Albores-Saavedra Jorge, Keenportz Brent, Bejarano Pablo A, Alexander Alan A Z, Henson Donald E
Instituto Nacional de Ciencias Médicàs Y Nutrición Salvador Zubirán, Mexico City, Mexico.
Am J Surg Pathol. 2007 Oct;31(10):1598-604. doi: 10.1097/PAS.0b013e31804fa10e.
We report 9 examples of segmental adenomyomatous hyperplasia of the gallbladder with perineural invasion. Five patients were women and 4 men. Their ages ranged from 49 to 81 years (mean age 64 y). Eight patients had gallbladder calculi. The original pathologic diagnosis of adenocarcinoma was made in 5 patients and of "adenoma malignum" in one. Six patients are disease-free for 2 to 11 years following cholecystectomy, 1 patient died of unrelated causes and 2 were lost to follow-up. Histologically 2 types of adenomyomatous hyperplasia were recognized. The first one characterized by numerous Rokitansky-Aschoff sinuses (RASs) was accompanied by smooth muscle hyperplasia and an expanded subserosal layer containing numerous nerve trunks (6 cases). The second type was characterized by an extensively fibrotic gallbladder wall with numerous RASs but with few or no smooth muscle bundles and an expanded subserosal layer containing abundant nerve-trunks (3 cases). Perineural (7 cases) and intraneural invasion (2 cases) was identified only in the subserosal layer. The lack of p53 reactivity and the very low MIB-1-labeling index provide additional support to the non-neoplastic nature of the lesion. The pseudoinvasive pattern of the RASs, reactive epithelial atypia, and the perineural and intraneural invasion probably contributed to the erroneous diagnosis of adenocarcinoma or "adenoma malignum." The mechanism by which the epithelial structures "invaded" the perineural spaces and the nerves is unclear. We favor the hypothesis that the migration of the benign glandlike structures into the nerves is related to the production of chemotactic factors or signaling substances and the activation of cell receptors.
我们报告了9例伴有神经侵犯的胆囊节段性腺肌样增生病例。5例为女性，4例为男性。年龄范围为49至81岁（平均年龄64岁）。8例患者有胆囊结石。5例最初病理诊断为腺癌，1例为“恶性腺瘤”。6例患者在胆囊切除术后2至11年无疾病复发，1例患者死于无关原因，2例失访。组织学上识别出2种腺肌样增生类型。第一种以大量罗-阿窦（RASs）为特征，伴有平滑肌增生和浆膜下层扩张，其中含有大量神经干（6例）。第二种类型的特征是胆囊壁广泛纤维化，有大量RASs，但平滑肌束很少或没有，浆膜下层扩张，含有丰富的神经干（3例）。仅在浆膜下层发现神经周围侵犯（7例）和神经内侵犯（2例）。p53无反应性以及极低的MIB-1标记指数为该病变的非肿瘤性质提供了额外支持。RASs的假浸润模式、反应性上皮异型性以及神经周围和神经内侵犯可能导致了腺癌或“恶性腺瘤”的错误诊断。上皮结构“侵入”神经周围间隙和神经的机制尚不清楚。我们支持这样的假说，即良性腺样结构向神经内的迁移与趋化因子或信号物质的产生以及细胞受体的激活有关。
