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Case Reports
. 2024 Nov 29;18(1):579.
doi: 10.1186/s13256-024-04927-z.

Syringocystadenocarcinoma of the perianal region: a case report

Imane Boujguenna  1 , Fatima Boukis  2 , Sanae Sefiani  3 , Mohammed Essaid Ramraoui  4
Affiliations
Case Reports

Syringocystadenocarcinoma of the perianal region: a case report

Imane Boujguenna et al. J Med Case Rep. .

Abstract

Introduction: Syringocystadenocarcinoma papilliferum is an extremely rare malignant adnexal tumor that typically arises from a papilliferous syringocystadenoma (World Health Organization classification of skin tumors, 2018.). This tumor predominantly occurs in the cephalic region.

Case presentation: We present the case of a 68-year-old Moroccan male with no significant medical history who presented with a slowly progressing skin mass in the perianal region. Despite the chronic nature of the swelling, the patient remained in overall good health. Physical examination revealed a firm 2.3-cm mass in the perianal area, with no other remarkable findings. The patient underwent surgical excision of the mass. Macroscopic examination showed a solid-cystic, rounded mass. Microscopic examination revealed a malignant adnexal tumor with apocrine differentiation and papillary architecture. Immunohistochemistry was positive for p63, cytokeratin 7 (CK7), and smooth muscle actin, with a Ki67 labeling index in 60% of tumor cells, and negative for p16. The diagnosis of syringocystadenocarcinoma papilliferum was confirmed. A further surgical resection was performed, and the patient's postoperative course was unremarkable.

Conclusion: Syringocystadenocarcinoma papilliferum is an extremely rare tumor, with its occurrence in the perianal region being particularly uncommon. This case contributes to the limited literature on this malignancy, highlighting its clinical and pathological features.

Keywords: Literature review; Pathology; Perianal; Syringocystadenocarcinoma papilliferum.

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Conflict of interest statement

Declarations. Ethics approval and consent to participate: Yes. Consent for publication: Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal. Competing interests: The authors declare no competing interests.

Figures

Fig. 1
Fig. 1
Papillae and cribriform clusters (25×)
Fig. 2
Fig. 2
Squamous differentiation (25×)
Fig. 3
Fig. 3
Positive staining with anti-p63 antibody (25×)
Fig. 4
Fig. 4
Positive staining with anti-cytokeratin 7 antibody (25×)
Fig. 5
Fig. 5
Positive staining with anti-Smooth Muscle Actin antibodies (× 25)
Fig. 6
Fig. 6
Positive labeling with anti-Smooth Muscle Actin antibody (25×)
Fig. 7
Fig. 7
Labeling of 60% of neoplastic cells with anti-Ki67 antibodies (25×)
See this image and copyright information in PMC

References

    1. WHO classification of skin tumours. 2018.
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    1. Chen J, et al. Syringocystadenocarcinoma papilliferum in situ, a variant of cutaneous adenocarcinoma in situ: a case report with literature review. Am J Dermatopathol. 2016. 10.1097/DAD.0000000000000687. - PubMed
    1. Iga N, et al. Syringocystadenocarcinoma papilliferum in the perianal area. Case Rep Dermatol. 2015. 10.1159/000381940. - PMC - PubMed
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