Anal canal adenocarcinoma with neuroendocrine features accompanying secondary extramammary Paget disease, successfully treated with modified FOLFOX6: a case report.
BMC Cancer
; 18(1): 1142, 2018 Nov 20.
Article
in En
| MEDLINE
| ID: mdl-30458812
ABSTRACT
BACKGROUND:
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. CASE PRESENTATION 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.CONCLUSIONS:
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.Key words
Full text:
1
Collection:
01-internacional
Database:
MEDLINE
Main subject:
Anal Canal
/
Anus Neoplasms
/
Adenocarcinoma
/
Antineoplastic Combined Chemotherapy Protocols
/
Paget Disease, Extramammary
Type of study:
Etiology_studies
Limits:
Aged
/
Humans
/
Male
Language:
En
Journal:
BMC Cancer
Journal subject:
NEOPLASIAS
Year:
2018
Document type:
Article
Affiliation country:
Japan