International Journal of

Introduction
 
Neuroendocrine carcinomas (NECS) are originated from endocrine cells of the diffuse neuroendocrine system, which are mainly from gastrointestinal tract, lungs and pancreas (1). Neuroendocrine neoplasms of the testis are unusual tumors and represent more than 1% of all testicular tumors (2). They are rarely seen in the female genital system, and most commonly in the ovaries and cervix. NEC occurs very exceptional in another part of the genital trace (e.g, the endometrium or vagina) (3). An analysis of the SEER database shows that the incidence of neuroendocrine tumor is increasing (1).
Tumor differentiation and tumor grade often are correlated with mitotic count and Ki-67 proliferation index (4). In most cases, well-differentiated, low grade tumors have a mitotic count of less than 2/10 high-power field (HPF) and Ki67 index of less than 3%. Well differentiated intermediate-grade tumors usually have a mitotic count of 2 to 20/10 HPF and/or a Ki-67 index of 3-20%. In high-grade tumors, the mitotic count usually exceeds 20/10 HPF and/or the Ki-67 exceeds 20% (4). In addition the margin status (Negative or positive), the presence of vascular or perineural invasion should be included in the pathology report, which has been suggested that these factors may also have prognostic significance (5).


Figure 1. Neuroendocrine neoplasm: small nests in rich vascular background. Cells have finely granular amphophilic cytoplasm.


Discussion
 
Lee et al presented a 40-year-old woman with a 30 cm pelvic mass. After the operation, she was diagnosed of stageIa NEC of left ovary. The patient received six cycles of paclitaxel-carboplatin chemotherapy postoperative similar to this case report primary neuroendocrine carcinoma presented with an adnexal mass (8). Berdjis and Mostofiin presented a series of 10 cases of carcinoid tumors of the testis: 2 of the patients died of 4-6 years (9).
Zavala-Pompa et al described 3 cases described can be categorized as low-grade (well-differentiated) tumors, while 1 case likely corresponds to an intermediate-grade (moderately differentiated) tumors like the case which was mentioned in this report (10). Crochet et al reported a primary neuroendocrine carcinoma of fallopian tube which presented with an adenexal mass. They performed laparotomy and staging and adjuvant chemotherapy 6 cycles of carbopatin/ etoposide. She was alive 9 months after the diagnosis with no evidence of relapse (11).
Like in this case report. primary neuroendocrine carcinoma presented with an adnexal mass, but pulmonary metastasis has been happened during chemotherapy and the origin of tumor was ovotestis. Gonadal Malignancies are the most reported neoplasm affected the ovotestis. Here in our case ovotestis is presented with neuroendocrine carcinoma.
 
Conclusion
 
Primary carcinoid tumors of the ovary account for less than 5% of all carcinoid tumors and for less than 0.12% of all ovarian neoplasms. Neuroendocrine tumor of the ovary represents 3% of all neuroendocrine tumors. The prevalence of ovotestis is 1/20000 births. For those patients in whom the diagnosis is known. Hysterectomy for early stage disease and adjuvant chemotherapy with etoposide/ cisplatin even without evidence of nodal metastasis should be considered as part of a multi-modality therapeutic strategy (3).
Neuroendocrine carcinoma of the ovary is a rare and aggressive tumor commonly associated with other surface epithelial and germ cell neoplasms. The prevalence of ovotestis is rare but we should consider it in patients with adnexal mass and in patients with simptoms like amenorrhea we should consider gental system anomalies to diagnosis of rare ovarian cancers in early stage.

Acknowledgements
 
The authors thank to Clinical Research Development Unit, Imam Hossein Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran.
 
Conflict of interests
 
None of the authors has any potential conflict of interest.