Case 202 - Discussion

Uploaded: 2008-03-28, Updated: 2008-09-10



HE, 20× HE, 20×

HE, 20× HE, 20×
HE, 40×

HE, 40×

Mucicarmine CK7
CK20 CK20
P16 P16
  • Previous Biopsy and Report I
    • A). Cervix biopsy: transformation zone mucosa with focal HPV-like changes;
    • B). ECC: focal atypical stromal nodule, benign endocervical glands and stroma;
    • C). EMC: early  proliferative endometrium.
    • AFIP Consultation Report for Part B: signet ring cells of uncertain origin, insufficient tissue for further evaluation, strongly recommend follow-up and rebiopsy.
  • Previous Biopsy and Report II
    • ECC. Original diagnosis: invasive squamous cell carcinoma.
    • AFIP Consultation Report: reactive squamous epithelium and benign endocervical glandular epithelium, no definite dysplasia or carcinoma.
  • Current Case: Signet Ring Cell Adenocarcinoma involving the uterine cervix, endocervix and endometrium. Uncertain primary origin.
  • Follow-up: a systemic workup was followed after issue this report, and an appendiceal tumor was found (case 212).

Appendiceal goblet cell carcinoid tumor involving uterine cervix

Pure or predominantly signet-ring cell carcinomas of the uterine cervix are rare, and only rarely, cervical involvement is the first manifestation of the disease. Primary signet-ring carcinoma of the cervix is extremely unusual, and it nearly always represents a metastasis, usually from stomach in most instances and less frequently from colon, ovary, or even breast. Therefore, it is always necessary to rule out a metastatic neoplasm. Focal signet-ring cell differentiation can be occasionally seen within primary cervical carcinomas and adenosquamous carcinomas


Histologically, the tumor in the cervix demonstrates diffuse submucosal infiltration by signet ring cells with severe desmoplastic reaction. Cells are isolated or organized in small nests. The surface mucosa is well preserved, and there is no transition between the mucosa and the neoplasm. The goblet cell carcinoid tumor show intracellular mucin with Periodic acid Schiff-diastase and mucicarmine stains. The neoplastic cells are diffusely positive for cytokeratin AE1-AE3, carcinoembryonic antigen, chromogranin A, NSE, and synaptophysin. The cervical tumors metastasized from the gastrointestinal tract typically reveal strong and diffuse positivity to cytokeratin 20 and CDX2 with focal or negative staining for cytokeratin 7. However, the primary cervical neoplasms are positive for cytokeratin 7, but negative for cytokeratin 20 or CDX2.


Appendiceal goblet cell carcinoid tumors are rare, accounting for 3.1- 13.8% of all appendiceal malignancies. The tumor are typically composed of groups of goblet cells containing predominantly vacuolated cytoplasm with basal round to crescentic nuclei containing a single small nucleolus. Less conspicuous are cells with pink granular cytoplasm and round nuclei. The tumor may show tubular structure, and the ratio of tubular structure and goblet cells can vary significantly. Focal tubular structures with occasional goblet cells are commonly seen in the primary appendiceal site, and foci of predominantly goblet cells can be present, especially in the extra-appendiceal infiltration sites.


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