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ovarian clear cell carcinoma

Wednesday 6 May 2009

clear cell carcinoma of the ovary


Definition: Ovarian clear cell carcinomas (CCC) typically present as large adnexal, stage I tumors and are generally considered highly malignant. They are frequently associated with endometriosis and, less often with clear cell adenofibromas.

Subdividing ovarian CCCs into cystic and adenofibromatous CCC reveals differences in a number of clinicopathologic features including their association with endometriosis, histologic patterns, stage distribution, and clinical behavior.


- Ovarian clear cell carcinoma

- Ovarian clear cell carcinoma with ’Bull’s eye’-type cells and Napsin-A+

- Clear cell ovarian carcinoma: hyalinized papillae, hobnailing, atypia with few mitoses

- Ovarian clear cell carcinoma with thick basement membrane


- Spongy, cystic


- Tubular-cystic pattern , papillary pattern or solid pattern
- Papillary cores have prominent hyalinization
- Large tumor cells, some with nuclei that protrude into lumina (hobnail), clear cytoplasm (glycogen, mucin, fat)
- Resembles mucinous tumor on frozen section
- tubulocystic pattern + papillary pattern
- hobnail cells
- PAS+ cytoplasm
- ER-
- CK7+
- EMA+
- 50% have ARID1A mutations


- cystic ovarian clear cell carcinoma
- adenofibromatous ovarian clear cell carcinoma


- Positive Stains

  • Keratin, Leu-M1 (90%), hyaline globules are PAS+ diastase resistant but AFP negative (Am J Clin Pathol 1989;91:511)
  • VHL gene product (90%, Am J Clin Pathol 2008;129:592),
  • hepatocyte nuclear factor 1 beta (Mod Pathol 2006;19:83)
  • Napsin A (Mod Pathol 2015;28:111)

- Negative Stains

  • AFP, WT1, ER, usually GPC3

Differential Diagnosis

- ovarian dysgerminoma

  • Oct3/4+

- ovarian serous carcinoma, high grade

  • usually WT1+, hepatocyte nuclear factor 1 beta negative, often ER+ (Am J Surg Pathol 2009;33:14)

- yolk sac tumor

  • AFP+, GPC3+ (Am J Surg Pathol 2008;32:600)

See also

- ovarian carcinomas

- clear cell tumors


- Cystic and Adenofibromatous Clear Cell Carcinomas of the Ovary: Distinctive Tumors That Differ in Their Pathogenesis and Behavior: A Clinicopathologic Analysis of 122 Cases. Veras E, Mao TL, Ayse A, Ueda S, Lai H, Hayran M, Shih IM, Kurman RJ. Am J Surg Pathol. 2009 Apr 1. PMID: 19342944