罕见肾上腺神经纤维瘤1例并文献复习

doi:10.3969/j.issn.1004-583X.2023.04.013

摘要/Abstract

摘要：

目的探讨肾上腺神经纤维瘤(adrenal neurofibroma, ANF)的临床特征,提高对其的认识及诊治技术。方法分析我院收治的1例病理证实为ANF患者的临床资料、诊治过程及随访结果,并结合已发表的国内外相关文献进行复习总结。结果本例患者术前相关肾上腺激素水平检查均正常,增强CT发现右侧肾上腺占位性病变,考虑良性肿瘤性病变。行后腹腔镜手术完整切除肾上腺肿物及少部分右肾上腺。术后病理示:灰黄灰红组织一块,大小6.5 cm×5.5 cm×4.0 cm;可见包膜,切面呈多结节状,灰白实性质软,局部结节质硬,可见少许肾上腺组织;光镜所见梭形细胞肿瘤及神经纤维末梢,间质黏液样变,未见异常核分裂及细胞异型性;结合免疫组化,符合ANF。术后1个月复查,肾上腺相关激素水平均正常,无复发和转移。结论 ANF为良性肿瘤,临床症状、体征缺乏特异性,术前实验室和影像检查并不能确诊,诊断依赖组织病理学检查。腹腔镜完整切除肿瘤是治疗的关键,腹膜后途径具有更大优势,大多数预后良好,但需密切观察及随访。

关键词: 肾上腺肿瘤, 神经纤维瘤, 腹腔镜, 病理学

Abstract:

Objective To analyze the clinical features of adrenal neurofibroma (ANF) and improve the understanding of its diagnosis and treatment techniques. Methods The clinical data, diagnosis and treatment process, and follow-up results of one pathologically confirmed ANF case admitted to our hospital were analyzed. Combined with published domestic and foreign literature review, case report was summarized. Results In this case, preoperative examination of relevant adrenal hormone levels was normal, and enhanced CT revealed a space occupying lesion in the right adrenal gland, indicating a benign neoplastic lesion. Posterior laparoscopic surgery was performed to remove the adrenal mass and a small portion of the right adrenal gland. Postoperative pathology showed a piece of grayish-yellow grayish-red tissue, with a size of 6.5 cm×5.5 cm×4.0 cm. The capsule was visible, with multiple nodular shapes on the section. The gray white solid was soft, the local nodules were hard, and a little adrenal tissue could be seen. Light microscopy showed spindle cell tumor, nerve fiber endings, and interstitial mucinous degeneration. No abnormal nuclear division or cell atypia was observed. Combined with immunohistochemistry, ANF was obtained. After one month of follow-up after operation, the levels of adrenal related hormones were normal, and there was no recurrence or metastasis. Conclusion ANF is a benign tumor with lack of specificity in clinical symptoms and signs. Preoperative laboratory and imaging examination cannot confirm the diagnosis, and the diagnosis depends on histopathology. Laparoscopic complete tumor resection is the key to treatment, and retroperitoneal approach has more advantages. Most of the cases have good prognosis, but close observation and follow-up are needed.

Key words: adrenal tumor, neurofibroma, peritoneoscopy, pathology

中图分类号:

R699.3

罗杰, 吴强, 李忠, 郑硕. 罕见肾上腺神经纤维瘤1例并文献复习[J]. 临床荟萃, 2023, 38(4): 359-363.

Luo Jie, Wu Qiang, Li Zhong, Zheng Shuo. Rare adrenal neurofibroma: A case report and literature review[J]. Clinical Focus, 2023, 38(4): 359-363.

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链接本文: https://huicui.hebmu.edu.cn/CN/10.3969/j.issn.1004-583X.2023.04.013

https://huicui.hebmu.edu.cn/CN/Y2023/V38/I4/359

图/表 3

图1 增强CT检查 a:动脉期,右侧肾上腺区可见团块影,边界清晰,紧邻下腔静脉,其内可见斑点状致密影,CT值约47 HU;b:门脉期,肿物与右肾上级毗邻,分界较清,CT值约50 HU;c:延迟期,CT值约64 HU

Fig.1 Enhanced CT examination a: In arterial phase, mass shadows were seen in the right adrenal area with clear boundaries, adjacent to the inferior vena cava, and mottled dense shadows were seen inside, with CT value of about 47 HU; b: In the portal vein phase, the mass was adjacent to the superior level of the right kidney with a clear boundary, and the CT value was about 50 HU; c: In the delay period, CT value was about 64 HU

图2 肿瘤剖面及病理图片 a: 手术切除肿物,大小约6.5 cm×5.5 cm×4.0 cm,可见包膜,切面呈多结节状,灰白实性质软,局部结节质硬,可见少许肾上腺组织;b~c:术后病理图片,光镜见梭形细胞及神经纤维末梢,间质黏液样变,未见异常核分裂及细胞异型性(HE染色,×100)

Fig.2 Tumor profile and pathological picture a: The mass that had been surgically removed, with a size of 6.5 cm×5.5 cm×4.0 cm. The capsule was visible, with multiple nodular shapes on the section. The gray white solid was soft, the local nodules were hard, and a little adrenal tissue could be seen; b-c: Postoperative pathological pictures. Light microscopy showed spindle cell tumor, nerve fiber endings, and interstitial mucinous degeneration. No abnormal nuclear division or cell atypia was observed (HE staining, ×100)

表1 肾上腺神经纤维瘤相关病例报道

Tab.1 Case report of adrenal neurofibroma

发表年份	作者	年龄(岁)	性别	生化指标	瘤体位置	瘤体大小(mm×mm×mm)	随访时间(月)
1997	赵军等^[2]	29	男	-	右	30×30×20	-
2000	杨长海等^[3]	51	男	正常	右	50×40×30	24
2001	赵晖等^[4]	26-44	男	正常	右	40×40×30~40×50×60	2~72
			男		右
			女		左
			女		左
2003	詹胜利等^[5]	46	女	高皮质醇,高肾上腺素	右	60×60×35	-
2005	李涛等^[6]	19	男	高去甲肾上腺素、肾上腺素	右	36	84
		35	女		右	56	8
		62	女		右	47	-
2006	赵海东等^[7]	40	女	正常	左	75×60×56	-
2006	崔书平等^[8]	37	女	高肾素	左	80×80×60	6
2007	赵亮等^[9]	64	男	正常	右	60×50×50	4
2007	汪中郎等^[10]	-	-	正常	右	>50	36
		-	-	正常	右	>50	36
2007	Falahatkar等^[11]	24	女	正常	右	46×39	3
2008	曹会彦等^[12]	25	男	正常	左	80×80×60	-
2010	陈金勇等^[13]	16	男	正常	左	40×30×20	24
2011	高江涛等^[14]	37	男	正常	左	160×100×80	3
2012	王慧慧等^[15]	71	女	正常	右	76×65×50	-
2013	余洪等^[16]	59	女	高皮质醇	左	85×50×75	18
2013	李莉等^[17]	31	男	正常	右	40×40×40	-
2015	傅兴宁等^[18]	60	男	高尿香草扁桃酸、肾上腺素	右	120×100×80	-
2015	陈玉峰等^[19]	30	女	高肾素、血管紧张素、低血钾	右	70×60×50	-
2015	Gupta等^[20]	51	女	正常	左	100×80	6
2017	赵闯绩等^[21]	59	女	正常	左	110×70×50	-
2017	刘玉建等^[22]	28	女	正常	右	68×53×70	48
		60	女	高去甲肾上腺素、皮质醇	右	80×50×30	48
2018	王超等^[23]	55	女	正常	左	50×40×30	0.5
2019	温生宝等^[24]	50	女	-	左	85×75×50	-
2021	本研究	21	男	正常	右	65×55×40	1

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