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Bladder hamartoma in Peutz-Jeghers syndrome: a rare case report

机译:Peutz-jeghers综合征的膀胱Hamartoma:一个罕见的案例报告

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Background:Peutz-Jeghers syndrome is an autosomal dominant disease characterized by mucocutaneous pigmentation and hamartomatous polyps in the gastrointestinal tract (GIT). There have also been cases of extra GIT polyps such as the renal pelvis, urinary bladder, lungs and nares. Bladder hamartoma is an extremely rare finding, with only 12 cases described in the literature up to now. The rarity of the condition necessitates a comprehensive compilation of managements up to now so as to provide a better tool for the treatment of such conditions in the future.Case presentationA twenty-year-old male, known to have Peutz-Jeghers syndrome, presented to us complaining of obstructive urinary symptoms. A urethrogram done showed a filling defect at the base of the urinary bladder. The mass was resected transurethrally, and histopathology revealed a hamartoma of the bladder. The patient has since remained tumor-free on follow-up.ConclusionsTransurethral resection of the bladder mass proved to be an effective therapy in this patient with no recurrence on the patient’s follow-up till now. There is still, however, a dearth of knowledge regarding the management of bladder hamartomas owing to the extreme rarity of the case.
机译:背景:Peutz-Jeghers综合征是一种常染色体显性疾病,其特征在于粘膜癌症和胃肠道(Git)中的Hamartomatous息肉。还有额外的Git息肉,如肾盂,膀胱,肺和鼻腔。 Bladder Hamartoma是一个非常罕见的发现,只有12例左右描述。该条件的罕见程度需要全面汇编管理层,以便为未来的治疗此类条件提供更好的工具。Case呈现,他已知有Peutz-jeghers综合征,提出美国抱怨阻塞性泌尿症症状。在膀胱的底部显示尿道造影显示出填充缺陷。肿块经过近似切除,组织病理学揭示了膀胱的流氓。此后患者仍然保持肿瘤的随访。膀胱肿块的组织urbral ral rese在该患者中被证明是患者的有效治疗,直到现在患者的后续随访。然而,仍然有关于膀胱浑浊的管理不良,由于案件的极度罕见。

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