HPV associated adenocarcinoma, signet ring morphology of uterine cervix: A rare presentation

Abstract

Cervical cancer continues to be the leading cancer among women in Nepal with most cases being squamous cell carcinoma. Signet ring cell carcinoma is defined as mucinous adenocarcinoma with over 50% of cells showing signet ring cell morphology. Adenocarcinomas of the uterine cervix have increased in both relative and absolute frequency, they represent only 10 to 25 % of cervical carcinomas; with HPV associated endocervical as the most frequent histological type, followed by mucinous adenocarcinoma. A middle aged lady presented with history of intermenstrual bleeding along with abdominal pain. She was known case of HPV DNA 16 positive (detected 2 years back). Cervical biopsy revealed presence of signet ring cells arranged in diffuse sheets. Individual tumor cells (>50%) were signet ring type of cells with hyperchromatic peripherally pushed nuclei and abundant vacuolated cytoplasm. Glands, tubule formation as well as sheets of tumor cells, some revealing marked atypia and bizarre nuclei were also seen. Villo-glandular projection of endocervical epithelium revealing dysplastic changes were also noted. The diagnosis of HPV associated adenocarcinoma, signet ring type was given since HPV DNA 16 was also positive. IHC report showed P16, PAX8 and CK7 all with diffuse positive in atypical cells. ER was positive in atypical cells meanwhile CDX2, WT-1, CK20 and cyclinD1 were negative, compatible with the diagnosis of adenocarcinoma of cervix, HPV associated. Metastatic work up done for signet ring adenocarcinoma was negative. Here, we present a rare case of HPV 16 associated primary signet ring adenocarcinoma of uterine cervix.