Ovary tumor
Germ cell tumors
Carcinoid tumor

Author: Rulong Shen, M.D. (see Authors page)

Revised: 2 May 2018, last major update April 2018

Copyright: (c) 2003-2018, PathologyOutlines.com, Inc.

PubMed Search: Ovary carcinoid tumors AND (free full text[sb] AND Humans[Mesh])

Cite this page: Shen, R. Carcinoid tumor. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/ovarytumorcarcinoid.html. Accessed May 27th, 2018.
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); 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 in general
  • Median survival time is 14 years for primary and 6 years for metastatic ovarian carcinoid patients (Endocr Pathol 2018;29:43)
Case reports
Treatment
  • Excision, although mucinous types are more aggressive
Gross description
  • Mean 10 cm, yellow, solid cut surface; usually unilateral
Gross images

Images hosted on PathOut server:

Contributed by AFIP
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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 (see goblet cell carcinoid images below) or signet ring pattern
Microscopic (histologic) images
Images hosted on PathOut server:

Contributed by Rulong Shen, M.D.
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Carcinoid H&E


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AE1 / AE3

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CDX2

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Synaptophysin

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Chromogranin

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Inhibin

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Carcinoid TTF1


Contributed by AFIP
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Goblet cell carcinoid

Insular tumor


Trabecular tumor

Positive stains
Negative stains
Electron microscopy description
  • Neurosecretory granules
Differential diagnosis
Board review question #1
    Which of the following indicate poor prognosis of patients with primary ovarian carcinoid?

  1. High Ki67 proliferative index
  2. Metastasis
  3. Tumor confined to the ovary
  4. All of the above
  5. Answers A and B
Board review answer #1
E. Answers A and B
Board review question #2
    Which of the following is true for ovarian carcinoid?

  1. Most cases are metastasis
  2. Most cases of primary ovarian carcinoid are associated with cystic teratoma
  3. Ovarian carcinoid can produce carcinoid syndrome
  4. All of the above
Board review answer #2
E. All of the above
Board review question #3
    Which of the following is true for ovarian insular carcinoid?

  1. It is the most common type carcinoid of ovary.
  2. The CDX2 can be positive, but negative for CK7, CK20 and TTF1.
  3. Liver metastases are not a requirement for the development of carcinoid syndrome.
  4. Answers A and B
  5. All of the above
Board review answer #3
E. All of the above