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Perianal Paget's disease: distinguishing primary and secondary lesions using immunohistochemical studies including gross cystic disease fluid protein-15 and cytokeratin 20 expression.
Arch Pathol Lab Med 1998; 122(12):1077-81AP

Abstract

BACKGROUND

Extramammary Paget's disease most commonly occurs on the female external genitalia and rarely occurs in the perianal region and male external genitalia. We present the clinical and pathologic features of 5 cases of perianal Paget's disease and review the literature.

METHODS

Clinical and pathologic data were recorded for 5 cases of perianal Paget's disease. Cases were studied retrospectively with special stains, including periodic acid-Schiff, mucicarmine, Alcian blue, carcinoembryonic antigen, S100 protein, pan-keratin, gross cystic disease fluid protein-15 (GCDFP-15), lysozyme, CD15 (Leu-M1), cytokeratin 7 (CK7), and cytokeratin 20 (CK20).

RESULTS

Three (60%) of 5 patients had concurrent rectal adenocarcinomas. All cases reacted positively for pankeratin, although the intensity and distribution of staining varied. Both cases not associated with an underlying carcinoma showed strong GCDFP-15 and CK7 expression and an absence of CK20 expression. The 3 cases associated with an underlying malignancy demonstrated CK7 and CK20 expression and an absence of GCDFP-15 expression. All cases were negative for lysozyme and CD15 (Leu-M1).

CONCLUSIONS

The 5 cases reported herein demonstrate that perianal Paget's disease is a heterogeneous entity. The high frequency of associated underlying malignancies and resultant poor clinical outcomes highlight the importance of an aggressive search for a second malignancy. In some cases, perianal Paget's disease merely represents a cutaneous manifestation of an underlying rectal adenocarcinoma and demonstrates a CK7+/CK20+/GCDFP-15-/lysozyme-/Leu-M1- immunophenotype and signet ring Paget's cells. Other cases represent primary adenocarcinomas of the skin, which are associated with a CK7+/CK20-/GCDFP-15+/lysozyme /Leu-M1- immunophenotype and an excellent prognosis if adequately resected. Immunohistochemical studies, particularly CK20 and GCDFP-15, are useful adjuncts in distinguishing primary and secondary perianal Paget's disease.

Authors+Show Affiliations

Department of Pathology, Western Reserve Care System, A Division of Forum Health, Youngstown, Ohio 44501, USA.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Case Reports
Journal Article
Review

Language

eng

PubMed ID

9870855

Citation

Nowak, M A., et al. "Perianal Paget's Disease: Distinguishing Primary and Secondary Lesions Using Immunohistochemical Studies Including Gross Cystic Disease Fluid Protein-15 and Cytokeratin 20 Expression." Archives of Pathology & Laboratory Medicine, vol. 122, no. 12, 1998, pp. 1077-81.
Nowak MA, Guerriere-Kovach P, Pathan A, et al. Perianal Paget's disease: distinguishing primary and secondary lesions using immunohistochemical studies including gross cystic disease fluid protein-15 and cytokeratin 20 expression. Arch Pathol Lab Med. 1998;122(12):1077-81.
Nowak, M. A., Guerriere-Kovach, P., Pathan, A., Campbell, T. E., & Deppisch, L. M. (1998). Perianal Paget's disease: distinguishing primary and secondary lesions using immunohistochemical studies including gross cystic disease fluid protein-15 and cytokeratin 20 expression. Archives of Pathology & Laboratory Medicine, 122(12), pp. 1077-81.
Nowak MA, et al. Perianal Paget's Disease: Distinguishing Primary and Secondary Lesions Using Immunohistochemical Studies Including Gross Cystic Disease Fluid Protein-15 and Cytokeratin 20 Expression. Arch Pathol Lab Med. 1998;122(12):1077-81. PubMed PMID: 9870855.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Perianal Paget's disease: distinguishing primary and secondary lesions using immunohistochemical studies including gross cystic disease fluid protein-15 and cytokeratin 20 expression. AU - Nowak,M A, AU - Guerriere-Kovach,P, AU - Pathan,A, AU - Campbell,T E, AU - Deppisch,L M, PY - 1998/12/31/pubmed PY - 1998/12/31/medline PY - 1998/12/31/entrez SP - 1077 EP - 81 JF - Archives of pathology & laboratory medicine JO - Arch. Pathol. Lab. Med. VL - 122 IS - 12 N2 - BACKGROUND: Extramammary Paget's disease most commonly occurs on the female external genitalia and rarely occurs in the perianal region and male external genitalia. We present the clinical and pathologic features of 5 cases of perianal Paget's disease and review the literature. METHODS: Clinical and pathologic data were recorded for 5 cases of perianal Paget's disease. Cases were studied retrospectively with special stains, including periodic acid-Schiff, mucicarmine, Alcian blue, carcinoembryonic antigen, S100 protein, pan-keratin, gross cystic disease fluid protein-15 (GCDFP-15), lysozyme, CD15 (Leu-M1), cytokeratin 7 (CK7), and cytokeratin 20 (CK20). RESULTS: Three (60%) of 5 patients had concurrent rectal adenocarcinomas. All cases reacted positively for pankeratin, although the intensity and distribution of staining varied. Both cases not associated with an underlying carcinoma showed strong GCDFP-15 and CK7 expression and an absence of CK20 expression. The 3 cases associated with an underlying malignancy demonstrated CK7 and CK20 expression and an absence of GCDFP-15 expression. All cases were negative for lysozyme and CD15 (Leu-M1). CONCLUSIONS: The 5 cases reported herein demonstrate that perianal Paget's disease is a heterogeneous entity. The high frequency of associated underlying malignancies and resultant poor clinical outcomes highlight the importance of an aggressive search for a second malignancy. In some cases, perianal Paget's disease merely represents a cutaneous manifestation of an underlying rectal adenocarcinoma and demonstrates a CK7+/CK20+/GCDFP-15-/lysozyme-/Leu-M1- immunophenotype and signet ring Paget's cells. Other cases represent primary adenocarcinomas of the skin, which are associated with a CK7+/CK20-/GCDFP-15+/lysozyme /Leu-M1- immunophenotype and an excellent prognosis if adequately resected. Immunohistochemical studies, particularly CK20 and GCDFP-15, are useful adjuncts in distinguishing primary and secondary perianal Paget's disease. SN - 0003-9985 UR - https://www.unboundmedicine.com/medline/citation/9870855/Perianal_Paget's_disease:_distinguishing_primary_and_secondary_lesions_using_immunohistochemical_studies_including_gross_cystic_disease_fluid_protein_15_and_cytokeratin_20_expression_ L2 - https://medlineplus.gov/analcancer.html DB - PRIME DP - Unbound Medicine ER -