Clear cell carcinoma of the uterine cervix

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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 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.
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