Abstract
BackgroundAnal gland carcinoma with perianal Paget’s disease is rare, and anal gland carcinoma in situ is extremely rare. No cases of anal gland carcinoma in situ with pagetoid spread have been previously reported.Case presentationPhysical examination in a 75-year-old woman revealed an erythematous, inflamed, perianal skin lesion. Neither colposcopy, cystoscopy, colonoscopy, computed tomography, nor magnetic resonance imaging showed evidence of malignant genitourinary or gastrointestinal lesions. Histopathological examination of a biopsy specimen showed many Paget’s cells in the perianal skin lesion and no malignant cells in the rectal or vaginal mucosa. Therefore, primary extramammary Paget’s disease of the anogenital region was suspected, and we performed anus-preserving wide local excision. However, immunohistochemistry revealed a diagnosis of secondary extramammary Paget’s disease due to adenocarcinoma arising from the anal gland. We therefore proceeded with a radical operation. Histopathological examination showed no residual cancer cells. The final diagnosis was anal gland adenocarcinoma in situ with pagetoid spread in the perianal skin.ConclusionsThis is the first case report of anal gland adenocarcinoma in situ with pagetoid spread. We recommend immunohistochemical analysis of biopsy and locally resected specimens to obtain an accurate diagnosis and determine the appropriate treatment when there is no visible tumor.
Highlights
Anal gland carcinoma with perianal Paget’s disease is rare, and anal gland carcinoma in situ is extremely rare
Immunohistochemical analysis revealed that the Paget’s cells were positive for cytokeratin (CK) 7, CK20, and caudal-related homeobox gene nuclear transcription factor (CDX) 2 and negative for gross cystic disease fluid protein (GCDFP) 15 (Fig. 4a–e). These data suggested that her perianal skin lesion was secondary Extramammary Paget’s disease (EMPD) arising from the rectum, there was no lesion found in the rectum
The final diagnosis was anal gland adenocarcinoma in situ with pagetoid spread in the perianal skin
Summary
This case report indicates that the symptomatic pagetoid changes in the perianal skin without a visible tumor in the rectum should raise the suspicion of pagetoid spread from anal gland adenocarcinoma in situ. MT and CO performed the immunohistochemical analysis. Anal duct carcinoma: a report of 5 cases. Neoplasms of anal canal and perianal skin. Consent for publication Written informed consent was obtained from the patient for the publication of this case report and any accompanying images. Author details 1Department of Surgery, Nara Medical University, 840 Shijo-cho, Kashihara, Nara 634-8522, Japan. Author details 1Department of Surgery, Nara Medical University, 840 Shijo-cho, Kashihara, Nara 634-8522, Japan. 2Department of Endoscopy, Nara Medical University Hospital, 840 Shijo-cho, Kashihara, Nara 634-8522, Japan. 3Department of Diagnostic Pathology, Nara Medical University, 840 Shijo-cho, Kashihara, Nara 634-8522, Japan. 4Department of Plastic Surgery, Nara Medical University, 840 Shijo-cho, Kashihara, Nara 634-8522, Japan
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