Clear cell carcinoma of the uterine cervix

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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.

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

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.
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