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Hepatic Cysts: Reappraisal of the Classification, Terminology, Differential Diagnosis, and Clinicopathologic Characteristics in 258 Cases
The American Journal of Surgical Pathology ( IF 4.5 ) Pub Date : 2022-09-01 , DOI: 10.1097/pas.0000000000001930
Ayse Armutlu 1 , Brian Quigley 2 , Hegyong Choi 3 , Olca Basturk 4 , Gizem Akkas 2 , Burcin Pehlivanoglu 2 , Bahar Memis 2 , Kee-Taek Jang 5 , Mert Erkan 6 , Burcu Erkan 7 , Serdar Balci 2 , Burcu Saka 1 , Pelin Bagci 2 , Alton B. Farris 2 , David A. Kooby 8 , Diego Martin 9 , Bobby Kalb 9 , Shishir K. Maithel 8 , Juan Sarmiento 8 , Michelle D. Reid 2 , N. Volkan Adsay 1
Affiliation  

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.



中文翻译:

肝囊肿:258例肝囊肿分类、术语、鉴别诊断及临床病理特征的再评价

关于肝囊肿的文献高度矛盾,主要是由于定义不同。使用更新的标准进行病理学评估的 258 个 ≥1 cm 囊肿可分类为: I. 导管板畸形相关(63%);即胆囊性错构瘤或未指明类型的良性胆管囊肿(35与多囊肝病)。这些以女性为主(F/M=2.4),大(10 cm),通常是多灶性的,伴有退行性/炎症性变化,经常被误分类为“肝胆囊腺瘤”。二、肿瘤(13%);27 (10.5%) 例有卵巢型间质 (OTS),符合世界卫生组织 (WHO) 的标准为粘液性囊性肿瘤 (MCN)。这些是女性,孤独,平均年龄 52 岁,平均大小 11 厘米,2 例与癌相关(1 例原位癌和 1 例微创癌)。导管内乳头状肿瘤 3 例,导管内嗜酸细胞乳头状肿瘤 1 例,囊性胆管癌 1 例,囊性转移瘤 2 例。三、感染性/炎症性(12%)。其中包括 23 个包虫囊肿(包括 2 个棘球绦虫均在术前误诊为癌症)、非特异性炎性囊肿(脓肿、炎性囊肿:3.4%)。四。先天性(7%)。大多较小(<3 厘米);胆总管囊肿(5%)、前肠囊肿(2%)。五、杂项(4%)。总之,肝囊肿主要发生在女性中(3/1),大多数(90%)是非肿瘤性的,很少(<2%)是恶性的。胆囊性胆管错构瘤及其相关的未指定类型的良性胆管囊肿通常是多灶性的,并且经常被误诊为“囊腺瘤/癌”。根据 OTS 的定义,MCN(真正的“肝胆囊腺瘤/癌”)是孤立的,仅占肝囊肿的 10.5%,与文献中的印象显着不同,因为基本上都是围绝经期的女性,很少与癌(7%)。

更新日期:2022-08-17
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