The Case Report

A 44-year-old woman came to us with a history of two paravesical masses, monitored via radiological imaging for 10 years.

The patient's medical history revealed multiple episodes of pulmonary embolism after orthopaedic surgery since the age of 22 years. The patient tested negative for any form of coagulopathy. To note, the patient had developed hypertension at a young age, necessitating polypharmacotherapy, accompanied by additional adrenergic symptoms such as flushing, limb tremors, panic attacks, and palpitations.

The first diagnosis of bladder masses occurred incidentally during a routine abdominal ultrasonography scan performed in the setting of an episode of suspected cystitis. Subsequent cystoscopy revealed two bulges on the left lateral wall, covered by normal bladder mucosa. The patient was followed up with repeated ultrasonography scans; neither clinical nor radiological alterations were noted until the patient reported an episode of gross haematuria in 2022. Subsequent abdominal CT scan and MRI (Figs 1 and S1) revealed a solid mass, 4.5 cm in maximum size, located near the posterior left bladder wall (in contact with but not infiltrating, the cervix) and another mass measuring 2.1 cm near the left lateral bladder wall.

Operative cystoscopy with biopsy was performed, which caused a hypertensive crisis complicated by acute pulmonary oedema, which required intubation and admission into the intensive care unit. The biopsy specimen's final pathology examination confirmed a bladder paraganglioma.

Further investigations during the hospital stay included a thorough cardiological evaluation (echocardiography, coronary CT-angiography), which suggested an atypical Takotsubo syndrome.

Takotsubo syndrome, also known as stress cardiomyopathy or ‘broken heart syndrome’, is a temporary heart condition that often mimics the symptoms of a heart attack. It is typically triggered by severe emotional or physical stress. The syndrome gets its name from the Japanese word ‘takotsubo’, which means ‘octopus pot’, due to the distinctive shape of the left ventricle seen in imaging tests during the condition [1].

Blood tests (serum chromogranin A, metadrenalines, and normetadrenaline) and urine analysis (urinary metadrenaline and normetadrenaline) revealed elevated levels of chromogranin A and normetadrenaline, while metadrenaline levels remained normal. A whole-body CT scan excluded distant metastases. Positron emission tomography (PET)/CT using gallium-tetraazacyclododecane tetraacetic acid (⁶⁸Ga-DOTA)-conjugated somatostatin receptor–targeting peptide and ¹⁸F-dihydroxyphenylalanine (DOPA) revealed hyper-fixation at the level of the masses, along with two small ileal foci and a right breast nodule (Fig. 2). These extravesical localisations were excluded from being metastatic. Genetic evaluation revealed a succinate dehydrogenase B subunit (SDHB) mutation linked to familial paraganglioma (paraganglioma type PGL4) [2].

After a careful multidisciplinary evaluation, given the masses’ size and the patient's age, the patient was counselled for bladder-sparing robot-assisted laparoscopic surgery (summarised in Video S1).

Prior to the operation, the patient first received alpha-blockade, followed by beta-blockade, and underwent aggressive intravenous fluid resuscitation while being treated in the endocrinology department. The surgery began by placing a left ureteric single-J stent in a retrograde fashion during cystoscopy. The robot-assisted laparoscopic phase started with incising the peritoneum at the level of the vesico-uterine pouch. The bigger mass was identified: its pedicle was first isolated, clipped, and divided. Only after controlling the pedicle was the dissection of the mass carried out. Notwithstanding the additional care, during the dissection of the main mass, a hypertensive crisis occurred (270/150 mmHg) with initial pulmonary oedema. The surgical procedure had to be paused, and the robot had to be undocked for ~10 min to allow for intensive care manoeuvres. The patient was positioned in an anti-Trendelenburg position. Beta-blockers and sodium nitroprusside were administered. The initial response was severe hypotension; however, normal blood pressure values were achieved only after the titration of the nitrate. In the case of nitrates, the titration involves starting with a low dose and gradually increasing it based on the patient's response regarding blood pressure, heart rate, and symptoms until the desired effect is achieved.

Once haemodynamic stability was achieved, the robotic part continued with the completion of the excision of the bigger mass, performed by a combination of extra- and transvesical approaches.

The second smaller mass was identified during the transvesical approach: first, the pedicle was secured. Second, the mass was excised with an extravesical approach. During the excision of the smaller mass, no intraoperative anaesthesiological adverse events occurred. The bladder defect was sutured in a double-layer fashion.

The postoperative course was uneventful. A drain and a Foley catheter were kept in situ for 2 and 10 days, respectively; the ureteric stent was removed after 7 days.

The pathological and immunohistochemical examination revealed a well-differentiated tumour, showing the typical cell-nests, synaptophysin, and chromogranin positive, suggesting paraganglioma (Fig. 3). Genetic analysis revealed mutations of SDHB, which are associated with an aggressive clinical presentation of paraganglioma (and pheochromocytoma).

中文翻译：

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来自意大利维罗纳大学的“本月案例”——应对膀胱副神经节瘤的医学和外科挑战：来自临床病例的见解

 病例报告

一名 44 岁的女性来找我们，有 2 个膀胱旁肿块的病史，通过放射成像监测了 10 年。

患者的病史显示，自 22 岁以来，骨科手术后多次发作肺栓塞。患者的任何形式的凝血病检测呈阴性。值得注意的是，患者在年轻时就患上了高血压，需要多药治疗，并伴有额外的肾上腺素能症状，如潮红、肢体震颤、惊恐发作和心悸。

膀胱肿块的首次诊断是在疑似膀胱炎发作的情况下进行的常规腹部超声扫描中偶然发生的。随后的膀胱镜检查显示左侧壁上有两个凸起，被正常的膀胱粘膜覆盖。对患者进行重复超声扫描随访;直到 2022 年患者报告了一次肉眼血尿发作，才发现临床和放射学改变。随后的腹部 CT 扫描和 MRI（图 1 和 S1）显示一个最大尺寸为 4.5 cm 的实性肿块，位于左膀胱后壁附近（与宫颈接触但未浸润），另一个肿块在左侧膀胱壁附近测量为 2.1 cm。

进行了手术膀胱镜检查和活检，这引起了高血压危象并发急性肺水肿，需要插管并入住重症监护病房。活检标本的最终病理检查证实了膀胱副神经节瘤。

住院期间的进一步检查包括全面的心脏病学评估（超声心动图、冠状动脉 CT 血管造影），提示非典型章鱼壶综合征。

章鱼壶综合征，也称为压力性心肌病或“心碎综合征”，是一种暂时性的心脏病，通常类似于心脏病发作的症状。它通常是由严重的情绪或身体压力引发的。该综合征得名于日语单词 “takotsubo”，意思是 “章鱼罐”，因为在该病症期间的影像学检查中可以看到左心室的独特形状 [1]。

血液检查（血清嗜铬粒蛋白 A、偏肾上腺素和去甲肾上腺素）和尿液分析（偏肾上腺素和去甲肾上腺素）显示嗜铬粒蛋白 A 和去甲肾上腺素水平升高，而偏肾上腺素水平保持正常。全身 CT 扫描排除了远处转移。使用镓-四氮杂环十二烷四乙酸 （⁶⁸Ga-DOTA） 偶联生长抑素受体靶向肽和 ¹⁸F-二羟基苯丙氨酸 （DOPA） 的正电子发射断层扫描 （PET）/CT 显示肿块水平过度固定，以及两个小回肠病灶和一个右乳结节（图 2）。这些膀胱外定位被排除在转移之外。遗传学评估显示琥珀酸脱氢酶 B 亚基 （SDHB） 突变与家族性副神经节瘤（副神经节瘤 PGL4 型）有关[2]。

经过仔细的多学科评估，考虑到肿块的大小和患者的年龄，建议患者进行保留膀胱的机器人辅助腹腔镜手术（在视频 S1 中总结）。

手术前，患者首先接受 α 受体阻滞剂，然后接受 β 受体阻滞剂，并在内分泌科接受治疗期间接受积极的静脉液体复苏。手术开始时在膀胱镜检查期间以逆行方式放置左输尿管单 J 支架。机器人辅助腹腔镜阶段从在膀胱子宫袋水平切开腹膜开始。确定了更大的肿块：首先分离、剪断和分裂它的椎弓根。只有在控制了椎弓根后，才进行了肿块的解剖。尽管进行了额外的护理，但在解剖主要肿块期间，发生了高血压危象 （270/150 mmHg） 并伴有初始肺水肿。外科手术必须暂停，并且必须将机器人从对接处 ~10 分钟，以便进行重症监护操作。患者处于反特伦德伦伯卧位。给予 β 受体阻滞剂和硝普钠。最初的反应是严重的低血压;然而，只有在硝酸盐滴定后才能达到正常的血压值。在硝酸盐的情况下，滴定包括从低剂量开始，然后根据患者对血压、心率和症状的反应逐渐增加剂量，直到达到预期效果。

一旦达到血流动力学稳定，机器人部分继续完成较大肿块的切除，通过膀胱外和经膀胱入路相结合进行。

在经膀胱入路期间确定了第二个较小的肿块：首先，固定了椎弓根。其次，用膀胱外方法切除肿块。在切除较小肿块期间，未发生术中麻醉不良事件。膀胱缺损以双层方式缝合。

术后过程平淡无奇。引流管和 Foley 导管分别原位保存 2 天和 10 天;输尿管支架在 7 天后被移除。

病理学和免疫组化检查显示分化良好的肿瘤，显示典型的细胞巢、突触素和嗜铬粒蛋白阳性，提示副神经节瘤（图 3）。基因分析揭示了 SDHB 突变，这与副神经节瘤 （和嗜铬细胞瘤） 的侵袭性临床表现有关。