Abstract
BackgroundAnal canal cancer occasionally accompanies extramammary Paget disease. Although most of them are squamous cell carcinoma, anal canal adenocarcinoma with neuroendocrine features accompanying secondary extramammary Paget disease has never been reported.Case presentationHere, we report a 76-year-old man presented with pruritus in the perianal area. Investigation revealed a fist-sized perianal erythema, diffuse liver tumors, and right inguinal lymph node swelling. Pathological examination of biopsies from the erythema suggested secondary extramammary Paget disease with positive cytokeratin-7 and -20 expressions and negative GCDFP-15 expression. The anal canal tumor was confirmed by digital examination and endoscopy. Biopsies from the anal canal tumor, swollen lymph node, and Paget lesion all showed poorly differentiated adenocarcinoma with neuroendocrine features expressing synaptophysin and chromogranin A. Serum CEA and NSE levels were high, 809.4 ng/ml and 85.8 ng/ml, respectively. After chemotherapy with modified FOLFOX6 for 2 months, the Paget lesion disappeared, and the primary anal canal tumor and liver metastases shrunk remarkably. Serum CEA and NSE levels decreased promptly to within normal ranges.ConclusionsThis is a clinically significant case, as it reveals novel pathological features about anal canal cancer with secondary Paget disease and successfully treated with modified FOLFOX6. Careful pathological investigation and appropriate treatment choice are needed for this rare cancer.
Highlights
Anal canal cancer occasionally accompanies extramammary Paget disease
We present a rare case of adenocarcinoma with neuroendocrine features of the anal canal accompanying secondary extramammary Paget disease (EPD) with diffuse liver involvements, successfully treated with an oxaliplatin-containing regimen
In secondary EPD, the tumor cells are positive for CK7 and CK20, but negative for GCDFP-15, whereas primary EPD is commonly positive for GCDFP-15 and CK-7, but negative for CK-20 [17,18,19,20,21]
Summary
We reported here the rare case of anal canal adenocarcinoma with neuroendocrine features accompanying secondary EPD, and found mFOLFOX6 to be a very effective treatment.
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