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肾上腺囊状淋巴管瘤样腺瘤样肿瘤:病例报告及文献复习。

Cystic lymphangioma-like adenomatoid tumor of the adrenal gland: Case presentation and review of the literature.

机构信息

Departments of Pathology, IRCCS, "Casa Sollievo della Sofferenza" Hospital, San Giovanni Rotondo, Italy.

出版信息

Adv Anat Pathol. 2009 Nov;16(6):424-32. doi: 10.1097/PAP.0b013e3181bb6c09.

DOI:10.1097/PAP.0b013e3181bb6c09
PMID:19851133
Abstract

Adenomatoid tumors (AT) are usually found in the genital tract of both sexes. They are very rarely located in extragenital sites, and are exceedingly rare in the adrenal. AT of the adrenal gland (AT-AG) are nonfunctioning, usually discovered incidentally and confused on imaging with other more common adrenal neoplasms. The overwhelming majority occur in males. Thirty-four cases have been reported so far, more often presenting grossly as solid tumors, rarely as solid with cystic areas, and 5 cases were almost entirely cystic. At histology they can be either circumscribed or locally infiltrative, and may pose diagnostic difficulties when the pathologist relies on morphology alone or is challenged on frozen section. On light microscopy the diagnosis may be very difficult if the tumor is rich in vacuolated cells, mimicking metastatic signet ring-cell adenocarcinoma. Immunophenotyping and/or electron microscopy are paramount in helping to ascertain their mesothelial lineage. Lymphangioma is the main histologic mimic of solid-cystic and cystic AT-AG, but lymphangioma is immunopositive for endothelial markers and negative for cytokeratins and mesothelial markers. Ultrastructural analysis has been performed in 10 published cases of AT-AG, in all of which the classical microvilli of coelomic type were always observed. In brief we report herein the sixth case of cystic lymphangioma-like AT, which was incidentally discovered during clinical follow-up in a 39-year-old man undergoing cancer staging and surveillance after surgery. The adrenal tumor was 5.5 cm in size and was fully investigated immunohistochemically and ultrastructurally. A complete review of the literature is also presented.

摘要

腺瘤样肿瘤(AT)通常发生在两性的生殖道。它们非常罕见于生殖系统外部位,在肾上腺中更是罕见。肾上腺腺瘤样肿瘤(AT-AG)为无功能性,通常是偶然发现的,在影像学上与其他更常见的肾上腺肿瘤混淆。绝大多数发生于男性。迄今为止,已有 34 例报道,通常大体上表现为实性肿瘤,很少表现为实性伴囊性区域,5 例几乎完全为囊性。在组织学上,它们可以是局限性的或局部浸润性的,如果病理学家仅依靠形态学或在冷冻切片检查时受到挑战,可能会导致诊断困难。在光镜下,如果肿瘤富含空泡细胞,类似于转移性印戒细胞腺癌,则诊断可能非常困难。免疫表型和/或电子显微镜对于确定其间皮细胞谱系至关重要。淋巴管瘤是实性-囊性和囊性 AT-AG 的主要组织学模拟物,但淋巴管瘤对内皮标志物呈阳性,对细胞角蛋白和间皮标志物呈阴性。已经对 10 例发表的 AT-AG 病例进行了超微结构分析,在所有这些病例中,均观察到经典的体腔型微绒毛。在此,我们报告了第六例囊性淋巴管瘤样 AT,这是在一名 39 岁男性接受癌症分期和手术后随访期间偶然发现的。肾上腺肿瘤大小为 5.5 厘米,进行了全面的免疫组织化学和超微结构研究。还对文献进行了全面回顾。

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