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伴有腹膜假黏液瘤的脐尿管黏液腺癌:病例报告

Urachal mucinous adenocarcinoma with pseudomyxoma peritonei: A case report.

作者信息

Liang Lei, Zhou Nan, Xu Hongbin, Liu Damiao, Lu Yiyan, Li Fang, Guo Jun

机构信息

Department of Ultrasound, Aero Space Central Hospital, Beijing, China Department of Surgery, Aero Space Central Hospital, Beijing, China Department of Pathology, Aero Space Central Hospital, Beijing, China.

出版信息

Medicine (Baltimore). 2017 Sep;96(35):e7548. doi: 10.1097/MD.0000000000007548.

Abstract

RATIONALE

Pseudomyxoma peritonei is an unusual clinical condition, and the appendix and ovaries are reported as the primary sites.

PATIENT CONCERNS

A 44-year-old man who was reported a 3-month history of lower abdominal pain and distention, along with increased abdominal girth, was admitted with a palpable tender mass in the central lower abdomen.

DIAGNOSIS

Ultrasonography showed a large well-circumscribed cystic-solid mass with lobulated margin, extending from the anterosuperior dome of the urinary bladder to the anterior abdominal wall. A computed tomography (CT) scan revealed a midline heterogeneous, hypodense, irregular polycystic-solid mass adjacent to the anterior wall of the abdomen and anterior to the dome of the urinary bladder. fluorodeoxyglucose positron-emission tomography/CT showed intense fluorodeoxyglucose uptake in the thickened wall of the mass. Intraperitoneal laparoscopic exploration also revealed a midline abdominal mass adjacent to the dome of the urinary bladder. Laparotomy showed that the mass originated from the dome of the urinary bladder and was disconnected with the urinary bladder lumen. The final histopathological diagnosis was urachal mucinous adenocarcinoma associated with high-grade pseudomyxoma peritonei.

INTERVENTIONS

The patient underwent surgical cytoreductive procedure and the perioperative intraperitoneal chemotherapy.

OUTCOMES

The patient made an uneventful recovery, and 7 months later had no recurrence.

LESSONS

The urachus is a tubular structure, which extends medially from the apex of the bladder to the allantoid during fetal development, and it usually obliterates after birth. Urachal remnants can cause urachal carcinoma or bladder cancers. Pseudomyxoma peritonei originating from mucinous neoplasm of the urachus is extremely rare.

摘要

理论依据

腹膜假黏液瘤是一种罕见的临床病症,据报道阑尾和卵巢是主要发病部位。

患者情况

一名44岁男性,有3个月的下腹部疼痛和腹胀病史,伴有腹围增大,因下腹部中央可触及压痛性肿块入院。

诊断

超声检查显示一个边界清晰的大囊实性肿块,边缘呈分叶状,从膀胱前上穹隆延伸至前腹壁。计算机断层扫描(CT)显示中线处有一个不均匀、低密度、不规则的多囊实性肿块,位于腹前壁附近和膀胱穹隆前方。氟脱氧葡萄糖正电子发射断层扫描/CT显示肿块增厚的壁内有强烈的氟脱氧葡萄糖摄取。腹腔镜腹腔探查也发现膀胱穹隆附近有一个中线腹部肿块。剖腹手术显示肿块起源于膀胱穹隆,与膀胱腔不相连。最终组织病理学诊断为脐尿管黏液腺癌伴高级别腹膜假黏液瘤。

干预措施

患者接受了手术减瘤手术及围手术期腹腔化疗。

结果

患者恢复顺利,7个月后无复发。

经验教训

脐尿管是一个管状结构,在胎儿发育过程中从膀胱顶端向尿囊内侧延伸,出生后通常闭塞。脐尿管残余可导致脐尿管癌或膀胱癌。起源于脐尿管黏液性肿瘤的腹膜假黏液瘤极为罕见。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/93c4/5585475/db5e4db22870/medi-96-e7548-g001.jpg

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