BACKGROUND: Neuroendocrine tumours are a heterogeneous group of separate clinico-pathological entities which have a common characteristic, i.e., expression of potential endocrine differentiation. In the ovary, the term "neuroendocrine" relates mainly to widely known carcinoids, but it may also be applied to rare neuroendocrine carcinomas as non-small cell type and small cell carcinomas of pulmonary type. In the literature only 11 cases of primary ovarian non-small cell neurendocrine carcinomas have been described and ten of these were associated with a surface epithelial ovarian tumour. Small cell neuroendocrine carcinoma of the ovary is a rare malignant tumour of the ovary. Advanced small cell carcinoma of the ovary is a very aggressive tumour with an overall poor prognosis and unfavourable outcome. CASE REPORT: The case reported is unique in the literature because the authors describe a rare case of endometrial metastasis of a primary ovarian non-small cell neurendocrine carcinoma without any surface epithelial ovarian tumour association. The tumour invaded up to less than half of the myometrium. The first symptoms were related to endometrial metastasis as metrorrhagia and pelvic pain while the asymptomatic presence of primary ovarian carcinoma was not acknowledged with physical examination, routine biochemistry, tumour markers, blood count and traditional transvaginal greyscale ultrasound. CONCLUSION: Magnetic resonance and three-dimensional (3D) ultrasonography with power Doppler are a great help in the diagnosis of ovarian localisation but only immunohistochemistry on histological material can provide a correct diagnosis. Immunohistochemistry expression of Ki67 is a useful marker of malignancy. Due to the rarity of this neoplasm, a general consensus for optimal treatment has yet to emerge. The reported biological aggressiveness of these tumours prompts combined treatment with radical surgery and adjuvant polychemotherapy.
BACKGROUND:Neuroendocrine tumours are a heterogeneous group of separate clinico-pathological entities which have a common characteristic, i.e., expression of potential endocrine differentiation. In the ovary, the term "neuroendocrine" relates mainly to widely known carcinoids, but it may also be applied to rare neuroendocrine carcinomas as non-small cell type and small cell carcinomas of pulmonary type. In the literature only 11 cases of primary ovarian non-small cell neurendocrine carcinomas have been described and ten of these were associated with a surface epithelial ovarian tumour. Small cell neuroendocrine carcinoma of the ovary is a rare malignant tumour of the ovary. Advanced small cell carcinoma of the ovary is a very aggressive tumour with an overall poor prognosis and unfavourable outcome. CASE REPORT: The case reported is unique in the literature because the authors describe a rare case of endometrial metastasis of a primary ovarian non-small cell neurendocrine carcinoma without any surface epithelial ovarian tumour association. The tumour invaded up to less than half of the myometrium. The first symptoms were related to endometrial metastasis as metrorrhagia and pelvic pain while the asymptomatic presence of primary ovarian carcinoma was not acknowledged with physical examination, routine biochemistry, tumour markers, blood count and traditional transvaginal greyscale ultrasound. CONCLUSION: Magnetic resonance and three-dimensional (3D) ultrasonography with power Doppler are a great help in the diagnosis of ovarian localisation but only immunohistochemistry on histological material can provide a correct diagnosis. Immunohistochemistry expression of Ki67 is a useful marker of malignancy. Due to the rarity of this neoplasm, a general consensus for optimal treatment has yet to emerge. The reported biological aggressiveness of these tumours prompts combined treatment with radical surgery and adjuvant polychemotherapy.