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一名67岁男性患者中,腹膜后实性假乳头状肿瘤酷似肾上腺恶性肿瘤。

Retroperitoneal Solid Pseudopapillary Tumor Mimicking Adrenal Malignant Tumor in a 67-Year-Old Man.

作者信息

Ishii Takahiro, Terasaka Tomohiro, Nishida Kenji, Wada Jun

机构信息

Department of Nephrology, Rheumatology, Endocrinology and Metabolism, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama, 700-8558, Japan.

Endocrine Center, Okayama University Hospital, Okayama, 700-8558, Japan.

出版信息

JCEM Case Rep. 2023 Aug 14;1(4):luad090. doi: 10.1210/jcemcr/luad090. eCollection 2023 Jul.

Abstract

Solid pseudopapillary tumor (SPT) is a low-grade malignant tumor of the pancreas. SPT typically affects women and can occur in ectopic pancreatic region; however, it also occurs rarely in retroperitoneum. The tumor may be bulky at the time of diagnosis since there is no specific clinical manifestation. Here we present an older male case with retroperitoneal SPT. A 67-year-old man consulted for intermittent fever and lumbago. His basal hormonal profile screened out a functional tumor. Computed tomography (CT) showed a gigantic mass in his left adrenal region. A normal left adrenal gland was not identified, and the tumor's feeding artery was recognized as the left adrenal artery by the contrast-enhanced CT. Adrenal malignant tumor was suspected, and tumor resection was performed. The resected tumor size was 15 × 10 × 9 cm. Histologically, epithelial-like cells with round nuclei and a small amount of eosinophilic cytoplasm proliferated in papillary (around the blood vessels) or uniformly solid form. By immunostaining, tumor cells were vimentin, CD56, cytokeratin AE1/AE3, CD10, β-catenin in the nucleus, cyclin D1, and PgR positive. These findings led to the diagnosis of SPT. Although rare, SPT should be considered as a differential diagnosis in cases of a mass arising from the adrenal region.

摘要

实性假乳头状肿瘤(SPT)是一种胰腺的低度恶性肿瘤。SPT通常好发于女性,可发生于胰腺异位区域;然而,它在腹膜后也很少见。由于没有特异性临床表现,肿瘤在诊断时可能体积较大。在此,我们报告一例老年男性腹膜后SPT病例。一名67岁男性因间歇性发热和腰痛就诊。其基础激素检查筛查出一个功能性肿瘤。计算机断层扫描(CT)显示其左肾上腺区域有一个巨大肿块。未发现正常的左肾上腺,增强CT显示肿瘤的供血动脉为左肾上腺动脉。怀疑为肾上腺恶性肿瘤,遂行肿瘤切除术。切除的肿瘤大小为15×10×9 cm。组织学上,核圆形、胞质少量嗜酸性的上皮样细胞呈乳头状(血管周围)或均匀实性增生。免疫组化显示,肿瘤细胞波形蛋白、CD56、细胞角蛋白AE1/AE3、CD10、细胞核β-连环蛋白、细胞周期蛋白D1和孕激素受体(PgR)呈阳性。这些结果确诊为SPT。尽管罕见,但对于肾上腺区域出现肿块的病例,SPT应作为鉴别诊断之一。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b981/10580579/49065fca25a7/luad090f1.jpg

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