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Histopathological changes in primary extramammary Paget disease with emphasis on syringomatous structures and syringocystadenocarcinoma papilliferum in situ‑like changes

  • Authors:
    • Yutaro Fukuma
    • Mitsuaki Ishida
    • Yoshinobu Hirose
  • View Affiliations / Copyright

    Affiliations: Department of Pathology, Osaka Medical and Pharmaceutical University, Takatsuki, Osaka 569‑8686, Japan
    Copyright: © Fukuma et al. This is an open access article distributed under the terms of Creative Commons Attribution License.
  • Article Number: 307
    |
    Published online on: May 20, 2026
       https://doi.org/10.3892/ol.2026.15662
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Abstract

Primary extramammary Paget disease (PEMPD) is a rare adenocarcinoma, and is histopathologically characterised by intraepithelial proliferation of carcinoma cells with abundant amphophilic cytoplasm. Minor histopathological findings in PEMPD, including syringomatous structures and syringocystadenocarcinoma papilliferum (SCACP) in situ‑like changes, have rarely been reported. The present study aimed to analyse histopathological changes in PEMPD. Consecutive patients with PEMPD who underwent surgical resection were included. Melanin within carcinoma cell cytoplasm, syringomatous structures and SCACP in situ‑like changes were assessed on haematoxylin and eosin‑stained slides. Overall, 55 patients (16 female and 39 male patients) were included; 23 patients (41.8%) had an invasive carcinoma component and the remainder had only in situ lesions. Melanin pigment within carcinoma cell cytoplasm was observed in 85.5% of patients, while 17.4% of patients with invasive PEMPD exhibited melanin pigment in invasive carcinoma cells. Syringomatous structures, characterised by ductal, tadpole‑like or small cystic structures composed of bland epithelial cells, were observed in 9.1% of patients (31.3% of all female patients), including in areas outside the PEMPD lesions. SCACP in situ‑like changes, characterised by papillary epidermal hyperplasia with replacement of carcinoma cells, were also observed in 9.1% of PEMPD cases. These findings demonstrated that a high frequency of the presence of melanin pigment within carcinoma cell cytoplasm, including in invasive carcinoma cells, could lead to misdiagnosis as malignant melanoma. Additionally, syringomatous structures (especially in female patients) and SCACP in situ‑like changes may lead to the misdiagnosis of syringoma and SCACP in situ, respectively. Therefore, recognition of these histopathological changes in PEMPD is required for accurate diagnosis.
View Figures

Figure 1

Histopathological features of PEMPD.
(A) Proliferation of carcinoma cells containing rich cytoplasm and
large, round to oval nuclei showing nest formation within the
epidermis. Within the nests of carcinoma cells, focal glandular
formation was observed (arrows; haematoxylin and eosin staining;
magnification, ×200). (B) Involvement of carcinoma cells into the
hair follicles and sebaceous ducts (arrows; haematoxylin and eosin
staining; magnification, ×200). (C) Involvement of carcinoma cells
into the eccrine ducts (arrows; haematoxylin and eosin staining;
magnification, ×100). (D) Presence of melanin pigment within the
cytoplasm of carcinoma cells in the in situ lesion
(haematoxylin and eosin staining; magnification, ×400). (E)
Invasive carcinoma cells also contained melanin pigment in their
cytoplasm (haematoxylin and eosin staining; magnification, ×400).
(F) Syringomatous structures in PEMPD. Small ductal or tadpole-like
structures comprised of attenuated bland epithelial cells within
the sclerotic stroma were observed in the upper dermis. A
syringomatous structure was present under the in situ lesion
of extramammary Paget disease. (haematoxylin and eosin staining;
magnification, ×100). (G) Syringocystadenocarcinoma papilliferum
in situ-like change in PEMPD. Epidermal hyperplasia with
papillary proliferation containing carcinoma cells was noted. Dense
plasma cell infiltration in the papillary dermis was observed
(inset) [haematoxylin and eosin staining; magnification, ×100 or
×400 (inset)]. PEMPD, primary extramammary Paget disease.
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Copy and paste a formatted citation
Spandidos Publications style
Fukuma Y, Ishida M and Hirose Y: Histopathological changes in primary extramammary Paget disease with emphasis on syringomatous structures and syringocystadenocarcinoma papilliferum <em>in situ</em>‑like changes. Oncol Lett 32: 307, 2026.
APA
Fukuma, Y., Ishida, M., & Hirose, Y. (2026). Histopathological changes in primary extramammary Paget disease with emphasis on syringomatous structures and syringocystadenocarcinoma papilliferum <em>in situ</em>‑like changes. Oncology Letters, 32, 307. https://doi.org/10.3892/ol.2026.15662
MLA
Fukuma, Y., Ishida, M., Hirose, Y."Histopathological changes in primary extramammary Paget disease with emphasis on syringomatous structures and syringocystadenocarcinoma papilliferum <em>in situ</em>‑like changes". Oncology Letters 32.1 (2026): 307.
Chicago
Fukuma, Y., Ishida, M., Hirose, Y."Histopathological changes in primary extramammary Paget disease with emphasis on syringomatous structures and syringocystadenocarcinoma papilliferum <em>in situ</em>‑like changes". Oncology Letters 32, no. 1 (2026): 307. https://doi.org/10.3892/ol.2026.15662
Copy and paste a formatted citation
x
Spandidos Publications style
Fukuma Y, Ishida M and Hirose Y: Histopathological changes in primary extramammary Paget disease with emphasis on syringomatous structures and syringocystadenocarcinoma papilliferum <em>in situ</em>‑like changes. Oncol Lett 32: 307, 2026.
APA
Fukuma, Y., Ishida, M., & Hirose, Y. (2026). Histopathological changes in primary extramammary Paget disease with emphasis on syringomatous structures and syringocystadenocarcinoma papilliferum <em>in situ</em>‑like changes. Oncology Letters, 32, 307. https://doi.org/10.3892/ol.2026.15662
MLA
Fukuma, Y., Ishida, M., Hirose, Y."Histopathological changes in primary extramammary Paget disease with emphasis on syringomatous structures and syringocystadenocarcinoma papilliferum <em>in situ</em>‑like changes". Oncology Letters 32.1 (2026): 307.
Chicago
Fukuma, Y., Ishida, M., Hirose, Y."Histopathological changes in primary extramammary Paget disease with emphasis on syringomatous structures and syringocystadenocarcinoma papilliferum <em>in situ</em>‑like changes". Oncology Letters 32, no. 1 (2026): 307. https://doi.org/10.3892/ol.2026.15662
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