Ovary
Germ cell tumors
Carcinoid tumor
Author: Rulong Shen, M.D.
Deputy Editor-in-Chief: Debra L. Zynger, M.D.
Last author update: 1 May 2018
Last staff update: 24 August 2023 (update in progress)
Copyright: 2003-2023, PathologyOutlines.com, Inc.
PubMed Search: Ovary carcinoid tumors
Table of Contents
Definition / general | Clinical features | Case reports | Treatment | Gross description | Gross images | Microscopic (histologic) description | Microscopic (histologic) images | Positive stains | Negative stains | Electron microscopy description | Differential diagnosis | Additional references | Board review style question #1 | Board review style answer #1 | Board review style question #2 | Board review style answer #2 | Board review style question #3 | Board review style question #3 | Board review style question #4 | Board review style question #4Cite this page: Shen R. Carcinoid tumor. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/ovarytumorcarcinoid.html. Accessed September 22nd, 2023.
Definition / general
- Ovarian carcinoid neoplasms are rare, < 0.1% of all ovarian carcinomas
- Primary ovarian carcinoid neoplasms are usually unilateral, localized to the ovary and indistinguishable histologically from metastasis
- Often arise within a cystic teratoma or dermoid tumor (J Egypt Natl Canc Inst 2016;28:267, J Exp Clin Cancer Res 2000;19:271)
- Four distinctive types: insular (most common, ~50%), strumal, trabecular and goblet cell (mucinous)
- Insular carcinoid is the most common type (~ 50%)
Clinical features
- 1/3 are associated with carcinoid syndrome, more often in tumors > 7 cm and > 50 year old
- Median survival time is 14 years for primary and 6 years for metastatic (Endocr Pathol 2018;29:43)
Case reports
- 54 year old woman with primary carcinoid tumor in mature cystic teratoma (Eur J Gynaecol Oncol 2009;30:223)
- 75 year old woman with carcinoid tumor arising in a mature cystic teratoma (Oncol Lett 2015;9:2236)
Treatment
- Excision
- Radical hysterectomy and bilateral salpingo-oophorectomy may be considered in post-menopausal patients
Gross description
- Mean 10 cm, yellow, solid cut surface; usually unilateral
Gross images
AFIP images
Insular tumor
Microscopic (histologic) description
- Resembles carcinoid tumors elsewhere; nuclear features of neuroendocrine tumor such as salt and pepper chromatin may be helpful but are also seen in follicular cells of strumal carcinoid
- Patterns are insular (resembles appendix or small bowel tumors), trabecular (resembles stomach or rectal tumors), strumal (below), mucinous
- All have a gastrointestinal counterpart except strumal carcinoid
- Mixed primary ovarian carcinoids, such as insular and trabecular or strumal and goblet cell do exist
- May have abundant fibrous stroma rich vessels
- Rarely has prominent pleomorphism, mucinous features or signet ring pattern
Microscopic (histologic) images
Contributed by Rulong Shen, M.D.
Insular carcinoid
Insular carcinoid
TTF1
AE1 / AE3
CDX2
Synaptophysin
Chromogranin
Inhibin
AFIP images
Goblet cell carcinoid
Insular tumor
Trabecular tumor
Positive stains
- Pankeratin AE1 / AE3, chromogranin, synaptophysin and CDX2 (insular)
Electron microscopy description
- Neurosecretory granules
Differential diagnosis
- Brenner tumor: nests of urothelial cells with nuclear grooves; chromogranin-
- Fibrothecoma: typical growth pattern, cells with pale nuclei and grooves; inhibin+, SF1+, FOXL2+, chromogranin-
- GI metastasis: bilateral, associated with peritoneal implants and no other teratomatous elements
Additional references
Board review style question #1
- Which of the following indicate poor prognosis of patients with primary ovarian carcinoid?
- High Ki67 proliferative index
- Metastasis
- Tumor confined to the ovary
- All of the above
- Answers A and B
Board review style answer #1
Board review style question #2
- Which of the following is true for ovarian carcinoid?
- Most cases are metastasis
- Most cases of primary ovarian carcinoid are associated with cystic teratoma
- Ovarian carcinoid can produce carcinoid syndrome
- All of the above
Board review style answer #2
Board review style question #3
- Which of the following is true for ovarian insular carcinoid?
- It is the most common type carcinoid of ovary.
- The CDX2 can be positive, but negative for CK7, CK20 and TTF1.
- Liver metastases are not a requirement for the development of carcinoid syndrome.
- Answers A and B
- All of the above
Board review style question #3
Board review style question #4
- Seen in the picture is the most common histologic pattern of ovarian carcinoid tumor. Which pattern is it?
- Insular
- Mucinous
- Stromal
- Trabecular
Board review style question #4
