Ependymoma and carcinoid tumor associated with ovarian mature cystic teratoma in a patient with multiple endocrine neoplasia I.

Reed Spaulding,Daniel Stewart Metzinger,Mana Moghadamfalahi,Houda Alatassi

doi:10.1155/2014/712657

Abstract

Ovarian teratomas rarely undergo new neoplastic transformation and account for a small percentage of malignant ovarian germ cell neoplasms. Here we report a case of a 51-year-old woman with multiple endocrine neoplasia type I (MEN I) who was found to have an ependymoma and neuroendocrine tumor (trabecular carcinoid) associated with mature cystic teratoma of her left ovary. The ependymoma component displayed cells with round nuclei and occasional small nucleoli which were focally arranged in perivascular pseudorosettes and true rosettes. Rare mitoses were identified. No necrosis was present. Immunohistochemical staining was positive for S-100 and GFAP. The Ki67 proliferation index was very low (2-3%). In contrast, the endocrine tumor component was composed of small uniform cells with eosinophilic cytoplasm, round nuclei, and speckled chromatin. Immunohistochemical staining was positive for synaptophysin and focally positive for chromogranin. This rare case illustrates that MEN I may have an influence on the pathogenesis of ovarian teratomas as they undergo malignant transformation.

Highlights

Mature cystic teratomas (MCTs) are the most common germ cell tumors of the ovary [1]
Based on the type of tumors that developed from the MCT seen in our patient, it raises the possibility that multiple endocrine neoplasia type I (MEN I) may alter the pathogenesis of MCTs as they undergo new neoplastic transformation
In addition to the classic presentation of parathyroid adenomas, enteropancreatic endocrine tumors, and pituitary tumors that are most often associated with MEN I, carcinoid tumors are rarely encountered

Summary

Introduction

Mature cystic teratomas (MCTs) are the most common germ cell tumors of the ovary [1]. We present a case of a patient with a long-standing history of MEN I, who developed a mature cystic ovarian teratoma with a mature epidermal and neural component. Her tumor underwent new neoplastic transformation to include both ependymoma and neuroendocrine tumor components. This rare association has not been reported before and it may raise the possibility that MEN I might have influenced the pathogenesis of neoplastic transformation of the ovarian teratoma in this novel case

Case Presentation

Findings

Discussion