Extramammary Paget Disease Workup

  • Author: Neil Sandhu, MD; Chief Editor: Dirk M Elston, MD   more...
 
Updated: Oct 20, 2011
 

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.

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  Resident Physician, Department of Dermatology, University of Medicine and Dentistry of New Jersey-New Jersey Medical School

Neil Sandhu, MD is a member of the following medical societies: American Dermatological Association, American Medical Association, American Medical Student Association/Foundation, American Roentgen Ray Society, American Society of Dermatology, and Radiological Society of North America

Disclosure: Nothing to disclose.

Coauthor(s)

Robert A Schwartz, MD, MPH  Professor and Head, Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, University of Medicine and Dentistry of New Jersey-New Jersey Medical School

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

Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD  Director, Ackerman Academy of Dermatopathology, New York

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

Disclosure: Nothing to disclose.

Additional Contributors

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. Nov 2 2009;[Medline].

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

  3. Bagby CM, MacLennan GT. Extramammary Paget's disease of the penis and scrotum. J Urol. Dec 2009;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. Feb 2005;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. Mar 2010;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. Jan 4 2010;[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. Sep 2009;16(5):81-3. [Medline].

  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. Oct 15 2009;[Medline].

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

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

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

  12. 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. Feb 15 1991;67(4):933-8. [Medline].

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

 
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