Published online Feb 26, 2024. doi: 10.12998/wjcc.v12.i6.1111
Peer-review started: November 27, 2023
First decision: December 19, 2023
Revised: December 28, 2023
Accepted: January 22, 2024
Article in press: January 22, 2024
Published online: February 26, 2024
Processing time: 85 Days and 0.3 Hours
Primary ovarian neuroendocrine carcinoma (NEC) are rare and there are no established treatment guidelines for NEC of the ovaries due to limited knowledge about this rare entity.
Primary ovarian NEC are rare, and we want to know more about it.
We retrospectively analyzed the clinicopathological features, treatment and survival of 12 patients with ovarian NEC, and hope to enhance our clinical understanding of NEC of the ovary.
The clinical data of 12 patients with ovarian NEC in our hospital were retrospectively analyzed.
Among more than 2000 patients with ovarian cancer during the same period, we identified 9 cases of small cell ovarian cancer and 3 cases of large cell NEC. Eleven patients underwent surgery, all of whom received adjuvant chemotherapy, and 1 patient with a BRCA2 mutation received PARP inhibitor maintenance therapy after chemotherapy. The median progression-free survival was 13 months, and the median overall survival was 19.5 months. Four patients were alive without tumor recurrence, 8 patients had tumor recurrence, and 3 of them died of tumor recurrence.Abdominal ultrasound showed that the masses were typically solid or cystic-solid with abundant blood supply within the lesion, occupying the pelvic cavity. The borders of the masses were clearly defined with irregular contours. The computed tomography (CT) scan typically revealed a soft tissue mass with varying density in the pelvic cavity. Contrast-enhanced CT demonstrated slight enhancement or uneven enhancement of the solid components. The neoplastic cells were positive for CD56 in all cases, chromogranin A in 5 out of 10 cases, and synaptophysin in 9 out of 11 cases. Additionally, cytokeratin (CK) was positive in 5 out of 6 cases, with CK7 being positive in 4 out of 6 cases, epithelial memberane antigen being positive in 6 out of 7 cases, and PAX-8 being positive in 4 out of 8 cases.
NEC of the ovary is a rare condition that is more common in women of childbearing age and is associated with aggressive behavior and poor clinical outcomes. Surgical resection remains the mainstay of treatment, with some patients benefiting from adjuvant chemoradiation therapy.
In my opinion, the future research direction may be pathogenesis and immunotherapy.