Pure Large Cell Neuroendocrine Carcinoma of Ovary: A Rare Clinical Entity and Review of Literature

Abstract

Large cell neuroendocrine carcinoma (LCNEC) of the ovary is a rare tumor and is now included in the World Health Organization tumor classification. Its prognosis is generally very poor even when the diagnosis is made at an early stage. We report a case of pure large cell neuroendocrine tumour of ovary, appearing 9 months following laparoscopic type I hysterectomy, bilateral pelvic lymph node dissection with ovarian preservation of anatomically normal looking ovaries performed for a cervical biopsy diagnosis of cervical intraepithelial neoplasia grade III with foci of invasion. The rarity lies in the rapid onset (9 months) of a large tumor following conservation of an anatomically normal ovaries. Surgical debulking and five cycles of chemotherapy (Etoposide and Cisplatin) were administered to the woman. She is on followup with no clinical or radiological evidence of disease recurrence for 6 months.

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

“…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.…”

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

“…Clinical symptoms at initial presentation are variable. The most common clinical manifestation was abdominal pain in 7 cases [ 3 , 13 ], followed by abdominal distension (n = 4) [ 14 – 17 ], and an abdominal palpable mass (n = 3) [ 3 , 5 , 16 ], abdominal bloating (n = 3) [ 3 ], abdominal discomfort (n = 1) [ 2 ], postmenopausal vaginal bleeding (n = 1) [ 3 ], and dysarthria due to brain metastasis (n = 1) [ 8 ]. In our report, abdominal distension, abdominal discomfort, and urinary frequency occurred in one case each.…”

“…The urinary frequency may have been due to compression of the bladder by the huge ovarian mass. Most of the LCNCs are partially solid or partially cystic, with size ranging from 9 and 30 cm (mean size, 16.6 cm) [ 2 , 3 , 8 , 14 , 17 ]. In this report, the mean size of the mass was 13 cm, and the mass was also partially solid or cystic.…”

“…[ 2 ] 64 Abdominal discomfort, nausea Cisplatin + Bleomycin + Etoposide 380 9 Shakuntala et al . [ 14 ] 40 Abdominal distension Cisplatin + Etoposide 280 6 Hinde et al [ 15 ] 54 Abdominal distension Carboplatin + Paclitaxcel _ 8 Dundr et al . [ 8 ] 73 Dysarthria, difficulty in verbal expression because of brain metastasis Carboplatin + Paclitaxcel 94 12 Cyberknife on brain lesion Tsuji et al .…”