levoleucovorin and Paget-Disease--Extramammary

Anal 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.. Here, 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.. This 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.

Topics: Adenocarcinoma; Aged; Anal Canal; Antineoplastic Combined Chemotherapy Protocols; Anus Neoplasms; Carcinoma, Neuroendocrine; Fluorouracil; Humans; Leucovorin; Male; Organoplatinum Compounds; Paget Disease, Extramammary; Treatment Outcome

Extramammary Paget carcinoma (EPC) is considered to originate from the eccrine and apocrine glands of the adnexal skin of the axilla, vulva or penis, scrotum, or perineum. If immunohistochemical techniques with the use of cytoskeletal markers are applied in addition to histologic examination, EPC lesions are found to be multifocal adenocarcinoma of the adnexal sweat glands. So far, therapeutic approaches have included primary surgery, followed optionally by radiation therapy. It is possible to achieve a cure only when manifestations are detected early and confined regionally. The role of palliative chemotherapy has not yet been assessed in this disease, particularly because of the lack of compliance and follow-up in patients with EPC.

Topics: Aged; Antineoplastic Combined Chemotherapy Protocols; Carboplatin; Fluorouracil; Humans; Immunohistochemistry; Leucovorin; Male; Paget Disease, Extramammary