Primary ovarian carcinoid tumors: Effective management of a rare and challenging condition

Aim: Primary ovarian carcinoid tumors are rare well- differentiated neuroendocrine tumors, considered to be of germ cell origin, accounting for less than 0.1% of all ovarian carcinomas and 5% of all carcinoid tumors.

Patients and method: Using the Department's database we retrospectively identified 8 women presenting with primary ovarian carcinoid tumors at the Department of Obstetrics and Gynaecology of Aschaffenburg University Hospital in Germany from January, 1991 to December, 2011.

Results: All patients received bilateral salpingo-oophorectomy, total hysterectomy, omentectomy plus pelvic and paraaortic lymph node dissection. No intraoperative complications occurred. Diagnosis was confirmed by histopathological examination. All tumors were unilateral and confined to the ovary. 4 patients had trabecular tumors, 2 of which were associated with mature teratomas, 1 patient had insular tumor and 3 patients had mixed type tumors, 1 of which was associated with mature teratoma. Metastatic lymph nodes were not found in any patient. No patient received adjuvant treatment. Patients were seen in follow-up every 3 months for the first year, every 6 months in the second year and yearly thereafter. Each visit included clinical examination, measurements of chromogranin A, serotonin and tumour markers (CEA, CA-125, αFP) in patients' blood and ultrasonography. No recurrences were noted. Better knowledge of this rare disease may improve preoperative evaluation and enable the surgery-anaesthesia team to take appropriate precautions for optimal surgical management.

Conclusion: When a carcinoid tumour is suspected, it is important to optimize the perioperative management, delaying surgery until further diagnostic testing can be performed.