青少年肾上腺血管瘤1例报告及文献复习

Adrenal Hemangioma in an Adolescent: A Case Report and Literature Review

摘要: 目的：报道1例青少年肾上腺血管瘤并探讨其临床特征、诊断思路及治疗策略。方法：回顾性分析1例16岁男性肾上腺血管瘤患者的临床资料，包括临床表现、实验室检查、影像学特征、手术治疗、病理结果及随访情况，并复习相关文献。结果：患者因腹痛就诊时偶然发现右侧肾上腺占位。详细的全身体格检查未见皮肤黏膜血管瘤、血管畸形或四肢发育异常，排除了血管瘤综合征。内分泌功能检查显示皮质醇昼夜节律、醛固酮/肾素活性比值、儿茶酚胺及其代谢产物均正常，证实为无功能性肿瘤。腹部CT平扫显示右肾上腺区55 mm × 51 mm肿块，CT值32 ± 18 HU，增强扫描呈明显不均匀强化(CT值60 ± 30 HU)，缺乏血管瘤典型的“灯泡征”和“向心性充填”强化模式，术前考虑肾上腺偶发瘤，恶性不能排除。行腹腔镜右侧肾上腺切除术，术中见肿瘤血供丰富，与周围组织分界清楚。术后病理证实为肾上腺血管瘤，免疫组化CD34 (+)、ERG (+)、SMA (脉管+)、Ki-67 (约1%+)，其余标记物阴性。术后随访6个月，患者恢复良好，无复发征象。结论：本例为目前文献报道最年轻的肾上腺血管瘤病例，扩展了该病的年龄发病谱。肾上腺血管瘤虽极为罕见，但可发生于任何年龄，临床医师在青少年肾上腺偶发瘤的鉴别诊断中应将其纳入考虑。详细的全身体格检查对排除血管瘤综合征、明确孤立性肾上腺病变至关重要。对于平扫CT值10~40 HU的乏脂性肾上腺肿瘤，当影像学缺乏典型血管瘤征象时，应重点关注CT值不均匀性、边缘结节状强化及延迟期持续强化等特征，结合内分泌功能评估进行综合鉴别。腹腔镜肾上腺切除术是安全有效的首选治疗方式，术中需警惕肿瘤血供丰富的特点。青少年患者需制定个体化长期随访方案，包括影像学监测、内分泌功能评估和生长发育监测，建议至少随访至成年期。

Abstract: Objective: To report a case of adrenal hemangioma in an adolescent and investigate its clinical characteristics, diagnostic approach, and therapeutic strategy. Methods: We retrospectively analyzed the clinical data of a 16-year-old male patient with adrenal hemangioma, including clinical presentation, laboratory examinations, imaging features, surgical treatment, pathological findings, and follow-up outcomes, with a comprehensive literature review. Results: The patient presented with an incidentally discovered right adrenal mass during evaluation for abdominal pain. Comprehensive physical examination revealed no cutaneous or mucosal hemangiomas, vascular malformations, or limb asymmetry, effectively excluding hemangioma syndromes. Endocrine workup confirmed a non-functional tumor with normal cortisol circadian rhythm, aldosterone-to-renin ratio, and catecholamine metabolite levels. Abdominal CT demonstrated a 55 mm × 51 mm right adrenal mass with unenhanced CT attenuation of 32 ± 18 HU and marked heterogeneous enhancement (60 ± 30 HU) on contrast-enhanced imaging, lacking the typical “light bulb sign” and “centripetal fill-in” pattern characteristic of hemangiomas. Preoperatively, the lesion was considered an adrenal incidentaloma with malignancy potential. Laparoscopic right adrenalectomy was performed, revealing a hypervascular tumor with well-defined margins. Histopathological examination confirmed Adrenal hemangioma with immunohistochemical findings of CD34 (+), ERG (+), SMA (vascular+), and Ki-67 (approximately 1%+), while other markers were negative. At 6-month follow-up, the patient recovered well without evidence of recurrence. Conclusion: This case represents the youngest reported adrenal hemangioma patient in the literature, expanding the age spectrum of this disease. Although extremely rare, adrenal hemangioma can occur at any age and should be included in the differential diagnosis of adrenal incidentalomas in adolescents. Thorough physical examination is crucial for excluding hemangioma syndromes and confirming isolated adrenal involvement. For lipid-poor adrenal tumors (unenhanced CT attenuation 10~40 HU) lacking typical hemangioma features, differential diagnosis should focus on CT attenuation heterogeneity, peripheral nodular enhancement pattern, and delayed persistent enhancement, integrated with endocrine functional assessment. Laparoscopic adrenalectomy is the safe and effective treatment of choice, though surgeons must be vigilant regarding the hypervascular nature of these tumors. Adolescent patients require individualized long-term follow-up protocols including imaging surveillance, endocrine function assessment, and growth and development monitoring, with follow-up recommended at least until adulthood.