累及肝胰胆系统的纤毛前肠囊肿:临床病理评估,重点关注非典型特征。

IF 4.6 Q2 MATERIALS SCIENCE, BIOMATERIALS ACS Applied Bio Materials Pub Date : 2024-09-19 DOI:10.1136/jcp-2023-208947
Pooja Navale, Jonathan Glickman, Imad Nasser, Jinru Shia, Monika Vyas
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引用次数: 0

摘要

目的:前肠囊肿畸形是一种罕见的发育异常,可能会累及肝胰胆管(HPBT)。这些囊肿由内层纤毛上皮、上皮下结缔组织层、平滑肌层和外层纤维层组成。虽然放射病理检查结果通常具有诊断意义,但不典型的位置和组织学特征会给诊断带来挑战。我们旨在研究 HPBT 中的纤毛前肠囊肿(CFCs),评估其临床病理特征,重点关注非典型特征:我们从三个大型学术医疗中心收集了涉及 HPBT 的 CFCs 病例。我们对每个病例的 H&E 染色切片和免疫组化染色(如有)进行了审查。从病历中收集了相关的人口统计学、临床和病理学信息。中位年龄为 53 岁(3-78 岁)。在肝脏中发现了17个囊肿(第4节段是最常见的位置,10个),在胰腺中发现了4个囊肿。囊肿大多是偶然发现的(13 例),腹痛是常见症状(5 例)。囊肿大小从0.7厘米到17.0厘米不等(中位数为2.5厘米)。17例患者有放射学检查结果。所有病例均能发现纤毛。21 个病例中有 19 个病例显示存在平滑肌层(厚度,结论:我们强调了 HPBT 中 CFC 的临床病理特征。组织形态学通常比较简单;但是,不寻常的位置和不典型的特征会给诊断带来挑战。
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Ciliated foregut cysts involving the hepatopancreaticobiliary system: a clinicopathological evaluation with focus on atypical features.

Aims: Foregut cystic malformations are rare developmental abnormalities, which may involve the hepatopancreaticobiliary tract (HPBT). These cysts are composed of inner ciliated epithelium; subepithelial connective tissue layer; smooth muscle layer; and an outer fibrous layer. While radiopathologic findings are often diagnostic, atypical location and histologic features can pose a diagnostic challenge. We aimed to study ciliated foregut cysts (CFCs) in the HPBT, assess their clinicopathological features with a focus on atypical features.

Methods: We collected cases of CFCs involving the HPBT from three large academic medical centres. H&E-stained slides and immunohistochemical stains (where available) were reviewed for each case. Relevant demographic, clinical and pathological information was collected from the medical records.

Results: 21 cases were identified. The median age was 53 years (range, 3-78 years). 17 cysts were identified within the liver (segment 4 was the most common location, n=10) and 4 in the pancreas. Cysts were mostly identified incidentally (n=13), abdominal pain was a common symptom (n=5). Cyst size ranged from 0.7 to 17.0 cm (median, 2.5 cm). Radiological findings were available in 17 cases. Cilia were identified in all cases. 19 of 21 cases demonstrated the presence of a smooth muscle layer (thickness, <0.1 mm to 3.0 mm). Three cases showed gastric metaplasia, while one case revealed additional low-grade dysplasia, with features similar to intraductal papillary neoplasm of the bile duct.

Conclusions: We highlight clinicopathological features of CFCs in the HPBT. The histomorphology is usually straightforward; however, unusual location and atypical features can pose a diagnostic challenge.

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来源期刊
ACS Applied Bio Materials
ACS Applied Bio Materials Chemistry-Chemistry (all)
CiteScore
9.40
自引率
2.10%
发文量
464
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