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原发于腹股沟皮肤的具有类固醇激素受体阳性的高级别神经内分泌癌:一例极其罕见实体的病例报告。

Primary high-grade neuroendocrine carcinoma with positive steroid hormone receptors arising in the inguinal skin: A case report of An exceedingly rare entity.

机构信息

Department of Pathology, Sichuan Mianyang 404 Hospital, Mianyang, Sichuan Province, China.

Department of Pathology, The People's Hospital of Santai County, Mianyang, Sichuan Province, China.

出版信息

Medicine (Baltimore). 2023 Dec 15;102(50):e36624. doi: 10.1097/MD.0000000000036624.

DOI:10.1097/MD.0000000000036624
PMID:38115331
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC10727585/
Abstract

INTRODUCTION

Neuroendocrine tumors usually arise from the gastrointestinal and pulmonary tracts and rarely from the skin. We report a unique case of high-grade neuroendocrine carcinoma with positive steroid hormone receptors in the primary skin of the groin.

CASE PRESENTATION

A 79-year-old female presented with a lump in her left inguinal region for 15 years that grew gradually. The tumor cells were arranged in sheets, solid nests, and bands within a rich network of thin-walled capillaries. Mucin was abundant in the stroma, and the tumor cells exhibited high-grade lesions, significant necrosis, and frequent mitosis, with small scattered foci of low-grade components. Immunohistochemistry revealed that the tumor cells diffusely and strongly expressed cytokeratin, synaptophysin, chromogranin A, GATA3, CAM5.2, and estrogen and progesterone receptors; partially expressed AR and GCDFP15.

DIAGNOSIS

Based on pathological morphology, and immunohistochemical staining, it was confirmed as Primary high-grade neuroendocrine carcinoma with positive steroid hormone receptors arising in the inguinal skin. The patient underwent resection of the inguinal tumor and left inguinal lymph node dissection.

INTERVENTIONS

The patient has been followed up for 16 months and has not undergone further examinations or received additional treatment. There is no evidence of tumor recurrence at the site of the original surgical resection, and the patient general condition is satisfactory.

CONCLUSIONS

The morphology of this tumor is unique and previously unreported, further expanding the possible pathogenesis and histological morphologies of this tumor type.

摘要

介绍

神经内分泌肿瘤通常起源于胃肠道和肺部,很少起源于皮肤。我们报告了一例罕见的腹股沟皮肤原发的高级别神经内分泌癌,且类固醇激素受体阳性。

病例介绍

一名 79 岁女性因左侧腹股沟区肿块 15 年,逐渐增大就诊。肿瘤细胞排列成片状、实性巢状和条索状,其间有丰富的薄壁毛细血管网。基质中富含黏液,肿瘤细胞表现为高级别病变,显著坏死,频繁有丝分裂,伴有少量低级别成分的散在灶。免疫组化显示肿瘤细胞弥漫且强烈表达细胞角蛋白、突触素、嗜铬粒素 A、GATA3、CAM5.2 和雌激素受体、孕激素受体;部分表达雄激素受体和 GCDFP15。

诊断

根据病理形态学和免疫组化染色,确诊为腹股沟皮肤原发的高级别神经内分泌癌,伴类固醇激素受体阳性。患者接受了腹股沟肿瘤切除术和左侧腹股沟淋巴结清扫术。

干预措施

患者已随访 16 个月,未进一步检查,也未接受额外治疗。原手术切除部位无肿瘤复发迹象,患者一般状况良好。

结论

该肿瘤的形态较为独特,此前未见报道,进一步扩大了此类肿瘤类型的可能发病机制和组织学形态。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/174b/10727585/1f82d335b9e4/medi-102-e36624-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/174b/10727585/cfa58d6f5a1d/medi-102-e36624-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/174b/10727585/8786f14f43c9/medi-102-e36624-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/174b/10727585/1f82d335b9e4/medi-102-e36624-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/174b/10727585/cfa58d6f5a1d/medi-102-e36624-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/174b/10727585/8786f14f43c9/medi-102-e36624-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/174b/10727585/1f82d335b9e4/medi-102-e36624-g003.jpg

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