Pathological features, clinical presentations and prognostic factors of ovarian large cell neuroendocrine carcinoma: a case report and review of published literature

Abstract

Background There is no consensus on the optimal chemotherapy regimen and the prognostic factors for ovarian large cell neuroendocrine carcinoma (LCNEC), a rare type of tumor. The objective of the present study is to present the case of a recent encounter of pure ovarian LCNEC and perform a brief review to summarize the clinicopathological features and prognostic factors of 57 cases of LCNEC patients that have been previously reported. Method: case presentation Eligible studies were searched for online and 57 cases with clear follow-up data were found to have been reported. We present the 58th case, which is of a 70-year-old woman with stage IIIc primary pure LCNEC of the ovary. The initial symptom of this patient was abdominal distension (more than 2 months). A recent ultrasound test showed a solid-cystic mass occupying the pelvic and abdominal cavity. She received two courses of cisplatin-etoposide chemotherapy as an adjuvant therapy. No signs of nonclinical or radiological evidence of disease recurrence was found at follow-up examinations during the first 3 months after operation. A retrospective review of these 58 cases was conducted and survival curves were estimated. Using the Kaplan-Meier method. Conclusion The patients included were aged between 18 and 80 years. A Kaplan-Meier survival curve revealed that the median overall survival was 10.000 months, while 26 (44.83%) patients died within 12 months. We compared the overall mean survival time of all patients with that of stage I patients (42.418 vs 42.047 months), which suggests that ovarian LCNEC has a very poor prognosis even at stage I. Mean survival was longer for patients who had undergone postoperative chemotherapy than for those without postoperative chemotherapy (48.082 vs 9.778 months). A small series, such as this, does not provide adequate data to establish a firm correlation between the postoperative chemotherapy and prognosis ( p = 0.176). In our review of 58 cases with ovarian LCNEC, prognosis was unfavorable in most cases. Given the rarity of LCNEC, it is highly recommended that a global medical database of ovarian LCNEC and a standard system of diagnosis and treatment is established.

Clinicopathologic characteristics and survival outcomes in neuroendocrine carcinoma of the ovary

“…The adjuvant chemotherapy regimens for patients with neuroendocrine carcinoma of the ovary are not reported in the SEER database. Yang et al reviewed sporadic case reports and found that 86% of 58 cases received adjuvant chemotherapy, and platinum-based regimens accounted for the majority of the cases, but this small group analysis did not confirm survival benefits from chemotherapy 21. The paucity of data still suggests that etoposide/platinum chemotherapy may be of benefit to patients.…”

Systematic retrospective analysis of 10 cases of neuroendocrine tumors of the ovary: diagnosis, treatment and follow-up

“…50% recurred within 6 months of chemotherapy, which suggested that these tumors might respond poorly to chemotherapy. Yang et al reviewed sporadic case reports, and their study did not con rm survival bene ts from chemotherapy [24]. Two patients who received EP have been followed up for 24 and 27 months, without disease.…”

Ovarian Neuroendocrine Tumors: a Single-institution Experience

“…50% recurred in 6 months of chemotherapy, which suggested that these tumors might respond poorly to the chemotherapy. Yang et al reviewed sporadic case reports, and their study failed to con rm survival bene ts from chemotherapy [24]. Two cases who received EP have been followed up for 24 and 27 months, without any diseases.…”