Ovary

• Primary ovarian carcinoid tumors are well differentiated neuroendocrine tumors resembling those arising in the gastrointestinal tract
• In some cases, teratomatous components are identified (component of mature cystic teratoma)
• 4 types: insular, trabecular, strumal and mucinous

• Asymptomatic, incidental finding
• Abdominal mass
• Ascites
• Carcinoid syndrome
  • ~33% of patients are > 50 years old
  • Presence of carcinoid syndrome correlates with size of the tumor; typically seen in tumors > 7 cm, rarely seen in tumors < 4 cm
  • Most common in insular type
  • Does not require the presence of liver metastasis since venous drainage bypasses portal venous circulation (Tex Heart Inst J 2019;46:21)
  • Carcinoid heart disease may be the initial manifestation
    • Occurs in < 10% of cases of primary ovarian carcinoid
    • Generally involves right chambers and valves
• Endocrine effects thought to occur due to stromal luteinization (Diagnostics (Basel) 2022;12:2706)
  • Virilization
  • Hirsutism
  • Endometrial hyperplasia
  • Cushing syndrome
• Constipation due to inhibitory effect on gastrointestinal motility of peptide YY secreted in strumal carcinoid (Obstet Gynecol Sci 2017;60:602)
• Hyperthyroidism in strumal carcinoid

Contributed by Chenxi Wu, M.D., Ph.D.

• 18 year old woman with mucinous adenocarcinoma and carcinoid tumor arising within an ovarian mature cystic teratoma (Oman Med J 2023;38:e538)
• 40 year old woman with insular carcinoid with hyperandrogenism and carcinoid heart syndrome (Am J Case Rep 2022;23:e937403)
• 40 year old woman with trabecular carcinoid tumor arising from a mature cystic teratoma (Ci Ji Yi Xue Za Zhi 2019;3:192)
• 51 year old postmenopausal woman with primary ovarian carcinoid exhibiting mixed growth pattern (Medicine (Baltimore) 2023;102:e34391)
• 56 year old woman with ovarian strumal carcinoid (Fukushima J Med Sci 2023;69:51)

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AFIP images

Contributed by Krisztina Hanley, M.D., Rulong Shen, M.D. and AFIP

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• Right ovary, oophorectomy:
  • Ovarian carcinoid tumor, arising from struma ovarii (see comment)
  • Comment: Sections show extensive involvement of the ovary by a well differentiated neuroendocrine tumor, showing acinar, trabecular, tubular, corded and some rare areas of solid growth patterns, confined to the ovary without surface involvement. Background shows benign thyroid tissue. Immunohistochemical stains including chromogranin and synaptophysin support neuroendocrine differentiation. Given the presence of benign thyroid tissue, this neoplasm is classified as a strumal carcinoid. The use of proliferative markers such as Ki67 has not been established in the grading of these tumors and in this case, it is < 1%

• Left ovarian cyst, resection:
  • Ovarian carcinoid tumor (4.5 mm), trabecular type, arising in a mature cystic teratoma (dermoid cyst) (see comment)
  • Comment: Extensive sampling of this mature teratoma shows a 4.5 mm trabecular carcinoid tumor. Immunohistochemical stains confirm neuroendocrine differentiation as the tumor cells are positive for synaptophysin, chromogranin, CD56, MCK (AE1 / AE3) and Ki67 is ~2%. This small focus of carcinoid tumor shows CDX2 positivity indicating midgut origin, similar to the gastrointestinal counterpart.

Which of the following is true for primary ovarian carcinoid?

1. Most cases of primary ovarian carcinoid are associated with mature cystic teratoma
2. Mucinous ovarian carcinoid behaves less aggressively than other types
3. Primary ovarian carcinoids are poorly differentiated neuroendocrine tumors
4. To cause carcinoid syndrome, the presence of liver metastasis is required

A. Most cases of primary ovarian carcinoid are associated with mature cystic teratoma. Despite mucinous ovarian carcinoids more frequently occurring in pure form, 3 of 4 types, including the most common insular type, mostly arise in mature cystic teratoma. Answer B is incorrect because mucinous ovarian carcinoid is more aggressive than other types of primary ovarian carcinoid. Answer C is incorrect because per definition, primary ovarian carcinoid tumors are well differentiated neuroendocrine tumors. Answer D is incorrect because ovarian veins drain directly into systemic circulation: the right ovarian vein drains directly into the inferior vena cava, while the left ovarian vein joins the left renal vein. Therefore, hormones produced by a carcinoid tumor do not undergo hepatic metabolism.

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Reference: Carcinoid tumor

Which of the following indicates poor prognosis of patients with primary ovarian carcinoid?

1. Carcinoid heart syndrome
2. Insular type
3. Low Ki67 proliferative index
4. Trabecular type
5. Tumor confined to the ovary

A. Carcinoid heart syndrome can be a presenting symptom even in the absence of liver metastases. The 3 year survival has been reported to be 31%. Answer B is incorrect because insular type is slow growing and only occasionally metastasizes. The prognosis is favorable. Answer C is incorrect because a high Ki67 proliferative index, not low, is associated with worse outcomes. Answer D is incorrect because trabecular type is not associated with metastasis and has favorable prognosis. Answer E is incorrect because the absence of tumor spread is a good prognostic factor.

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Reference: Carcinoid tumor