Appendiceal signet ring cell carcinoid-like adenocarcinoma (crypt cell carcinoma) metastatic to the ovaries

Definition

Appendiceal tumour with ovarian metastases, in which the tumour in both organs includes a signet ring cell component, need to be differentiated from more indolent pure goblet cell carcinoids of the appendix.

Macroscopic appearances

The ovarian tumour is usually bilateral.

Histopathology

The ovarian tumours each show multiple patterns range for goblet cell carcinoid to signet ring cell carcinoma.

Immunohistochemistry

Expression of neuroendocrine markers is seen in only a minority of cells in each tumour

	Appendiceal primary	Ovarian metastasis
Chromogranin	6/16: mean 12% of tumour cells positive1	7/19: mean 6% of tumour cells positive1
Synaptophysin	6/15: mean 17% of tumour cells positive1	4/18: mean 8% of tumour cells positive1

Differential diagnosis

Primary ovarian goblet cell carcinoid is usually unilateral and associated with a teratoma.

Prognosis

This condition is frequently fatal, with a median survival of 16 months.

References

1 Hristov AC, Young RH, Vang R, et al. Ovarian metastases of appendiceal tumors with goblet cell carcinoidlike and signet ring cell patterns: a report of 30 cases. Am J Surg Pathol 2007; 31:1502-11

This page last revised 12.1.2008