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.

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]

  • Like clear cell carcinoma elsewhere:
    • Clear cytoplasm - key feature.
      • May be 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

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. 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 6.3 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.