title: Neuroendocrine tumors in the ovary: histogenesis, pathologic differentiation, and clinical presentation.
abstract: OBJECTIVE
  Primary neuroendocrine tumors in the ovary are rare. These tumors arise from the neuroendocrine cell system of ovarian stroma and surface epithelium, and may also arise from teratoma. We present four primary ovarian neuroendocrine tumors and compare clinicopathologic findings based on tumor histogenesis and site of origin.
  
  
  METHODS
  Four primary ovarian neuroendocrine tumors were identified from our 10-year departmental archives. H&E slides and immunostains were reviewed and the diagnoses were confirmed. Clinical history, imaging studies, and follow-up data were obtained from medical records.
  
  
  RESULTS
  Patients' ages ranged from 26 to 63. All patients presented with abdominal discomfort and unilateral or bilateral ovarian masses. MRI and CT scans from cases 1 and 2 revealed a solid ovarian mass with no extra-ovarian extension. In case 1, the patient also had a cystic mass in the opposite ovary and an elevated urine 5-HIAA. Microscopically, case 1 revealed a well-differentiated carcinoid tumor with no surface epithelial involvement, and a mature teratoma in the contralateral ovary. Case 2 revealed a stromal carcinoid within the ovarian parenchyma. Imaging studies from cases 3 and 4 showed large complex masses with peritoneal implants and ascites. In both cases 3 and 4, tumor grossly involved both ovarian parenchyma and surface epithelium with multiple pelvic implants. In addition, liver metastases were present in case 4. Microscopically, these tumors were poorly differentiated carcinoma with neuroendocrine differentiation. Histologic sections revealed extensive necrosis, and both cases showed positivity for neuroendocrine markers.
  
  
  CONCLUSIONS
  Primary neuroendocrine tumors in the ovary are rare and consist of a group of heterogeneous malignancies that express similar immunohistochemical markers. Primary neuroendocrine tumors that are limited to the ovarian parenchyma often arise from ovarian stroma and teratoma, and are carcinoid tumors with a good prognosis. Neuroendocrine tumors that arise from surface epithelium or dedifferentiate from de novo carcinoma often involve both ovarian stroma and surface epithelium and clinically present as aggressive malignancies with poor prognoses.
authors: Department of Pathology, Louisiana State University Health Sciences Center, 1501 King's Highway, Shreveport, LA, 71103, USA.
authors: Moiz
authors: M
authors: Vora
authors: Department of Obstetrics and Gynecology, Louisiana State University Health Sciences Center, 1501 King's Highway, Shreveport, LA, 71103, USA.
authors: Robin A
authors: RA
authors: Lacour
authors: Department of Obstetrics and Gynecology, Louisiana State University Health Sciences Center, 1501 King's Highway, Shreveport, LA, 71103, USA.
authors: Destin R
authors: DR
authors: Black
authors: Department of Pathology, Louisiana State University Health Sciences Center, 1501 King's Highway, Shreveport, LA, 71103, USA.|Department of Obstetrics and Gynecology, Louisiana State University Health Sciences Center, 1501 King's Highway, Shreveport, LA, 71103, USA.
authors: Elba A
authors: EA
authors: Turbat-Herrera
authors: Department of Pathology, Louisiana State University Health Sciences Center, 1501 King's Highway, Shreveport, LA, 71103, USA. xgu@lsuhsc.edu.
authors: Xin
authors: X
authors: Gu
mesh_terms: D000328:Adult; D002276:Carcinoid Tumor / Q000473:pathology* / Q000601:surgery; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D007044:Hysterectomy; D008279:Magnetic Resonance Imaging; D008875:Middle Aged; D018358:Neuroendocrine Tumors / Q000473:pathology* / Q000601:surgery; D010051:Ovarian Neoplasms / Q000473:pathology* / Q000601:surgery; D010052:Ovariectomy; D012489:Salpingostomy; D013724:Teratoma / Q000473:pathology; D016896:Treatment Outcome
keywords: Carcinoid; Carcinoma; Neuroendocrine tumor; Ovary