Difference between revisions of "Clear cell carcinoma of the ovary"

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#redirect [[Ovarian_tumours#Clear_cell_carcinoma_of_the_ovary]]
{{ Infobox diagnosis
| Name      = {{PAGENAME}}
| Image      = Ovarian_clear_cell_carcinoma_-a-_very_high_mag_-_cropped.jpg
| Width      =
| Caption    = Ovarian clear cell carcinoma. [[H&E stain]].
| Synonyms  = ovarian clear cell carcinoma, ovarian clear cell adenocarcinoma, clear cell adenocarcinoma of the ovary
| Micro      = cystic/tubular architecture (may be papillary and/or solid), clear cytoplasm (may be sparse), [[hobnail morphology]] - apical surface larger than basal surface ("nuclei bulge into the lumen"), +/-hyaline droplets, prominent [[nucleoli]]
| Subtypes  =
| LMDDx      = [[serous carcinoma of the ovary]], other [[serous carcinoma]]s, other [[clear cell tumours]] (e.g. [[clear cell renal cell carcinoma]]), [[Arias-Stella reaction]] within [[endometriosis]]
| Stains    =
| IHC        = [[HNF-1beta]] +ve, WT-1 -ve, [[CK7]] +ve, [[CD10]] -ve, CK20 -ve
| EM        =
| Molecular  =
| IF        =
| Gross      = mass, yellow colour, usu. unilateral
| Grossing  =
| Site      = [[ovary]] - see ''[[ovarian tumours]]''
| Assdx      = [[endometriosis]], [[pulmonary embolism]]
| Syndromes  =
| Clinicalhx =
| Signs      =
| Symptoms  =
| Prevalence = uncommon
| Bloodwork  =
| Rads      =
| Endoscopy  =
| Prognosis  = poor
| Other      =
| ClinDDx    = other [[ovarian tumours]], other pelvic and abdominal tumours
| Tx        =
}}
'''Clear cell carcinoma of the ovary''', also '''ovarian clear cell carcinoma''' (abbreviated '''OCCC'''), is an aggressive [[malignant]] [[ovarian tumours|tumour of the ovary]].
 
It often forms glands; thus, it is also known as '''ovarian clear cell [[adenocarcinoma]]''', and '''clear cell adenocarcinoma of the ovary'''.
 
==General==
Prognosis:
* Worse prognosis versus other surface epithelial tumours.<ref name=pmid18035678>{{cite journal |author=Hauptmann S, Köbel M |title=[Prognostic factors in ovarian carcinoma] |language=German |journal=Verh Dtsch Ges Pathol |volume=89 |issue= |pages=92-100 |year=2005 |pmid=18035678 |doi= |url=}}</ref>
**Poor response to platinum-based chemotherapy.<ref name=pmid22885379/>
**~2.5x risk for thromboembolism compared to other ovarian carcinomas.<ref name=pmid19922988>{{Cite journal  | last1 = Duska | first1 = LR. | last2 = Garrett | first2 = L. | last3 = Henretta | first3 = M. | last4 = Ferriss | first4 = JS. | last5 = Lee | first5 = L. | last6 = Horowitz | first6 = N. | title = When 'never-events' occur despite adherence to clinical guidelines: the case of venous thromboembolism in clear cell cancer of the ovary compared with other epithelial histologic subtypes. | journal = Gynecol Oncol | volume = 116 | issue = 3 | pages = 374-7 | month = Mar | year = 2010 | doi = 10.1016/j.ygyno.2009.10.069 | PMID = 19922988 }}</ref>
 
Epidemiology:
* Asians appear to be at an increased risk.<ref name=pmid22885379>{{Cite journal  | last1 = Offman | first1 = SL. | last2 = Longacre | first2 = TA. | title = Clear cell carcinoma of the female genital tract (not everything is as clear as it seems). | journal = Adv Anat Pathol | volume = 19 | issue = 5 | pages = 296-312 | month = Sep | year = 2012 | doi = 10.1097/PAP.0b013e31826663b1 | PMID = 22885379 }}</ref>
* Thought to be related to endometrioid carcinoma.<ref name=Ref_PBoD1098>{{Ref PBoD |1098}}</ref>
*Association with endometriosis:<ref name=pmid22885379/>
** Increased risk of ovarian clear cell carcinoma ~ 3x.
** Seen in 50-70% of ovarian CCC.
 
==Gross==
Features:<ref name=pmid22885379/>
*Usually unilateral (unlike [[serous carcinoma of the ovary|serous carcinoma]]).
*Yellow colour.
 
==Microscopic==
Features:
*Cystic/tubular architecture - important low power feature.
**May be papillary and/or solid.
*Clear cells - cytoplasm is clear - '''key feature'''. †
*[[Hobnail morphology]] - apical surface larger than basal surface.
**"Nuclei bulge into the lumen".
*Hyaline droplets -- common, as in ''[[clear cell renal cell carcinoma]]''.
**Eosinophilic bodies within lumen.
**Seen in approximately 1/4 of cases.<ref name=pmid22885379/>
*Nucleoli - prominent.
 
Note:
*† Clear cell adenocarcinoma does not have to have clear cells. Yes, this is stupid; it is like ''[[papillary thyroid carcinoma]]'' -- which often isn't papillary -- see ''[[no truth in names]]''.
**The ''hobnail morphology'' is important if this is the case.
 
DDx:
*[[Serous carcinoma of the ovary]] - have larger papillae +/- branching +/-multilayered epithelium, more mitotic activity,<ref name=pmid22885379/> more group pleomorphism.
*[[Serous borderline tumour of the ovary]].
*High-grade [[endometrioid carcinoma of the ovary]].
*[[Metastasis|Metatstatic]] [[clear cell carcinoma]], esp. [[clear cell renal cell carcinoma]].
*Borderline clear cell lesion of the ovary - very rare; an under sampled carcinoma considered more probable.<ref name=pmid22885379/>
*[[Arias-Stella reaction]] within [[endometriosis]].
 
===Images===
<gallery>
Image:Ovarian_clear_cell_carcinoma_-a-_very_high_mag_-_cropped.jpg | Clear cell carcinoma - very high mag. - cropped (WC)
Image:Ovarian_clear_cell_carcinoma_-a-_intermed_mag.jpg | Clear cell carcinoma - intermed. mag. (WC)
Image:Ovarian_clear_cell_carcinoma_-_low_mag.jpg | Clear cell carcinoma - low mag. (WC)
Image:Ovarian clear cell carcinoma - intermed mag.jpg | Clear cell carcinoma - intermed. mag. (WC)
Image:Ovarian clear cell carcinoma - high mag.jpg | Clear cell carcinoma - high mag. (WC)
Image:Ovarian clear cell carcinoma - very high mag.jpg | Clear cell carcinoma - very high mag. (WC)
</gallery>
www:
*[http://path.upmc.edu/cases/case343.html Clear cell carcinoma of the ovary - several images (upmc.edu)].
 
==IHC==
*HNF-1beta +ve.<ref>{{Cite journal  | last1 = Tsuchiya | first1 = A. | last2 = Sakamoto | first2 = M. | last3 = Yasuda | first3 = J. | last4 = Chuma | first4 = M. | last5 = Ohta | first5 = T. | last6 = Ohki | first6 = M. | last7 = Yasugi | first7 = T. | last8 = Taketani | first8 = Y. | last9 = Hirohashi | first9 = S. | title = Expression profiling in ovarian clear cell carcinoma: identification of hepatocyte nuclear factor-1 beta as a molecular marker and a possible molecular target for therapy of ovarian clear cell carcinoma. | journal = Am J Pathol | volume = 163 | issue = 6 | pages = 2503-12 | month = Dec | year = 2003 | doi =  | PMID = 14633622 | url = http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1892387/?tool=pubmed }}</ref><ref name=omim189907>{{OMIM|189907}}</ref>
**Usu. -ve in serous carcinoma.
*WT-1 -ve.
**Usu. +ve in serous carcinoma.
 
Panel for high grade serous vs. clear cell:<ref name=pmid18830127>{{cite journal |author=Köbel M, Kalloger SE, Carrick J, ''et al.'' |title=A limited panel of immunomarkers can reliably distinguish between clear cell and high-grade serous carcinoma of the ovary |journal=Am. J. Surg. Pathol. |volume=33 |issue=1 |pages=14–21 |year=2009 |month=January |pmid=18830127 |doi=10.1097/PAS.0b013e3181788546 |url=}}</ref>
*ER (+ve in serous),<ref name=pmid21164285>{{Cite journal  | last1 = DeLair | first1 = D. | last2 = Oliva | first2 = E. | last3 = Köbel | first3 = M. | last4 = Macias | first4 = A. | last5 = Gilks | first5 = CB. | last6 = Soslow | first6 = RA. | title = Morphologic spectrum of immunohistochemically characterized clear cell carcinoma of the ovary: a study of 155 cases. | journal = Am J Surg Pathol | volume = 35 | issue = 1 | pages = 36-44 | month = Jan | year = 2011 | doi = 10.1097/PAS.0b013e3181ff400e | PMID = 21164285 }}</ref> HNF-1beta (+ve in clear cell), WT-1 (+ve in serous).
 
Others:<ref name=pmid22885379/>
*CK7 +ve -- may be focal.
*CK20 -ve.
*CD10 -ve.
**Often +ve in [[clear cell renal cell carcinoma]].
 
==See also==
*[[Serous carcinoma]].
*[[Ovarian tumours]].
*[[Ovary]].
*[[Clear cell tumours]].
 
==References==
{{Reflist|2}}


[[Category:Diagnosis]]
[[Category:Diagnosis]]
[[Category:Ovarian tumours]]

Latest revision as of 21:27, 2 July 2020

Clear cell carcinoma of the ovary
Diagnosis in short

Ovarian clear cell carcinoma. H&E stain.
Synonyms ovarian clear cell carcinoma, ovarian clear cell adenocarcinoma, clear cell adenocarcinoma of the ovary
LM cystic/tubular architecture (may be papillary and/or solid), clear cytoplasm (may be sparse), hobnail morphology - apical surface larger than basal surface ("nuclei bulge into the lumen"), +/-hyaline droplets, prominent nucleoli
LM DDx serous carcinoma of the ovary, other serous carcinomas, other clear cell tumours (e.g. clear cell renal cell carcinoma), Arias-Stella reaction within endometriosis
IHC HNF-1beta +ve, WT-1 -ve, CK7 +ve, CD10 -ve, CK20 -ve
Gross mass, yellow colour, usu. unilateral
Site ovary - see ovarian tumours
Associated Dx endometriosis, pulmonary embolism
Prevalence uncommon
Prognosis poor
Clin. DDx other ovarian tumours, other pelvic and abdominal tumours

Clear cell carcinoma of the ovary, also ovarian clear cell carcinoma (abbreviated OCCC), is an aggressive malignant tumour of the ovary.

It often forms glands; thus, it is also known as ovarian clear cell adenocarcinoma, and clear cell adenocarcinoma of the ovary.

General

Prognosis:

  • Worse prognosis versus other surface epithelial tumours.[1]
    • Poor response to platinum-based chemotherapy.[2]
    • ~2.5x risk for thromboembolism compared to other ovarian carcinomas.[3]

Epidemiology:

  • Asians appear to be at an increased risk.[2]
  • Thought to be related to endometrioid carcinoma.[4]
  • Association with endometriosis:[2]
    • Increased risk of ovarian clear cell carcinoma ~ 3x.
    • Seen in 50-70% of ovarian CCC.

Gross

Features:[2]

Microscopic

Features:

  • Cystic/tubular architecture - important low power feature.
    • May be papillary and/or solid.
  • Clear cells - cytoplasm is clear - key feature. †
  • Hobnail morphology - apical surface larger than basal surface.
    • "Nuclei bulge into the lumen".
  • Hyaline droplets -- common, as in clear cell renal cell carcinoma.
    • Eosinophilic bodies within lumen.
    • Seen in approximately 1/4 of cases.[2]
  • Nucleoli - prominent.

Note:

  • † Clear cell adenocarcinoma does not have to have clear cells. Yes, this is stupid; it is like papillary thyroid carcinoma -- which often isn't papillary -- see no truth in names.
    • The hobnail morphology is important if this is the case.

DDx:

Images

  • Clear cell carcinoma - very high mag. - cropped (WC)

  • Clear cell carcinoma - intermed. mag. (WC)

  • Clear cell carcinoma - low mag. (WC)

  • Clear cell carcinoma - intermed. mag. (WC)

  • Clear cell carcinoma - high mag. (WC)

  • Clear cell carcinoma - very high mag. (WC)

www:

IHC

  • HNF-1beta +ve.[5][6]
    • Usu. -ve in serous carcinoma.
  • WT-1 -ve.
    • Usu. +ve in serous carcinoma.

Panel for high grade serous vs. clear cell:[7]

  • ER (+ve in serous),[8] HNF-1beta (+ve in clear cell), WT-1 (+ve in serous).

Others:[2]

See also

References

  1. ↑ Hauptmann S, Köbel M (2005). "[Prognostic factors in ovarian carcinoma]" (in German). Verh Dtsch Ges Pathol 89: 92-100. PMID 18035678.
  2. ↑ 2.0 2.1 2.2 2.3 2.4 2.5 2.6 2.7 Offman, SL.; Longacre, TA. (Sep 2012). "Clear cell carcinoma of the female genital tract (not everything is as clear as it seems).". Adv Anat Pathol 19 (5): 296-312. doi:10.1097/PAP.0b013e31826663b1. PMID 22885379.
  3. ↑ Duska, LR.; Garrett, L.; Henretta, M.; Ferriss, JS.; Lee, L.; Horowitz, N. (Mar 2010). "When 'never-events' occur despite adherence to clinical guidelines: the case of venous thromboembolism in clear cell cancer of the ovary compared with other epithelial histologic subtypes.". Gynecol Oncol 116 (3): 374-7. doi:10.1016/j.ygyno.2009.10.069. PMID 19922988.
  4. ↑ Cotran, Ramzi S.; Kumar, Vinay; Fausto, Nelson; Nelso Fausto; Robbins, Stanley L.; Abbas, Abul K. (2005). Robbins and Cotran pathologic basis of disease (7th ed.). St. Louis, Mo: Elsevier Saunders. pp. 1098. ISBN 0-7216-0187-1.
  5. ↑ Tsuchiya, A.; Sakamoto, M.; Yasuda, J.; Chuma, M.; Ohta, T.; Ohki, M.; Yasugi, T.; Taketani, Y. et al. (Dec 2003). "Expression profiling in ovarian clear cell carcinoma: identification of hepatocyte nuclear factor-1 beta as a molecular marker and a possible molecular target for therapy of ovarian clear cell carcinoma.". Am J Pathol 163 (6): 2503-12. PMID 14633622. http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1892387/?tool=pubmed.
  6. ↑ Online 'Mendelian Inheritance in Man' (OMIM) 189907
  7. ↑ Köbel M, Kalloger SE, Carrick J, et al. (January 2009). "A limited panel of immunomarkers can reliably distinguish between clear cell and high-grade serous carcinoma of the ovary". Am. J. Surg. Pathol. 33 (1): 14–21. doi:10.1097/PAS.0b013e3181788546. PMID 18830127.
  8. ↑ DeLair, D.; Oliva, E.; Köbel, M.; Macias, A.; Gilks, CB.; Soslow, RA. (Jan 2011). "Morphologic spectrum of immunohistochemically characterized clear cell carcinoma of the ovary: a study of 155 cases.". Am J Surg Pathol 35 (1): 36-44. doi:10.1097/PAS.0b013e3181ff400e. PMID 21164285.
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