Difference between revisions of "Clear cell carcinoma of the uterine cervix"

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#redirect [[Uterine_cervix#Clear_cell_carcinoma_of_the_uterine_cervix]]
{{ Infobox diagnosis
| Name      = {{PAGENAME}}
| Image      = Clear cell carcinoma - gynecologic tract -- very high mag.jpg
| Width      =
| Caption    = Clear cell carcinoma of the gynecologic tract with focal clear cells. [[H&E stain]].
| Synonyms  =
| Micro      = cells with clear cytoplasm (may be focal), hobnail pattern), moderate-to-severe [[nuclear pleomorphism]], usu. tubular and/or cystic morphology (may be (simple) papillary and/or solid)
| Subtypes  =
| LMDDx      = [[clear cell carcinoma of the vagina]], [[clear cell carcinoma of the endometrium]], [[serous carcinoma of the endometrium]], [[serous carcinoma]] of the uterine cervix, [[mesonephric adenocarcinoma]], other [[clear cell tumours]]
| Stains    = PAS +ve, PASD -ve
| IHC        = CK7 +ve, ER -ve, PR -ve, CA-125 +ve, CEA -ve
| EM        =
| Molecular  =
| IF        =
| Gross      =
| Grossing  =
| Staging    =
| Site      = [[uterine cervix]]
| Assdx      =
| Syndromes  =
| Clinicalhx = +/-diethylstilbestrol (DES) exposure, "young" (median age ~38 years old)
| Signs      =
| Symptoms  =
| Prevalence = rare
| Bloodwork  =
| Rads      =
| Endoscopy  =
| Prognosis  =
| Other      =
| ClinDDx    =
| Tx        =
}}
'''Clear cell carcinoma of the uterine cervix''', also '''cervical clear cell carcinoma''', is a rare malignancy of the [[uterine cervix]] that classically is associated with diethylstilbestrol (DES) exposure.  It typically occurs in younger women.
 
==General==
*Associated with ''diethylstilbestrol'' exposure ''in utero''.<ref name=pmid19857300>{{Cite journal  | last1 = van Dijck | first1 = JA. | last2 = Doorduijn | first2 = Y. | last3 = Bulten | first3 = JH. | last4 = Verloop | first4 = J. | last5 = Massuger | first5 = LF. | last6 = Kiemeney | first6 = BA. | title = [Vaginal and cervical cancer due to diethylstilbestrol (DES); end epidemic] | journal = Ned Tijdschr Geneeskd | volume = 153 | issue =  | pages = A366 | month =  | year = 2009 | doi =  | PMID = 19857300 }}</ref>
*Less common in the cervix - when compared to other gynecologic sites.<ref name=Ref_WMSP442>{{Ref WMSP|442}}</ref>
**More common in the [[clear cell carcinoma of the endometrium|endometrium]].<ref>{{Cite journal  | last1 = Babić | first1 = D. | last2 = Kos | first2 = M. | last3 = Jukić | first3 = S. | last4 = Ilić | first4 = J. | last5 = Vecek | first5 = N. | last6 = Kos | first6 = M. | last7 = Mahnik | first7 = N. | title = [Clear cell carcinoma of the female genital tract]. | journal = Jugosl Ginekol Perinatol | volume = 31 | issue = 3-4 | pages = 102-4 | month =  | year =  | doi =  | PMID = 1749271 }}</ref>
*Typical young women - median age ~38 in one series.<ref name=pmid24470762>{{Cite journal  | last1 = Jiang | first1 = X. | last2 = Jin | first2 = Y. | last3 = Li | first3 = Y. | last4 = Huang | first4 = HF. | last5 = Wu | first5 = M. | last6 = Shen | first6 = K. | last7 = Pan | first7 = LY. | title = Clear cell carcinoma of the uterine cervix: clinical characteristics and feasibility of fertility-preserving treatment. | journal = Onco Targets Ther | volume = 7 | issue =  | pages = 111-6 | month =  | year = 2014 | doi = 10.2147/OTT.S53204 | PMID = 24470762 }}</ref>
 
Note:
*[[HPV]] does '''not''' appear to be important in the oncogenesis;<ref name=pmid21620450>{{Cite journal  | last1 = Kocken | first1 = M. | last2 = Baalbergen | first2 = A. | last3 = Snijders | first3 = PJ. | last4 = Bulten | first4 = J. | last5 = Quint | first5 = WG. | last6 = Smedts | first6 = F. | last7 = Meijer | first7 = CJ. | last8 = Helmerhorst | first8 = TJ. | title = High-risk human papillomavirus seems not involved in DES-related and of limited importance in nonDES related clear-cell carcinoma of the cervix. | journal = Gynecol Oncol | volume = 122 | issue = 2 | pages = 297-302 | month = Aug | year = 2011 | doi = 10.1016/j.ygyno.2011.05.002 | PMID = 21620450 }}</ref> however, this is not completely settled.<ref name=pmid22885379/>
 
==Microscopic==
Features:<ref name=pmid22885379/>
*Cells with clear cytoplasm - '''key feature'''.
**May be focal/absent!
**Cells have large free/luminal surface area (hobnailing pattern) and small non-free surface.
*Moderate-to-severe [[nuclear pleomorphism]].
*Tubular and/or cystic morphology.
**May be (simple) papillary and/or solid.
*Adenosis - typically adjacent.
 
DDx:
*[[Clear cell carcinoma of the endometrium]] - diagnosis based on clinico-radiologic correlation (IHC & histology not useful).<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>
*[[Serous carcinoma of the endometrium]].
*[[Serous carcinoma]] of the uterine cervix.
*[[Mesonephric adenocarcinoma]].
 
===Images===
www:
*[http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3891640/figure/f1-ott-7-111/ Cervical CCC (nih.gov)].<ref name=pmid24470762/>
*[http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3862311/figure/f0010/ Cervical CCC (nih.gov)].
 
==IHC==
Features:<ref>{{Ref_GP|120}}</ref>
*CK7 +ve.
*ER -ve.
*PR -ve.
 
Others - one case only:<ref>{{Cite journal  | last1 = Terada | first1 = T. | title = Clear cell adenocarcinoma of the uterine cervix in a young pregnant woman: a case report with immunohistochemical study. | journal = Med Oncol | volume = 28 | issue = 1 | pages = 290-3 | month = Mar | year = 2011 | doi = 10.1007/s12032-009-9410-x | PMID = 20054717 }}</ref>
*CA-125 +ve.
*p53 +ve.
*CEA -ve.
*CK34betaE12 -ve.
 
==See also==
*[[Uterine cervix]].
*[[Clear cell carcinoma]].
*[[Clear cell carcinoma of the vagina]].
 
==References==
{{Reflist|2}}


[[Category:Diagnosis]]
[[Category:Diagnosis]]
[[Category:Uterine cervix]]

Latest revision as of 06:45, 30 December 2015

Clear cell carcinoma of the uterine cervix
Diagnosis in short

Clear cell carcinoma of the gynecologic tract with focal clear cells. H&E stain.

LM cells with clear cytoplasm (may be focal), hobnail pattern), moderate-to-severe nuclear pleomorphism, usu. tubular and/or cystic morphology (may be (simple) papillary and/or solid)
LM DDx clear cell carcinoma of the vagina, clear cell carcinoma of the endometrium, serous carcinoma of the endometrium, serous carcinoma of the uterine cervix, mesonephric adenocarcinoma, other clear cell tumours
Stains PAS +ve, PASD -ve
IHC CK7 +ve, ER -ve, PR -ve, CA-125 +ve, CEA -ve
Site uterine cervix

Clinical history +/-diethylstilbestrol (DES) exposure, "young" (median age ~38 years old)
Prevalence rare

Clear cell carcinoma of the uterine cervix, also cervical clear cell carcinoma, is a rare malignancy of the uterine cervix that classically is associated with diethylstilbestrol (DES) exposure. It typically occurs in younger women.

General

  • Associated with diethylstilbestrol exposure in utero.[1]
  • Less common in the cervix - when compared to other gynecologic sites.[2]
  • Typical young women - median age ~38 in one series.[4]

Note:

  • HPV does not appear to be important in the oncogenesis;[5] however, this is not completely settled.[6]

Microscopic

Features:[6]

  • Cells with clear cytoplasm - key feature.
    • May be focal/absent!
    • Cells have large free/luminal surface area (hobnailing pattern) and small non-free surface.
  • Moderate-to-severe nuclear pleomorphism.
  • Tubular and/or cystic morphology.
    • May be (simple) papillary and/or solid.
  • Adenosis - typically adjacent.

DDx:

Images

www:

IHC

Features:[7]

  • CK7 +ve.
  • ER -ve.
  • PR -ve.

Others - one case only:[8]

  • CA-125 +ve.
  • p53 +ve.
  • CEA -ve.
  • CK34betaE12 -ve.

See also

References

  1. van Dijck, JA.; Doorduijn, Y.; Bulten, JH.; Verloop, J.; Massuger, LF.; Kiemeney, BA. (2009). "[Vaginal and cervical cancer due to diethylstilbestrol (DES); end epidemic]". Ned Tijdschr Geneeskd 153: A366. PMID 19857300.
  2. Humphrey, Peter A; Dehner, Louis P; Pfeifer, John D (2008). The Washington Manual of Surgical Pathology (1st ed.). Lippincott Williams & Wilkins. pp. 442. ISBN 978-0781765275.
  3. Babić, D.; Kos, M.; Jukić, S.; Ilić, J.; Vecek, N.; Kos, M.; Mahnik, N.. "[Clear cell carcinoma of the female genital tract].". Jugosl Ginekol Perinatol 31 (3-4): 102-4. PMID 1749271.
  4. 4.0 4.1 Jiang, X.; Jin, Y.; Li, Y.; Huang, HF.; Wu, M.; Shen, K.; Pan, LY. (2014). "Clear cell carcinoma of the uterine cervix: clinical characteristics and feasibility of fertility-preserving treatment.". Onco Targets Ther 7: 111-6. doi:10.2147/OTT.S53204. PMID 24470762.
  5. Kocken, M.; Baalbergen, A.; Snijders, PJ.; Bulten, J.; Quint, WG.; Smedts, F.; Meijer, CJ.; Helmerhorst, TJ. (Aug 2011). "High-risk human papillomavirus seems not involved in DES-related and of limited importance in nonDES related clear-cell carcinoma of the cervix.". Gynecol Oncol 122 (2): 297-302. doi:10.1016/j.ygyno.2011.05.002. PMID 21620450.
  6. 6.0 6.1 6.2 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.
  7. Nucci, Marisa R.; Oliva, Esther (2009). Gynecologic Pathology: A Volume in Foundations in Diagnostic Pathology Series (1st ed.). Churchill Livingstone. pp. 120. ISBN 978-0443069208.
  8. Terada, T. (Mar 2011). "Clear cell adenocarcinoma of the uterine cervix in a young pregnant woman: a case report with immunohistochemical study.". Med Oncol 28 (1): 290-3. doi:10.1007/s12032-009-9410-x. PMID 20054717.