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Prostein (p501s) is expressed in primary extramammary Paget disease
Daniel J Shepherd1, Cody Craig1, Aida L Valencia-Guerrero1
1Department of Pathology, Microbiology, and Immunology, Vanderbilt University Medical Center, Nashville, Tennessee, USA.
Histopathology
|January 8, 2026
Summary
Prostein is a promising marker for diagnosing primary extramammary Paget disease (EMPD). It was found to be positive in all primary EMPD cases but negative in secondary EMPD and mammary Paget disease, aiding in differential diagnosis.
Area of Science:
- Dermatopathology
- Oncology
- Uropathology
Background:
- Extramammary Paget disease (EMPD) is an intraepidermal carcinoma affecting the skin of the urogenital, perineal, and perianal regions.
- EMPD can be primary (arising in the skin) or secondary (metastatic from other cancers like urothelial or colorectal).
- Prostein (p501s) is a marker typically associated with prostatic epithelial cells and adenocarcinoma.
Purpose of the Study:
- To investigate the expression of prostein in primary EMPD, secondary EMPD, and mammary Paget disease (MPD).
- To evaluate prostein's utility as an immunohistochemical marker for differentiating primary EMPD.
Main Methods:
- Immunohistochemistry was used to detect prostein expression.
- The study analyzed tissue samples from patients with primary EMPD, secondary EMPD, and MPD.
- Expression of GATA3 and androgen receptor (AR) was also assessed in primary EMPD cases.
Main Results:
- Prostein was positive in all tested cases of primary EMPD (11/11), including invasive and non-invasive components and a nodal metastasis.
- Prostein was negative in all cases of mammary Paget disease (0/11) and secondary EMPD (0/5).
- Primary EMPD cases showed varying degrees of prostein staining and also expressed GATA3 and androgen receptor (AR).
Conclusions:
- Prostein demonstrates high sensitivity and specificity for primary EMPD.
- Prostein is a potentially valuable immunohistochemical marker for diagnosing primary EMPD.
- This finding aids in distinguishing primary EMPD from secondary EMPD and MPD.

