Metastatic carcinoid tumor of the ovary-A rare entity and role of CDX2 to distinguish pure primary carcinoid from metastatic carcinoid to the ovary

Ranjana Giri,Subrat Kumar Sahu,Urmila Senapati

doi:10.18231/j.ijogr.2020.058

Abstract

Metastatic ovarian carcinoid is a very rare entity which is usually of primary gastrointestinal origin. Distinction is difficult between pure primary ovarian carcinoid from metastatic carcinoid tumor of ovary, but distinction is very much crucial because it has clinical and prognostic implications.We are reporting a case of a 55yr old female presented with abdominal pain. Ultrasonography revealed bilateral solid ovarian mass. Macroscopically both the ovaries were enlarged, solid with surface nodularity. Morphology showed tumor cells mostly in insular and acinar configuration, round to oval nucleus, stippled chromatin and granular eosinophilic cytoplasm. IHC for synaptophysin and chromograin were strong positive. CDX2 was done which showed strong nuclear positive in 80% of tumor cells. The tumor was diagnosed as a metastatic carcinoid tumor to the ovary and primary tumor in the small intestine. This case was reported because of its rarity and usefulness of CDX2 to distinguish pure primary carcinoid from metastatic carcinoid to ovary.

Highlights

Carcinoid tumors are rare causes of ovarian malignancy and represent o.1% of all ovarian neoplasms. 1 Primary ovarian carcinoids are 2nd most frequent monodermal teratomas
4 Midgut tumors and their metastatic tumors express high level of CDX2 compared to foregut and hindgut carcinoids. 8–10 CDX2 positivity is seen in tumors arising in small intestine(95%), appendix (92%) and duodenum (80%) but rare in tumors of colorectum (12%) and stomach (0%)
To distinguish between primary or metastatic carcinoid, IHC of CDX2 was done and it showed strong nuclear positive in 80% of tumor cells (Figure 3)

Summary

Introduction

Carcinoid tumors are rare causes of ovarian malignancy and represent o.1% of all ovarian neoplasms. 1 Primary ovarian carcinoids are 2nd most frequent monodermal teratomas. 1 Primary ovarian carcinoids are 2nd most frequent monodermal teratomas. 1,2 Metastatic ovarian carcinoids are very rare. They are usually of primary gastrointestinal origin. Immunohistochemistry of CDX2 has been used to distinguish primary carcinoid of ovary from metastatic. CDX2 is a nuclear transcriptional factor that appears to be expressed in intestinal epithelium. It has a role in regulation of cell proliferation and differentiation and acts as a tumor suppressor.[7] CDX2 expression by carcinoid tumors points to GI origin. 4 Midgut tumors and their metastatic tumors express high level of CDX2 compared to foregut and hindgut carcinoids. CDX2 expression by carcinoid tumors points to GI origin. 4 Midgut tumors and their metastatic tumors express high level of CDX2 compared to foregut and hindgut carcinoids. 8–10 CDX2 positivity is seen in tumors arising in small intestine(95%), appendix (92%) and duodenum (80%) but rare in tumors of colorectum (12%) and stomach (0%)

Case History

Findings

Conclusion