Chen Chin-Fan, Huang Che-Jen, Kang Wan-Yi, Hsieh Jan-Sing
Department of Surgery, Kaohsiung Medical University Hospital, Kaohsiung, Taiwan.
World J Surg Oncol. 2008 Nov 11;6:118. doi: 10.1186/1477-7819-6-118.
Pseudomyxoma peritonei (PMP) is a rare condition characterized by mucinous tumors, disseminated intra-peritoneal implants, and mucinous ascites. So far its diagnosis remains challenging to most clinicians.
A 55-year-old male patient had suffered from acute onset of abdominal pain and abdominal distension for one day prior to his admission. Physical examination revealed tenderness over the right lower quadrant of the abdomen without diffuse muscle guarding. A large amount of ascites was identified by abdominal computed tomography (CT) scan. Paracentesis showed the appearance of sticky mucinous ascites. He underwent laparotomy under the impression of pseudomyxoma peritonei. There was a lot of mucinous ascites, one appendiceal tumor and multiple peritoneal implants disseminated from the subphrenic space to the recto-vesicle pouch. Pseudomyxoma Peritonei caused by mucinous adenocarcinoma of appendiceal origin, was confirmed by histopathology. We performed an excision of the appendiceal tumor combined with copious irrigation and debridement. After the operation, he received 10 cycles of systemic chemotherapy with FOLFOX4 regimen, without specific morbidity. Follow-up of abdominal CT and colonoscopy at post-operative 17 months showed excellent response without evidence of local recurrence or distal metastasis. He made an uneventful recovery (up to the present) for 21 months after the operation.
This case report emphasizes the possible new role of systemic chemotherapy in the treatment of patients with this rare clinical syndrome.
腹膜假黏液瘤(PMP)是一种罕见疾病,其特征为黏液性肿瘤、腹膜内播散性种植灶及黏液性腹水。迄今为止,对大多数临床医生而言,其诊断仍具有挑战性。
一名55岁男性患者入院前一天出现急性腹痛和腹胀。体格检查发现右下腹压痛,无弥漫性肌紧张。腹部计算机断层扫描(CT)显示大量腹水。腹腔穿刺抽出黏性黏液性腹水。他在腹膜假黏液瘤的诊断下接受了剖腹手术。术中见大量黏液性腹水、一个阑尾肿瘤以及从膈下间隙至直肠膀胱陷凹的多个腹膜种植灶。组织病理学证实为阑尾黏液腺癌所致的腹膜假黏液瘤。我们进行了阑尾肿瘤切除并大量冲洗和清创。术后,他接受了10个周期的FOLFOX4方案全身化疗,无特殊并发症。术后17个月的腹部CT和结肠镜检查随访显示反应良好,无局部复发或远处转移迹象。术后21个月(截至目前)他恢复顺利。
本病例报告强调了全身化疗在治疗这种罕见临床综合征患者中可能的新作用。