Ultrasound features of appendiceal adenoneuroendocrine carcinoma metastatic to ovaries.

Abstract

A 15-year-old patient was referred to our center after ascites, increased ovarian volume and pleural and pericardial effusion were diagnosed on computed tomography (CT). She was being treated for ulcerative rectocolitis with immunomodulators (infliximab). A CT scan was performed due to diagnoses of subclavian venous thrombosis, supraclavicular lymphadenomegaly and pleural effusion. Transrectal ultrasound examination showed bilateral solid ovarian masses of 84 × 46 mm (right) and 81 × 55 mm (left) in size, with hyperechoic echotexture, multiple cystic areas and irregular margins (Figure 1, Videoclip S1). On color Doppler examination, poor vascularization was detected in both lesions (Figure 1). Both ascites and pelvic carcinomatosis were detected. Laparoscopic surgery including left salpingo-oophorectomy and multiple pelvic peritoneum biopsy was performed. Final histology showed ovarian metastasis from an appendiceal adenoneuroendocrine carcinoma with peritoneal spread. Postoperatively, the patient was treated with adjuvant chemotherapy consisting of fluorouracil, oxaliplatin and irinotecan. The patient's clinical condition deteriorated rapidly at the end of the fourth course of chemotherapy and she died 7 months after the initial diagnosis. Malignant epithelial tumors of the appendix account for 1% of all gastrointestinal neoplasms1. Mixed appendiceal adenoneuroendocrine carcinomas are a very rare entity including all carcinomas of the appendix that arise from a pre-existing goblet cell carcinoid. They are composed of goblet cell nests and carcinoma cells, with each component representing at least 30% of the tumor1, 2. The ovaries and peritoneum are the most common metastatic sites from mixed adenoneuroendocrine carcinomas. Up to 83% of patients with Stage-IV disease present with ovarian masses3. Ulcerative rectocolitis diagnosed in childhood, as well as family history of colorectal cancer in a first-degree relative and extensive colonic involvement, represent risk factors for the development of colorectal or extracolonic cancer4. Ultrasound features of ovarian metastasis from the appendix have been described previously (including both mucinous and carcinoid)5. However, no data are available on the ultrasound features of mixed adenoneuroendocrine carcinomas metastatic to the ovaries. The vast majority of metastases deriving from the colon, rectum, appendix or biliary tract are multilocular or multilocular-solid masses5. The findings in the present case demonstrate that ovarian metastases from an adenoneuroendocrine tumor appear as large, bilateral and solid masses with inhomogeneous echogenicity and poor vascularization. This case is an example of metastasis to the ovary from a mixed appendiceal adenoneuroendocrine carcinoma presenting ultrasound features different from those observed in other metastases to the ovaries, such as metastases from the colon or biliary tract, as well as those from other histotypes of appendiceal carcinoma. Videoclip S1 Grayscale ultrasound imaging showing bilateral ovarian masses in patient with appendiceal adenoneuroendocrine carcinoma metastatic to ovaries. Please note: The publisher is not responsible for the content or functionality of any supporting information supplied by the authors. Any queries (other than missing content) should be directed to the corresponding author for the article.