1J Med Adv Clin Case Rephttps://www.jmaccr.com

1 INTRODUCTION

Paragangliomas and pheochromocytomas are rare neuroendo-crine tumors with an estimated occurrence of 2–5 patients/mil-lion/year [1]. Therefore, primary paraganglioma of the urinary bladder is also a rare disease, accounting for 0.06% of all cases of bladder tumors [2]. Paraganglioma may be fatal because of hypertensive crisis during surgery. Clinical syndromes related to paraganglioma are severe and include angina, brain stroke, vascular shock, acute renal failure, pulmonary edema, and in-testinal ischemia. If paraganglioma is present, blood pressure should be lowered using adrenergic receptor blockers, and the treatment strategy should be switched for safe and complete resection. For the bladder, transurethral resection could be switched to partial cystectomy. Clinicians should be aware of the disease and suspect it in relevant cases.

2 CASE REPORT

A 44-year-old man visited our hospital because of drainage and swelling at the base of the penis following penile enlarge-ment surgery at another hospital. Magnetic resonance imaging showed a bladder tumor in the posterior wall (Figure 1a). Cys-toscopy revealed a solitary, 15-mm-sized, multinodular (ginger

like), pedunculated tumor, which retained the blood vessels of the submucosa (Figure 1b). He had no history of hypertension. The blood pressure was 114/78 mmHg, and the heart rate was 81 beats/min. He had no family history of hypertension or en-docrine disorders. After admission, transurethral resection of the bladder tumor was performed. No abnormalities other than the tumor were observed, and blood pressure was stable during observation by a cystoscope. As the tumor was excised by en-ergizing the resection loop of the cystoscope, a sharp increase in systolic blood pressure up to 200 mmHg with a heart rate of 105 beats/min was observed. Immediately, antihypertensive treatment by an anesthesiologist using an adrenergic receptor blocker was required. With careful attention to the blood pres-sure, tumor excision and hemostasis were completed. Histo-pathological examination revealed that the tumor was a para-ganglioma with positive immunostaining for chromogranin A and synaptophysin.Postoperatively, endocrine tests of blood and urine revealed catecholamine products within normal limits (blood: adren-aline, 27 pg/mL; noradrenaline, 294 pg/mL; dopamine, 10 pg/mL; urine: metanephrine, 0.04 mg/day; normetanephrine, 0.04 mg/day). However, after removal of the urethral catheter, headache during urination and hypertension (systolic blood pressure of 140 mmHg or more) were observed. Oral adminis-tration of doxazosin 2 mg/day was required. I-123 metaiodo-benzylguanidine (MIBG) scintigraphy showed no abnormali-

CASE REPORT

Received: 20 August 2020 Accepted: 26 August 2020 Published: 02 September 2020

Hypertensive Crisis During Surgery of Solitary Ginger like Bladder Tumor with Retained Submucosal Blood Vessels

Kaori Endo1 | Daisuke Yamada2* | Motofumi Suzuki2 | Taketo Kawai2 | Yusuke Sato2 | Masaki Nakamura2 | Yuta Yamada2 | Yoshiyuki Akiyama2 | Atsushi Kato1 | Haruki Kume2

1Department of Urology, Saitama Medical Center, Saitama, Japan

2Department of Urology, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan

Journal of Medical & Advanced Clinical Case Reports

CorrespondenceDr. Daisuke YamadaDepartment of UrologyGraduate School of MedicineThe University of Tokyo, JapanE-mail: dyamada-tky@umin.ac.jpTel: +81-3-3815-5411

AbstractIntroduction: Paragangliomas are rare neuroendocrine tumors and may be fatal because of hypertensive crisis during surgery. So, it’s important to notice before surgery.Case presentation: A 44-year-old man who had no history of hypertension re-ceived transurethral resection of a bladder tumor which was solitary, ginger-like (multinodular), pedunculated tumor. As the tumor was excised, a sharp increase in systolic blood pressure up to 200 mmHg was observed. Immediately, anti-hypertensive treatment was required. Partial cystectomy was subsequently per-formed for complete resection. Cystoscopy images with relevant findings were found in 34 cases, including the solitary (94%), ginger like multinodular (61%), pedunculated (32%), and retained submucosal blood vessels (94%) tumor.Conclusions: In cases of a solitary ginger like with retained submucosal blood vessels tumor on cystoscopy, surgeons should consider the possibility of paragan-glioma, which is rare but dangerous, and prepare adrenergic receptor blockers.KEYWORDSParaganglioma, Bladder, Nodular, Hypertension, Case report

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3 DISCUSSION

I-131 or I-123 MIGB scintigraphy or 18F- dihydroxy-phenylalanine positron emission tomography is useful for the preoperative diagnosis [3], but characteristic urinary hyperten-sion symptoms are present only in 41% of all cases [4]. There-fore, it is difficult to suspect paraganglioma in half of them before surgery. In our case, hypertension was observed only after the transurethral resection of the tumor. We searched 648

ties, but residual disease was suspected in the bladder. Partial cystectomy was subsequently performed for complete resec-tion. To determine the excision range, a surgeon punctured the bladder from the serosa side to the mucous membrane. Cystos-copy was used to see if the needle was outside the transurethral resection mark made at the previous operation (Figure 2a and 2b). Pathological examination of the resected specimen con-firmed a residual lesion of paraganglioma, and the resection margin was negative (Figure 2c). Subsequently, hypertension was not observed, even during urination.

1a) 1b)

Figure 1: Preoperative evaluations. a) Magnetic resonance imaging findings (sagittal section, T2 weighted image). Tumor measuring 15 mm on the posterior wall of the urinary bladder (arrowhead). b) Cystoscopy findings. There is a solitary, multinodular (ginger like), pedunculated tumor on the posterior wall of the urinary bladder (arrowhead). Blood vessels of the submucosa are relatively preserved.

2 a)

2 c)

2 b)

Figure 2: Partial resection of the urinary bladder for complete resection. Using cystoscopy during surgery, residual lesions could be reliably excised. a) The transurethral resection mark was identified with a cystoscope. b) A needle was pierced from the serosa side to mark the residual lesion site. c) Pathological examination of the resected specimen confirmed residual lesions (arrowhead). The resection margin was negative. Scale bar is 100 µm.

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reports in PubMed (search words: bladder and paraganglioma) on August 1, 2020. Reports from 1957 to 2020 were relevant. Cystoscopy images with relevant findings were found in 33 cases. Including our case, the multinodular tumor, peduncu-lated tumor, solitary tumor, and retained submucosal vessels were 21 (61%), 11 (32%), 32 (94%), and 32 (94%) cases, re-spectively (Table 1) [5-32].

4 CONCLUSION

In cases of a solitary multinodular (ginger like) tumor with retained blood vessels of the submucosa on cystoscopy, sur-geons should consider the possibility of paraganglioma. Sub-sequently, the indication for antihypertensive agents and need for modification of the surgical procedure should be discussed with the anesthesiologist.

Case Appearance Stem Site Number Submucosa vessels References

1 Nodular Sessile Left Solitary Retain 52 Multinodular Pedunculated Right Solitary Retain 63 Multinodular Sessile Posterior Solitary Retain 74 Nodular Sessile Anterior Solitary Retain 85 Multinodular Pedunculated Left, Posterior Multiple Retain 96 Smooth No Anterior Solitary Retain 107 Multinodular Pedunculated Posterior Solitary Retain 118 Multinodular Pedunculated Left Solitary Retain 129 Nodular Sessile Trigone Solitary Retain 1310 Nodular Sessile Trigone Solitary Retain 1411 Multinodular Sessile Posterior Solitary Retain 1512 Multinodular Sessile Trigone Solitary Retain 1613 Nodular Sessile Trigone Solitary Retain 1714 Multinodular Sessile Posterior Solitary Retain 1815 Multinodular Sessile Trigone Solitary Retain 1916 Multinodular Sessile Trigone Solitary Retain 1917 Nodular Sessile Right Solitary Retain 2018 Multinodular Sessile Anterior Solitary Retain 2119 Nodular Sessile Anterior Solitary Retain 2220 Multinodular Sessile Anterior Solitary Retain 2321 Nodular Sessile Anterior Solitary Retain 2422 Nodular Sessile Right Solitary Retain 2523 Multinodular Pedunculated Anterior Solitary Partially destroyed 2624 Nodular Sessile Anterior Solitary Retain 2625 Smooth No Anterior Solitary Retain 2626 Multinodular Pedunculated Posterior Solitary Retain 2727 Multinodular Pedunculated Anterior Solitary Retain 2828 Multinodular Sessile Posterior Solitary Retain 2829 Multinodular Pedunculated Posterior Solitary Almost destroyed 2830 Multinodular Sessile Right Solitary Retain 2931 Multinodular Pedunculated Left Solitary Retain 3032 Nodular Sessile Posterior Solitary Retain 3133 Multinodular Pedunculated Left, Right Multiple Retain 3234 Multinodular Pedunculated Posterior Solitary Retain Own

Table 1: Cystoscopic findings of urinary bladder paraganglioma.

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