Ovary tumor
Clear cell carcinoma
Clear cell carcinoma

Author: Sonali Lanjewar, M.D., M.B.B.S.
Editor: Raavi Gupta, M.D.
Deputy Editor in Chief: Debra Zynger, M.D.

Revised: 17 July 2018, last major update April 2018

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

PubMed Search: Ovary tumor clear cell carcinoma [title] AND (free full text[sb] AND Humans[Mesh])

Related topics: Oxyphilic variant
Page views in 2018: 16,394
Page views in 2019 to date: 1,262
Cite this page: Lanjewar, S. Clear cell carcinoma. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/ovarytumorclearcell.html. Accessed January 24th, 2019.
Definition / general
Diagrams / tables

Images hosted on PathOut server:

Contributed by Ayse Ayhan, M.D.
Missing Image

Clear cell carcinoma, case 1: Schema

Clinical features
  • Frequent association with ovarian or pelvic endometriosis (Gynecol Oncol 2010;116:374)
  • Commonly associated with paraneoplastic hypercalcemia and venous thromboembolism
Radiology description
  • Clear cell carcinomas are commonly identified on USG by the presence of a single large cystic mass with smooth marginated solid component
  • Presence of ground glass appearance in a cyst suggests an endometrioma, which in postmenopausal women should be evaluated carefully to rule out a component of clear cell carcinoma (Ultrasonography 2015;34:173)
Prognostic factors
  • When confined to the pelvis, clear cell carcinoma has much better prognosis than high grade serous carcinoma
  • However, advanced stage clear cell carcinoma has a worse prognosis (Am J Surg Pathol 2011;35:36)
  • Presence of GPC3 is associated with poor prognosis (J Clin Pathol 2010;63:962),
Case reports
  • Comprehensive surgical staging is required in early stage clear cell carcinoma, which includes bilateral salpingo-oophorectomy, total hysterectomy, lymph node dissection and omental biopsies
  • At an advanced stage standard treatment of paclitaxel plus carboplatin is recommended (Ann Oncol 2016;27:i50)
  • Clear cell carcinoma is resistant to platinum based chemotherapy, making metastatic disease difficult to treat (Am J Surg Pathol 2011;35:36)
Gross description
  • Commonly unilateral with a size up to 30 cm (mean 15 cm)
  • Cut surface is thick walled unilocular cyst with multiple yellow-beige fleshy nodules protruding into the lumen, these nodules may be lined by an endometriotic cyst which typically contains chocolate–brown fluid
Gross images

Images hosted on PathOut server:

Contributed by Ayse Ayhan, M.D.
Clear cell carcinoma, case 1

Outer appearance

Cut surface

Fixed pictures of surgical material

Clear cell carcinoma, case 2

Outer appearance

Cut surface

Fixed cut surface

Contributed by AFIP

Polypoid tumor arising
in endometriotic cyst

Microscopic (histologic) description
  • Tubulocystic, papillary and solid patterns
  • Papillae are small, regular and frequently hyalinized
  • Tumor cells are polygonal to cuboidal and flattened with clear to eosinophilic cytoplasm; may have hobnail morphology
  • Tubules and cysts contain dense eosinophilic "targetoid" secretions
  • Intracytoplasmic mucin can give signet ring appearance
  • Eosinophilic hyaline bodies can be seen (Am J Surg Pathol 2011;35:36, Adv Anat Pathol 2012;19:296)
Microscopic (histologic) images

Images hosted on PathOut server:

Contributed by Sonali Lanjewar, M.D., M.B.B.S.

Clear cell carcinoma


Left: cystic pattern; right: lined by atypical hobnail cells

Poorly differentiated

With adenofibroma component

With extensive round cell infiltration

Numerous intracytoplasmic hyaline bodies


Images hosted on other servers:

Contributed by Dr. Semir Vranic

Positive stains
Negative stains
Molecular / cytogenetics description
Differential diagnosis
Board review question #1
    When associated with Lynch syndrome, most common germline mutation seen in clear cell carcinoma is:

  1. BRAF mutation
  2. Methylation of MLH1 promoter
  3. MSH2 mutation
  4. All of the above
Board review answer #1
C. MSH2 mutation