Adrenal Perivascular Epithelioid Cell Tumor A Case Report With Discussion of Differential Diagnoses

Adrenal Perivascular Epithelioid Cell Tumor A Case Report With Discussion of Differential Diagnoses
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DOI:
10.1043/2010-0202-cr.1
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发表时间:
2011-04-01
影响因子:
4.6
通讯作者:
Silverman, Jan F.
Silverman, Jan F.
中科院分区:
医学2区
文献类型:
--
作者:
Zarineh, Alireza;Silverman, Jan F.

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一位50岁男性,因恶心及腹部膨胀感持续数月,经电脑断层扫描评估后,发现肾上腺有一个大肿块,并予以切除。组织病理学检查显示上皮样细胞和梭形细胞具有透明至颗粒状嗜酸性细胞质和小的、中央的、圆形至椭圆形的核,其中有小的核仁。肿瘤细胞表现出对平滑肌肌动蛋白、HMB-45、Melan-A和波形蛋白的反应性,结蛋白和S100蛋白呈局灶性阳性,而突触素、嗜铬粒蛋白呈阴性染色。诊断为血管周围上皮样细胞瘤。血管周围上皮样细胞肿瘤可发生于多种部位,包括网膜/肠系膜、女性生殖道、盆腔软组织、皮肤和镰状韧带,但据我们所知,肾上腺原发性肿瘤以前未见报道。鉴别诊断是广泛的和部位依赖性的,在我们的病例中,包括几个透明细胞的肿瘤,可以涉及肾上腺,包括原发性肾上腺皮质肿瘤。本文回顾相关文献,并讨论其鉴别诊断。(Arch Pathol Lab Med. 2011;135:499-502)
A 50-year-old man presented with nausea and abdominal bloating sensation of a few months' duration and was found to have a large adrenal mass that was resected after computed tomography scan evaluation. The histopathologic examination showed epithelioid and spindle cells having clear to granular eosinophilic cytoplasm, and small, central, round to oval nuclei with small nucleoli. The tumor cells demonstrated reactivity for smooth muscle actin, HMB-45, Melan-A, and vimentin, with focal positivity for desmin and S100 protein and negative staining for inhibin, synaptophysin, and chromogranin. A diagnosis of perivascular epithelioid cell tumor was made. Perivascular epithelioid cell tumors can arise in a variety of locations, including omentum/mesentery, female genital tract, pelvic soft tissues, skin, and falciform ligament, but an adrenal primary tumor has not been previously reported, to the best of our knowledge. The differential diagnosis is broad and site-dependant and in our case includes several neoplasms with clear cells that can involve the adrenal gland, including a primary adrenocortical neoplasm. A review of the related literature and discussion of the differential diagnoses are presented. (Arch Pathol Lab Med. 2011;135:499-502)