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一例罕见的同时发生的嗜铬细胞瘤伴回盲部神经内分泌肿瘤病例。

A Rare Case of Simultaneous Pheochromocytoma Accompanied with an Ileocecal Neuroendocrine Tumor.

作者信息

Eltawil Karim M, Whalen Carly, Leal Julie N, MacDonald John Kelly, Prowse Owen

机构信息

Department of Surgery, La Verendrye General Hospital, Riverside Health Care Facilities, Fort Frances, Ontario, Canada.

Northern Ontario School of Medicine, Thunder Bay, Ontario, Canada.

出版信息

Case Rep Oncol. 2022 Jan 24;15(1):27-33. doi: 10.1159/000520384. eCollection 2022 Jan-Apr.

Abstract

The detection rates of neuroendocrine tumors (NETs) are rising, which has led to a better understanding of their histopathologic characteristics. We present a case of adrenal pheochromocytoma with an incidental finding of a concurrent ileocecal NET detected on early post-adrenalectomy imaging. A 59-year-old male was noted to have microscopic hematuria on a routine workup. Further imaging identified a right adrenal lesion suggestive of pheochromocytoma. He was reporting intermittent headaches, and his urinary normetanephrine and norepinephrine levels were elevated. He underwent a right retroperitoneal adrenalectomy, and pathology confirmed a pheochromocytoma with no malignancy. Postoperatively, he developed a hematoma at the surgical bed that was identified on CT scan. An incidental lesion at the level of the ileocecal valve was noted on the CT scan as well. This was further investigated by colonoscopy and biopsy that confirmed a carcinoid tumor. He had no gastrointestinal symptoms, chromogranin A was normal, and 24-h urinary 5-hydroxyindolacetic acid levels were slightly elevated. He subsequently underwent a laparoscopic right hemicolectomy 3 months from his initial adrenalectomy. Final pathology confirmed a grade 2 NET with metastatic involvement of 4 regional lymph nodes. We report an extremely rare case of simultaneous pheochromocytoma accompanied with an ileocecal NET. Genetic testing for multiple endocrine neoplasia was undertaken and was found negative for 9 tested genes.

摘要

神经内分泌肿瘤(NETs)的检出率正在上升,这使得人们对其组织病理学特征有了更好的了解。我们报告一例肾上腺嗜铬细胞瘤病例,在肾上腺切除术后早期影像学检查中意外发现同时存在回盲部NET。一名59岁男性在常规检查中发现镜下血尿。进一步的影像学检查发现右肾上腺病变,提示嗜铬细胞瘤。他自述有间歇性头痛,尿去甲变肾上腺素和去甲肾上腺素水平升高。他接受了右腹膜后肾上腺切除术,病理证实为嗜铬细胞瘤,无恶性病变。术后,他在手术床处出现血肿,CT扫描发现了这一情况。CT扫描还发现回盲瓣水平有一个意外病变。通过结肠镜检查和活检进一步检查,证实为类癌肿瘤。他没有胃肠道症状,嗜铬粒蛋白A正常,24小时尿5-羟吲哚乙酸水平略有升高。他在初次肾上腺切除术后3个月接受了腹腔镜右半结肠切除术。最终病理证实为2级NET,有4个区域淋巴结转移。我们报告了一例极为罕见的同时发生嗜铬细胞瘤和回盲部NET的病例。对多发性内分泌肿瘤进行了基因检测,发现9个检测基因均为阴性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/70b4/8832201/6824640c3b89/cro-0015-0027-g01.jpg

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