invasion noted on initial skin biopsy specimens.[4]

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Skin Biopsy and Histologic Findings

Because EMPD extends beyond the visibly involved margins, obviously involved skin should be examined by using transverse frozen sections or serial vertical sections. Perform skin biopsy to evaluate possible EMPD in patients in whom ongoing therapy is ineffective. See the image below.

Photomicrograph of malignant melanoma in situ of s Photomicrograph of malignant melanoma in situ of skin displays prominent intraepidermal pagetoid spread. Note that melanoma cells are present in all layers of epidermis, mostly in single units. Cytoplasm of melanoma cells is vacuolated. Moderate upper dermal chronic inflammatory infiltrate is present (hematoxylin-eosin, original magnification ×250). S-100 protein and homatropine methylbromide immunostains are positive in melanoma cells, whereas carcinoembryonic antigen is negative. No epithelial mucin is seen in these tumor cells.

The epidermis is diffusely infiltrated with large vacuolated cells that have a bluish cytoplasm; these are called Paget cells. These distinctive cells are found in the lower epidermis and may proliferate to the rete ridges and adnexa. The epidermis shows varying degrees of acanthosis, hyperkeratosis, and parakeratosis. With histochemical analysis, Paget cells are stained with sialomucin by using periodic acid–Schiff (PAS) followed by diastase digestion.

It is important to keep in mind the differential diagnosis of tumors with an epidermotropic growth pattern and the importance of immunohistochemical staining in the histologic workup of such tumors. The following should all be considered[5] :

  • Squamous cell carcinoma in situ
  • Melanoma
  • Mycosis fungoides
  • Eccrine porocarcinoma
  • Sebaceous carcinoma of the eyelid
  • Mammary Paget disease (PD) and EMPD
  • Merkel cell carcinoma
  • Epidermotropic metastases

Cytokeratin 20 (CK20) and BRST-2 are both positive in large subsets of primary and secondary EMPD. Using HER2/neu and CDX2 may be beneficial to distinguish primary EMPD from secondary EMPD due to anorectal adenocarcinoma but not due to urothelial or prostatic malignancy.[6]

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Contributor Information and Disclosures
Author

Neil Sandhu, MD Dermatologist (Medical/Cosmetics) and Mohs Surgeon, Gulf Coast Dermatology

Neil Sandhu, MD is a member of the following medical societies: American Academy of Dermatology, American Society for Dermatologic Surgery, American Society for MOHS Surgery

Disclosure: Nothing to disclose.

Coauthor(s)

Robert A Schwartz, MD, MPH Professor and Head of Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, Rutgers New Jersey Medical School; Visiting Professor, Rutgers University School of Public Affairs and Administration

Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, New York Academy of Medicine, American Academy of Dermatology, American College of Physicians, Sigma Xi

Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD Professor and Chairman, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina College of Medicine

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Acknowledgements

Marjan Garmyn, MD, PhD Professor, Faculty of Medicine, Katholieke Universiteit Leuven, Belgium; Chair and Adjunct Head, Department of Dermatology, University of Leuven, Belgium

Disclosure: Nothing to disclose.

Michael J Wells, MD Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center, Paul L Foster School of Medicine

Michael J Wells, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, and Texas Medical Association

Disclosure: Nothing to disclose.

Joseph L Wilde, MD Chief, Department of Dermatology, Vicenza Army Health Center, Italy

Joseph L Wilde, MD is a member of the following medical societies: American College of Mohs Micrographic Surgery and Cutaneous Oncology

Disclosure: Nothing to disclose.

References
  1. Roy J, Mirnezami A, Gatt M, Sasapu K, Scott N, Sagar P. A rare case of Paget's disease in a retrorectal dermoid cyst. Colorectal Dis. 2009 Nov 2. [Medline].

  2. Chanda JJ. Extramammary Paget's disease: prognosis and relationship to internal malignancy. J Am Acad Dermatol. 1985 Dec. 13(6):1009-14. [Medline].

  3. Bagby CM, MacLennan GT. Extramammary Paget's disease of the penis and scrotum. J Urol. 2009 Dec. 182(6):2908-9. [Medline].

  4. Cho SB, Yun M, Lee MG, Chung KY. Variable patterns of positron emission tomography in the assessment of patients with extramammary Paget's disease. J Am Acad Dermatol. 2005 Feb. 52(2):353-5. [Medline].

  5. D'Agostino M, Cinelli C, Willard R, Hofmann J, Jellinek N, Robinson-Bostom L. Epidermotropic Merkel cell carcinoma: a case series with histopathologic examination. J Am Acad Dermatol. 2010 Mar. 62(3):463-8. [Medline].

  6. Perrotto J, Abbott JJ, Ceilley RI, Ahmed I. The Role of Immunohistochemistry in Discriminating Primary From Secondary Extramammary Paget Disease. Am J Dermatopathol. 2010 Jan 4. [Medline].

  7. Beleznay KM, Levesque MA, Gill S. Response to 5-fluorouracil in metastatic extramammary Paget disease of the scrotum presenting as pancytopenia and back pain. Curr Oncol. 2009 Sep. 16(5):81-3. [Medline]. [Full Text].

  8. Sendagorta E, Herranz P, Feito M, et al. Successful treatment of three cases of primary extramammary Paget's disease of the vulva with Imiquimod - proposal of a therapeutic schedule. J Eur Acad Dermatol Venereol. 2009 Oct 15. [Medline].

  9. Marchitelli C, Peremateu MS, Sluga MC, et al. Treatment of primary vulvar paget disease with 5% imiquimod cream. J Low Genit Tract Dis. 2014 Oct. 18(4):347-50. [Medline].

  10. Takahagi S, Noda H, Kamegashira A, et al. Metastatic extramammary Paget's disease treated with paclitaxel and trastuzumab combination chemotherapy. J Dermatol. 2009 Aug. 36(8):457-61. [Medline].

  11. Cecchi R, Pavesi M, Bartoli L, Brunetti L, Rapicano V. Perineal extramammary Paget disease responsive to topical imiquimod. J Dtsch Dermatol Ges. 2010 Jan. 8(1):38-40. [Medline].

  12. Appert DL, Otley CC, Phillips PK, Roenigk RK. Role of multiple scouting biopsies before Mohs micrographic surgery for extramammary Paget's disease. Dermatol Surg. 2005 Nov. 31(11 Pt 1):1417-22. [Medline].

  13. Coldiron BM, Goldsmith BA, Robinson JK. 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. 1991 Feb 15. 67(4):933-8. [Medline].

  14. Hendi A, Brodland DG, Zitelli JA. Extramammary Paget's disease: surgical treatment with Mohs micrographic surgery. J Am Acad Dermatol. 2004 Nov. 51(5):767-73. [Medline].

 
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Photomicrograph of malignant melanoma in situ of skin displays prominent intraepidermal pagetoid spread. Note that melanoma cells are present in all layers of epidermis, mostly in single units. Cytoplasm of melanoma cells is vacuolated. Moderate upper dermal chronic inflammatory infiltrate is present (hematoxylin-eosin, original magnification ×250). S-100 protein and homatropine methylbromide immunostains are positive in melanoma cells, whereas carcinoembryonic antigen is negative. No epithelial mucin is seen in these tumor cells.
 
 
 
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