Xing Yan, Jiang Xuguang, Li Xiang, Li Xuan, Liu Haifeng
Department of Ultrasound, Zaozhuang Municipal Hospital, Zaozhuang, China.
Department of Imaging, Zaozhuang Municipal Hospital, Zaozhuang, China.
Medicine (Baltimore). 2025 Apr 25;104(17):e42298. doi: 10.1097/MD.0000000000042298.
Pseudomyxoma peritonei (PMP) is a rare condition, typically associated with the rupture of an appendiceal mucinous tumor. Due to its rarity and the complexity of its pathophysiology, PMP presents a significant diagnostic challenge.
A 59-year-old woman presented with a 6-month history of abdominal distension, anorexia, and significant weight loss. Her medical history was notable for chronic hepatitis B. She was initially treated for suspected cirrhosis, but her symptoms did not improve and therefore further diagnostic evaluation was needed.
Initial imaging, including liver magnetic resonance imaging (MRI) and abdominal ultrasound, showed irregular liver margins and ascites, consistent with cirrhosis. However, subsequent diagnostic tests, including uterine MRI, positron emission tomography-computed tomography, and abdominal ultrasound, revealed features suggestive of PMP. Immunohistochemistry and histopathological examination of tissue samples confirmed low-grade mucinous adenocarcinoma of gastrointestinal origin, specifically from the appendix.
The patient underwent cytoreductive surgery, and postoperative pathology confirmed mucinous adenocarcinoma originating from the appendix. The patient was also treated with intraperitoneal hyperthermic chemotherapy.
The final diagnosis was PMP.
This case illustrates a rare presentation of PMP in a patient with co-existing hepatitis B, in which the initial diagnosis was biased towards cirrhosis: a more common cause of ascites in hepatitis B patients. This case highlights the importance of considering PMP in the differential diagnosis for patients presenting with unexplained ascites and abdominal distension.
腹膜假黏液瘤(PMP)是一种罕见疾病,通常与阑尾黏液性肿瘤破裂有关。由于其罕见性及其病理生理学的复杂性,PMP带来了重大的诊断挑战。
一名59岁女性,有6个月腹胀、厌食及显著体重减轻的病史。她有慢性乙型肝炎病史。她最初因疑似肝硬化接受治疗,但症状未改善,因此需要进一步的诊断评估。
最初的影像学检查,包括肝脏磁共振成像(MRI)和腹部超声,显示肝脏边缘不规则及腹水,符合肝硬化表现。然而,随后的诊断检查,包括子宫MRI、正电子发射断层扫描-计算机断层扫描和腹部超声,发现了提示PMP的特征。组织样本的免疫组织化学和组织病理学检查证实为胃肠道来源的低级别黏液腺癌,具体来自阑尾。
患者接受了减瘤手术,术后病理证实为起源于阑尾的黏液腺癌。患者还接受了腹腔热灌注化疗。
最终诊断为PMP。
该病例说明了PMP在一名合并乙型肝炎患者中的罕见表现,其中最初的诊断偏向于肝硬化,这是乙型肝炎患者腹水更常见的原因。该病例强调了在对出现不明原因腹水和腹胀的患者进行鉴别诊断时考虑PMP的重要性。