“The glowing white ball”: ovarian carcinoid arising in a mature teratoma - case report and systematic review of treatment and follow-up strategies

Objective Given the rarity of primary ovarian carcinoid tumors, evidence-based recommendations for management and follow-up are lacking. We aimed to address this gap by presenting a case and systematically reviewing current treatment and follow-up strategies. Case report A 53-year-old postmenopausal patient with an adnexal mass underwent bilateral laparoscopic salpingo-oophorectomy, which confirmed a 7-mm ovarian carcinoid tumor arising within a 21-mm mature teratoma. The patient remains disease-free under regular follow-up as recommended by the multidisciplinary team. Systematic review A systematic literature search of PubMed and Embase was conducted (last search: 24 February 2025) in accordance with PRISMA guidelines (PROSPERO registration No. 1048302). Data from eligible studies were extracted and narratively synthesized. Risk of bias was assessed using the JBI tool. Results A total of 84 publications were included in the review. Of these, 52 publications, comprising 119 reported cases, provided sufficient case-level data for inclusion in the descriptive table, while the remaining publications were used in the discussion. Tumors were most frequently detected by transvaginal ultrasound. Surgical resection was the mainstay of treatment, most commonly salpingo-oophorectomy with or without hysterectomy. Adjuvant therapy was rare and primarily used in metastatic disease. Follow-up strategies were inconsistently reported, typically involving clinical assessment, laboratory testing, and imaging at 3–6-month intervals. Recurrence or metastasis was uncommon and occurred predominantly within the first 3.5 years. Conclusion To our knowledge, this is among the first systematic reviews to summarize treatment and follow-up strategies for ovarian carcinoid tumors. Surgical management ranges from fertility-sparing unilateral salpingo-oophorectomy in selected early-stage cases to cytoreductive surgery in advanced disease, with chemotherapy considered for metastatic tumors with a high Ki-67 proliferation index. Peptide receptor radionuclide therapy and somatostatin analogues may be used in receptor-positive disease and for symptom control in carcinoid syndrome. Given the limited avaible evidence, a pragmatic follow-up approach may include assessment at 6 weeks postoperatively, followed by surveillance every 3–6 months during the first year and annually thereafter for up to 4.5 years, using imaging (e.g., transvaginal ultrasound) and biomarkers such as chromogranin A, with cross-sectional imaging reserved for clinically suspicious findings.