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Three case reports of ovarian neuroendocrine carcinoma
Abstract
Introduction: Being very rare entities, neuroendocrine tumors of the ovary are mostly benign while few types are malignant. Based on the WHO classification, an acceptable categorization is carcinoid, atypical carcinoid, small-, and large-cell neuroendocrine carcinomas which are of low-, intermediate-, and high-grade, respectively. In this study, the authors present three cases of ovarian neuroendocrine carcinoma. Because such large-cell tumor occurs rarely, they present the similar cases which have been reported in the past. Cases: The first two cases were 71- and 56-year-old women who had been referred with an ovarian mass. Total abdominal hysterectomy with bilateral salpingo-oophorectomy (TAH-BSO) was performed and a Stage 1A ovarian carcinoid tumor was diagnosed in both cases. The two cases received no special type of adjuvant therapy following surgery and their disease-free survival rate was two and 14 years, respectively. The third patient was a 54-year-old woman who was referred with abdominal pain, symptoms of peritonitis, and a pelvic mass. A pelvic mass was found intraoperatively with extensive adhesions to the small and large intestines. She underwent right salpingooophorectomy and ileostomy. In pathologic study, ovarian large-cell neuroendocrine tumor and teratoma with intestinal invasion (Stage 3) were diagnosed. She received chemotherapy, then underwent TAH-LSO (TAH-left salpingo-oophorectomy) and sigmoid resection. No complication was reported in her one-year follow-up. Conclusion: Neuroendocrine tumors of the ovary may present with non-specific symptoms and diagnosis is made just based on pathologic evaluation.
Primary gynecologic neuroendocrine carcinomas (gNECs) are a heterogeneous spectrum of rare and highly aggressive neoplasms, accounting for about 2% of all gynecologic malignancies, which mostly resemble the small cell lung carcinoma (SCLC). Due to the lack of standardized treatment guidelines, their management poses a noteworthy clinical challenge. Currently, cumulative data retrieved from the management of SCLC and from retrospective studies supports a multimodality strategy, based on surgery, chemotherapy, and radiotherapy. Nevertheless, the prognosis remains poor and recurrences are extremely frequent. Hence, there is an urgent need for novel treatment options and promising molecular targets. Recently, there has been an increasing interest on the potential role of immune checkpoint inhibitors, especially in the recurrent setting. However, only scant evidence exists and there is still a long road ahead. A solid collaboration between gynecologists and oncologists worldwide is required to improve the treatment of these puzzling tumors.
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.
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