Hepatic Cysts: Reappraisal of the Classification, Terminology, Differential Diagnosis, and Clinicopathologic Characteristics in 258 Cases

Ayse Armutlu, Brian Quigley, Hegyong Choi, Olca Basturk, Gizem Akkas, Burcin Pehlivanoglu, Bahar Memis, Kee Taek Jang, Mert Erkan, Burcu Erkan, Serdar Balci, Burcu Saka, Pelin Bagci, Alton B. Farris, David A. Kooby, Diego Martin, Bobby Kalb, Shishir K. Maithel, Juan Sarmiento, Michelle D. ReidN. Volkan Adsay

Research output: Contribution to journalArticlepeer-review

3 Scopus citations


The literature on liver cysts is highly conflicting, mostly owing to definitional variations. Two hundred and fifty-eight ≥1 cm cysts evaluated pathologically using updated criteria were classifiable as: I. Ductal plate malformation related (63%); that is, cystic bile duct hamartoma or not otherwise specified-type benign biliary cyst (35 with polycystic liver disease). These were female predominant (F/M=2.4), large (10 cm), often multifocal with degenerative/inflammatory changes and frequently misclassified as "hepatobiliary cystadenoma." II. Neoplastic (13%); 27 (10.5%) had ovarian-type stroma (OTS) and qualified as mucinous cystic neoplasm (MCN) per World Health Organization (WHO). These were female, solitary, mean age 52, mean size 11 cm, and 2 were associated with carcinoma (1 in situ and 1 microinvasive). There were 3 intraductal papillary neoplasms, 1 intraductal oncocytic papillary neoplasm, 1 cystic cholangiocarcinoma, and 2 cystic metastasis. III. Infectious/inflammatory (12%). These included 23 hydatid cysts (including 2 Echinococcus alveolaris both misdiagnosed preoperatively as cancer), nonspecific inflammatory cysts (abscesses, inflammatory cysts: 3.4%). IV. Congenital (7%). Mostly small (<3 cm); choledochal cyst (5%), foregut cyst (2%). V. Miscellaneous (4%). In conclusion, hepatic cysts occur predominantly in women (3/1), are mostly (90%) non-neoplastic, and seldom (<2%) malignant. Cystic bile duct hamartomas and their relative not otherwise specified-type benign biliary cysts are frequently multifocal and often misdiagnosed as "cystadenoma/carcinoma." Defined by OTS, MCNs (the true "hepatobiliary cystadenoma/carcinoma") are solitary, constitute only 10.5% of hepatic cysts, and have a significantly different profile than the impression in the literature in that essentially all are perimenopausal females, and rarely associated with carcinoma (7%). Since MCNs can only be diagnosed by demonstration of OTS through complete microscopic examination, it is advisable to avoid the term "cystadenoma/cystadenocarcinoma" solely based on radiologic examination, and the following simplified terminology would be preferable in preoperative evaluation to avoid conflicts with the final pathologic diagnosis: (1) noncomplex (favor benign), (2) complex (in 3 subsets, as favor benign, cannot rule out malignancy, or favor malignancy), (3) malignant features.

Original languageEnglish (US)
Pages (from-to)1219-1233
Number of pages15
JournalAmerican Journal of Surgical Pathology
Issue number9
StatePublished - Sep 1 2022


  • biliary cystadenocarcinoma
  • biliary cystadenoma
  • hepatic cysts
  • liver cysts
  • neoplasia
  • Diagnosis, Differential
  • Cystadenoma/pathology
  • Humans
  • Middle Aged
  • Pancreatic Neoplasms/pathology
  • Male
  • Choledochal Cyst/pathology
  • Cystadenocarcinoma/pathology
  • Bile Ducts, Intrahepatic/pathology
  • Cysts
  • Female
  • Liver Diseases
  • Bile Duct Neoplasms/pathology

ASJC Scopus subject areas

  • Anatomy
  • Surgery
  • Pathology and Forensic Medicine


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