Carcinoid tumors are distinct neuroendocrine neoplasms with characteristic histological, clinical and biological features.
Material and methods: An extremely rare case of a primary carcinoid tumor arising in a mature cystic teratoma is reported. A 23-year-old woman was admitted with pain in the lower abdomen and for further examination of a tumor in the left anexa. Transabdominal ultrasonography demonstrated a cystic mass measuring 110/70 mm, with irregularly thickened walls in the left anexa. The left ovary could be visualized separate from this lesion.
Conservative surgery was performed (due to the age of the patient)- left saplingo-oopherectomy.
Results: Macroscopically, the ovarian tumor was partly cystic and partly solid, and the solid part measured 40 mm in diameter. On cut section, cyst was multiloculated, and was filled with hair and sebaceous material. Microscopic examination of the cystic area showed a cyst linied by stratified squamous epithelium with sebaceous glands, sweat ducts and hair shafts in the wall. The solid part showed thyroid follicular structure and a tumor arranged in insular and trabecular pattern. On immunohistochemistry, the tumor showed strong positivity for chromogranin A, neurospecific enolase and synaptophysin.
The pathological diagnosis was mature cystic teratoma with primary carcinoid.
Conclusions: Insular carcinoids are often associated with carcinoid syndrome, while in the trabecular variant, endocrine manifestations are rarely present. The patient did not have a carcinoid syndrome. She has been followed for 31 months with no recurrence.
Primary ovarian carcinoids are rare, accounting for 0.3% of all carcinoid tumors and developed in pure form or in association with other tumors.
Prague, Czech Republic
24 - 28 Apr 2010
European Society of Endocrinology