Zhang Xiaoyang, Bishop Justin A, Yang Chunrui, Yang Yuyan, Cui Jiangtao, Liu Meizhou
Department of Pathology, Second Hospital of Tianjin Medical University, 23 Pingjiang Road, Hexi District, Tianjin, 300211, China.
Department of Pathology, UT Southwestern Medical Center, Dallas, TX, USA.
Head Neck Pathol. 2025 Jul 14;19(1):86. doi: 10.1007/s12105-025-01824-9.
The past two decades have seen the introduction of several new variants of established salivary gland tumors, along with occasional, entirely novel entities. Palisading adenocarcinoma was described in 2023 with a handful of subsequent additional case reports. We encountered several seemingly inherited cases of palisading adenocarcinoma in a familial cohort.
Within the affected family, all available archival surgical pathology and cytopathology material was retrieved and reviewed. Clinical history was obtained through chart review. Whole-exome sequencing was performed on two cases.
Six patients were affected by palisading adenocarcinoma. The patients ranged from 33 to 72 years (mean, 52.3 years) and were all women. Each patient had ≥ 2 documented tumors, and the locations of involvement were: parotid gland (n = 7), floor of mouth (n = 4), submandibular gland (n = 3), neck (n = 2), orbit (n = 1), and maxilla (n = 1). Although not every clinically documented case was available for histologic review, all cases that were evaluated demonstrated the features described for palisading adenocarcinoma, including two cell populations of ducts with polygonal neuroendocrine-like cells arranged as trabeculae with palisading, and strong positivity for CD56 but not other neuroendocrine markers. Whole-exome sequencing did not reveal any recurrent alterations.
We present 18 new cases of palisading adenocarcinoma, including 12 documented pathologically, occurring in women in the same family. This series doubles the published experience of this emerging tumor. While most of the previously published features of palisading adenocarcinoma were confirmed (female predominance, Asian population, sublingual and submandibular location), this series expands what is known about this neoplasm with novel sites (with parotid gland being most common) and apparent inheritability, a very rare characteristic among salivary gland neoplasms. While we were unable to uncover its molecular driver, this potential inheritability is a clue that may allow for future studies to unravel the genetics of palisading adenocarcinoma.
在过去二十年中，出现了几种已确诊的涎腺肿瘤的新变体，以及偶尔出现的全新肿瘤类型。栅栏状腺癌于2023年被描述，随后又有一些病例报告。我们在一个家族队列中遇到了几例看似遗传性的栅栏状腺癌病例。
在受影响的家族中，检索并复查了所有可用的存档手术病理和细胞病理材料。通过查阅病历获取临床病史。对两例患者进行了全外显子组测序。
六名患者患有栅栏状腺癌。患者年龄在33至72岁之间（平均52.3岁），均为女性。每位患者有≥2个记录在案的肿瘤，受累部位包括：腮腺（n = 7）、口腔底部（n = 4）、颌下腺（n = 3）、颈部（n = 2）、眼眶（n = 1）和上颌骨（n = 1）。虽然并非每个临床记录的病例都有组织学检查资料，但所有接受评估的病例均显示出栅栏状腺癌的特征，包括两种细胞群，即导管与多边形神经内分泌样细胞，呈小梁状排列并伴有栅栏状结构，且CD56呈强阳性，但其他神经内分泌标志物呈阴性。全外显子组测序未发现任何复发性改变。
我们报告了18例新的栅栏状腺癌病例，其中12例经病理证实，均发生在同一家族的女性中。该系列病例使这种新兴肿瘤的已发表经验增加了一倍。虽然栅栏状腺癌先前发表的大多数特征得到了证实（女性占主导、亚洲人群、舌下和颌下部位），但该系列病例扩展了对这种肿瘤的认识，包括新的发病部位（腮腺最为常见）和明显的遗传性，这在涎腺肿瘤中是非常罕见的特征。虽然我们未能发现其分子驱动因素，但这种潜在的遗传性是一条线索，可能有助于未来研究揭示栅栏状腺癌的遗传学机制。
