ovarian clear cell carcinoma

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.

Images

 Ovarian clear cell carcinoma

• https://twitter.com/solcosao/status/732734222419398656
• https://twitter.com/seattlequinns/status/793341519046422528
• https://twitter.com/AltaherN88/status/826716995349536769
• https://twitter.com/davidacohenmd/status/852587764612829184

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

• https://twitter.com/Konzult_/status/741253352797941760

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

• https://twitter.com/BeynonMD/status/723308225316048898

 Ovarian clear cell carcinoma with thick basement membrane

• https://twitter.com/davidacohenmd/status/852587764612829184

Macroscopy

 Spongy, cystic

Microscopy

 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

Subtypes

 cystic ovarian clear cell carcinoma
 adenofibromatous ovarian clear cell carcinoma

IHC

 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

• clear cell carcinomas

References

 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