Hepatic cysts: Reappraisal of the classification, terminology, differential diagnosis, and clinicopathologic characteristics in 258 cases Journal Article


Authors: Armutlu, A.; Quigley, B.; Choi, H.; Basturk, O.; Akkas, G.; Pehlivanoglu, B.; Memis, B.; Jang, K. T.; Erkan, M.; Erkan, B.; Balci, S.; Saka, B.; Bagci, P.; Farris, A. B.; Kooby, D. A.; Martin, D.; Kalb, B.; Maithel, S. K.; Sarmiento, J.; Reid, M. D.; Adsay, N. V.
Article Title: Hepatic cysts: Reappraisal of the classification, terminology, differential diagnosis, and clinicopathologic characteristics in 258 cases
Abstract: 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. © 2022 Lippincott Williams and Wilkins. All rights reserved.
Keywords: middle aged; clinical feature; disease classification; pancreatic neoplasms; nuclear magnetic resonance imaging; preoperative evaluation; computer assisted tomography; diagnosis, differential; differential diagnosis; cystadenoma; pathology; age; liver tumor; pancreas tumor; liver disease; hepatectomy; liver diseases; bile duct neoplasms; bile ducts, intrahepatic; nomenclature; lobectomy; partial hepatectomy; cystadenocarcinoma; bile duct cancer; sex; cyst; cysts; biliary tract disease; liver cyst; congenital malformation; intrahepatic bile duct; fenestration; neoplasia; hepatic cysts; neoplastic cyst; cystic duct; choledochal cyst; common bile duct cyst; humans; human; male; female; article; bile duct cyst; segmentectomy; ductal plate malformation; foregut cyst; biliary cystadenoma; biliary cystadenocarcinoma; liver cysts; echinococcosis
Journal Title: American Journal of Surgical Pathology
Volume: 46
Issue: 9
ISSN: 0147-5185
Publisher: Lippincott Williams & Wilkins  
Date Published: 2022-09-01
Start Page: 1219
End Page: 1233
Language: English
DOI: 10.1097/pas.0000000000001930
PUBMED: 35778790
PROVIDER: scopus
DOI/URL:
Notes: Article -- Export Date: 3 October 2022 -- Source: Scopus
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  1. Olca Basturk
    352 Basturk