Large cell neuroendocrine carcinoma of the ovary: a case report and a brief review of the literature

Abstract

Large cell neuroendocrine carcinoma (LCNC) of the ovary, or ovarian undifferentiated non-small cell carcinoma of neuroendocrine type, is a rare entity that is frequently associated with ovarian surface epithelial tumors. Few cases have been reported in the literature. LCNC is an aggressive tumor with tendency to present at advanced stages and to cause death after a short postoperative duration. We report three cases of LCNC diagnosed histopathologically. Immunohistochemically, the tumor cells were positive for chromogranin A, NSE, CD56, and pancytokeratin. The patients were treated postoperatively with combination chemotherapy. Due to the rarity of LCNC, the general consensus on standard therapy is not established. Although most patients are at stage I, the biological aggressiveness and poor prognosis of the tumors have been reported in previous reports despite extensive surgery and chemotherapy.

Neuroendocrine tumours in rare sites: differences in nomenclature and diagnostics—a rare and ubiquitous histotype

“…A few cases of LCNEC have been described 63. The mere presence of NED in a poorly differentiated ovarian cancer is indicative of poor prognosis.…”

Role of diagnostic laparoscopy in patients with large cell neuroendocrine carcinoma of the ovary with cancerous peritonitis: case report and review of the literature

“…Therefore, the patient received the chemotherapy with etoposide and cisplatin, although postoperative chemotherapy did not improve the clinical outcome in the present case with carcinomatous peritonitis. Previous reports have shown the possible efficacy of paclitaxel and carboplatin, which can be less toxic than cisplatin in cases of even pure LCNEC of the ovary [9,15,20,27,33,35], as well as in cases of NEC of the uterine cervix [43], suggesting that these chemotherapeutic regimens could be considered in patients with poor performance status and prognosis because of unresectable carcinomatous peritonitis. Taken together, in LCNEC, diagnostic laparoscopy followed by primary debulking surgery or neoadjuvant chemotherapy might be useful in cases without carcinomatous peritonitis, whereas it might also be useful for deciding whether patients should receive less invasive chemotherapy or best supportive care in cases with carcinomatous peritonitis with much poorer outcomes.…”

“…The clinicopathological features of 31 cases with FIGO stage I/II (Table 1) and 25 cases with FIGO stage III/IV (Table 2) [7,8,14,17,18,20,[25][26][27][28][29][30][31][32][33][34][35] were reviewed. A Kaplan-Meier survival curve (SPSS Statistics version 24; IBM, Armonk, NY) of all 53 cases excluding 3 cases without clinical outcome data showed total 5year survival of 34.6% and median survival time of 17 months.…”

“…Therefore, pathological examination has to be performed to differentiate non-epithelial ovarian cancer or other diseases including inflammation in these LCNEC cases. In the previous cases with stage III/IV [7,8,14,17,18,20,[25][26][27][28][29][30][31][32][33][34], most patients underwent laparotomy for diagnosis and treatment. However, complete surgery could not be performed in many cases, leading to the deterioration of the patients' general condition and much poorer outcomes.…”

Carcinomas epiteliales del ovario de alto y bajo grado: Estado de Arte

“…El tipo de células no pequeñas suele estar asociado con otro tipo celular de carcinoma epitelial de superficie, en especial el mucinoso. 29,42 Carcinogénesis y biología molecular: se ha asociado con mutaciones en TP53 y hace poco con mutaciones somáticas en SMARCA4. 43…”