Abstract
A 57-year-old male patient was referred to our department with complaints of his right adrenal gland occupancy and hypertension about 6 months. When admitted to the hospital, the blood pressure was about 160/100 mmHg, and the heart rate was 110 beats/min. He was no obvious obesity, acne, abnormal mood, without weakness of limbs, acral numbness, palpitation and headache. He presented with type 2 diabetes for more than 3 years, with oral administration of metformin enteric coated tablets and subcutaneous injection of insulin glargine to control blood glucose, and satisfied with blood glucose control. Enhanced CT showed that: the right adrenal gland showed a kind of oval isodense, slightly hypodense shadow, the edge was clear, lobular change, the size was about 5.8 cm×5.4 cm, uneven density, there were nodular and strip calcification, round lipid containing area and strip low density area, and the CT value of solid part was about 34 HU. Enhanced scan showed heterogeneous nodular enhancement in the solid part of the right adrenal gland, nodular enhancement could be seen inside. The CT values of solid part in arterial phase, venous phase and delayed phase were 45 HU, 50 HU and 81 HU, respectively. Considering from the right adrenal gland, cortical cancer was more likely. No obvious abnormality was found in his endocrine examination. After adequate preoperative preparation, retroperitoneal laparoscopic adrenalectomy was performed under general anesthesia. During the operation, the 6 cm adrenal tumor was closely related to the inferior vena cava and liver, and after careful separation, the tumor was completely removed and normal adrenal tissue was preserved. The operation lasted 180 min and the blood loss was 100 mL, and the blood pressure was stable during and after the operation. There was no obvious complication. The results of pathological examinations were as follows: the size of the tumor was 7.5 cm×6.0 cm×3.5 cm, soft, with intact capsule and grayish-red cystic in section. Pathological diagnosis: (right adrenal gland) cavernous hemangioma, secondary intravascular thrombosis, old hemorrhagic infarction with calcification and ossification. After 6 months of observation, no obvious complications and tumor recurrence were found. In summary, cavernous hemangioma of adrenal gland is a rare histopathological change. Its essence is a malformed vascular mass. Blood retention is the cause of thrombosis and calcification in malformed vessels. The imaging findings were inhomogeneous enhancement of soft tissue masses, and the adrenal function examination showed no obvious abnormalities. Retroperitoneal laparoscopic surgery is feasible after adequate preoperative preparation. It is difficult to diagnose the disease preoperatively and needs to be confirmed by postoperative pathology.
Keywords: Adrenal gland neoplasms, Cavernous hemangioma, Adrenalectomy
肾上腺海绵状血管瘤(adrenal cavernous hemangioma，ACH)是肾上腺的罕见病种，以往文献报道多为个例[1-2]，ACH的术前诊断和鉴别诊断均较为困难[3]。手术切除是治疗本病的有效方法，然而手术风险随肿瘤直径增大而升高。2020年7月，延安市中医医院泌尿外科收治1例ACH患者，术后组织病理检查明确了ACH的诊断，现结合此患者的临床资料并复习相关文献报道如下，以提高泌尿外科医师对此种罕见病的认识。
1. 病例资料
患者男，57岁，因发现右侧肾上腺占位、血压升高6个月入院，入院时血压160/100 mmHg，心率110次/min。患者无明显肥胖、痤疮、情绪异常，无四肢无力、肢端麻木、心悸、头痛等症状。既往有2型糖尿病病史3年余，一直口服二甲双胍肠溶片及注射甘精胰岛素控制血糖，血糖控制满意。
入院后增强CT检查(图 1)提示：右侧肾上腺见一类椭圆形等密度-稍低密度影，边缘清晰，呈分叶状改变，大小约5.8 cm×5.4 cm，密度不均匀，其内见结节状和条形钙化灶、类圆形含脂质区及条形低密度区，实性部分CT值约34 HU，增强扫描显示，右侧肾上腺区实性部分不均匀渐进性结节状强化，内部可见结节样强化，动脉期、静脉期、延迟期实性部分的CT值分别为45 HU、50 HU、81 HU，考虑来源于右侧肾上腺，皮质癌可能性大。
图 1.
肾上腺海绵状血管瘤CT表现
CT findings of adrenal cavernous hemangioma
A, plain scan of the right adrenal gland showed a kind of elliptic isopensity and slightly lower density shadow with clear edge and lobulated change; B, enhanced arterial level: uneven progressive nodular enhancement of the right adrenal mass, with no enhancement in the low-density area; C, delayed phase level: right adrenal mass still had enhancement; D, coronal scanning: a large, well-defined adrenal mass with calcification in the right adrenal gland.
患者各项内分泌激素检查均未见明显异常，考虑无功能肾上腺肿瘤的可能性大，但患者血压高、心率快，故术前给予口服盐酸酚苄明及琥珀酸美托洛尔缓释片准备4周，将血压控制在110~120/60~80 mmHg，患者出现轻度鼻塞症状，体格检查甲床红润。
充分准备后，予患者全身麻醉，行后腹腔镜下右侧肾上腺肿瘤切除术。手术经后腹腔建立操作空间，术中见肿瘤直径约6 cm，边界清晰，与下腔静脉关系密切，仔细分离肿瘤并保护下腔静脉，充分游离肿瘤后将肿瘤完整切除，保留正常肾上腺组织。手术时间180 min，出血量100 mL，手术过程中及术后患者血压平稳，术后无明显并发症。大体标本：肿瘤为灰红色，大小7.5 cm×6.0 cm×3.5 cm，包膜完整，临床已剖开，呈囊性，切面灰红色，质软(图 2)。组织病理检查见广泛的血管腔团，管腔内充满血液，内皮细胞单层排列，可见凝固性坏死和出血区域。病理诊断：(右侧肾上腺)海绵状血管瘤，继发血管腔内血栓形成、陈旧性出血梗死伴钙化及骨化(图 3)。
图 2.
右侧肾上腺肿物大体标本
The right adrenal mass specimen was completely resected
2A, the tumor size was 7.5 cm×6.0 cm×3.5 cm, and the capsule was complete; 2B, the profile of the mass was cystic, grayish-red and soft; 3, adrenal tumor with large, thin-walled, cystic dilated vessels, followed by intravascular thrombosis, old hemorrhagic infarction with calcification and ossification.
图 3.
右侧肾上腺肿物组织病理检查(HE ×10)
Histopathological examination of the right adrenal mass (HE ×10)
2A, the tumor size was 7.5 cm×6.0 cm×3.5 cm, and the capsule was complete; 2B, the profile of the mass was cystic, grayish-red and soft; 3, adrenal tumor with large, thin-walled, cystic dilated vessels, followed by intravascular thrombosis, old hemorrhagic infarction with calcification and ossification.
患者术后随访6个月，一直未服用降压药物，血压为120~135/80~85 mmHg，无明显不适症状，复查无肿瘤复发。
2. 讨论
海绵状血管瘤是常见于皮肤和肝脏的病变，发生于肾上腺的极为罕见。海绵状血管瘤组织病理学改变的实质是畸形血管团，血液滞留是畸形血管内形成血栓和钙化的原因。ACH最早报道于1955年[4]，由于此病发病率极低且缺乏特异性症状，因此术前的鉴别诊断具有相当大的挑战性，大多数ACH是由手术切除后的组织病理学检查才得以确诊[1]。ACH早期不容易被发现，有部分病例在体检时偶然被检出。随着影像学检查技术(CT、MRI和超声)在临床中的广泛使用，ACH的检出率逐渐提高。整体来说，虽然所有肾上腺肿瘤在腹部CT检查中被偶然发现的病例约占1%~5%[5-6]，但ACH仍然罕见。
ACH常发生于单侧，老年人中比例较高，男女比例约为2 : 3，肿瘤直径2~25 cm，其中大多数直径>5 cm[2]。ACH患者多数没有明显的症状和体征，但是当肿瘤增大至一定程度时可对周围器官和组织产生压迫症状，如同侧腰部胀痛、消化系统症状和可被触及的腹部包块，也有自发性腹膜后大出血、伴发肾上腺恶性肿瘤、血栓形成引起症状的报道[5]。
ACH在影像学检查上没有典型特征，CT扫描可见肿瘤边缘结节、斑片状强化及向中心扩展的强化灶，提示可能存在海绵状血管瘤，平扫肿瘤内可有钙化，原因可能与较多的静脉石位于扩张的血管空间内有关。本例患者于增强CT可见右侧肾上腺一类椭圆形等密度-稍低密度影，边缘清晰，呈分叶状改变，密度不均匀，其内见结节状和条形钙化灶、类圆形含脂质区及条形低密度区，实性部分CT值约34 HU，增强扫描右侧肾上腺区实性部分不均匀渐进性结节状强化，低密度区部分不强化。然而，上述影像学特点在其他肾上腺疾病(如嗜铬细胞瘤、肾上腺腺瘤)中也可以表现出来，因此并不能根据这些特点确诊为海绵状血管瘤。MRI在诊断ACH时有显著优势，表现为在T1WI以低密度信号影为主，在T2WI以高密度信号影为主。嗜铬细胞瘤、皮质腺瘤在T1WI和T2WI均为环形低密度信号影[1]，因此MRI在诊断ACH及其他肾上腺疾病时具有较好的鉴别意义。
手术是治疗肾上腺肿瘤的有效方法，一般认为，肿瘤直径>6 cm的肾上腺偶发瘤需切除，因其发生肾上腺癌及自发性肿瘤破裂的风险高。当考虑到伴发肾上腺恶性肿瘤、出现邻近器官的压迫症状、出血坏死和血栓形成等情况时，对于>3 cm的肿瘤也可手术治疗[5]。ACH属于良性肿瘤，直径较小的无症状者可采取定期随访复查的方法。目前为止，文献报道的大多数ACH瘤患者(肿瘤>3 cm)均接受了手术治疗。肿瘤直径 < 6 cm的ACH可采用腹腔镜手术切除，较大的肿瘤，恶性的可能性也随之升高，同时手术风险也增加，最佳的手术方案可选择经腹正中、经腰或胸腹联合切口的方式行开放手术。有研究报道，腹腔镜手术切除的ACH最大直径为12 cm，且腹腔镜肾上腺切除术在技术上是安全可行的[6]。本例肿瘤直径为6 cm左右，采用了后腹腔镜手术治疗，术中仔细游离下腔静脉，避免其损伤，完整切除肿物的同时保留了正常肾上腺组织，手术效果满意。
本例ACH的组织病理学检查提示为肾上腺罕见组织学类型的肿瘤，综合实验室检查、病理结果可除外原发于肾上腺皮质及髓质的恶性肿瘤，也可除外肾上腺神经鞘瘤、髓脂肪瘤、肾上腺囊肿等良性病变。患者术后随访6个月，无复发。
综上，ACH极为少见，组织病理学改变的实质是畸形血管团，血液滞留是畸形血管内形成血栓和钙化的原因，手术切除为治疗ACH的主要方法，术前虽然不易诊断和鉴别诊断，但MRI的影像学特征有一定帮助，最终确诊仍需术后的组织病理检查。
Contributor Information
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