Shi Guan-Jun, Wang Chong, Zhang Pu, Lu Yi-Yan, Zhou Hai-Peng, Ma Rui-Qing, An Lu-Biao
Department of Myxoma, Aerospace Center Hospital, Beijing 100049, China.
Department of Pathology, Aerospace Center Hospital, Beijing 100049, China.
World J Clin Oncol. 2025 Apr 24;16(4):103564. doi: 10.5306/wjco.v16.i4.103564.
Pseudomyxoma peritonei (PMP) is a distinct form of peritoneal malignancy characterized by diffuse intra-abdominal gelatinous ascites, with an estimated incidence of 1-3 per 1000000. PMP is predominantly secondary to appendiceal mucinous neoplasms, with rarer origins including the ovaries, colon, and urachus. However, PMP originating from small intestine is extremely rare.
A 60-year-old male patient presented with anorexia and abdominal distension. Computed tomography revealed the presence of abdominopelvic effusions and multiple intra-abdominal space-occupying lesions. Ultrasound-guided aspiration indicated that the aspirated tissue was mucinous. Exploratory laparoscopy and tissue biopsy identified diffuse tumor nodules in peritoneum, omentum, pelvic region, intestinal walls, and mesentery. Histopathological analysis of the resected tumors confirmed the presence of mucinous adenocarcinoma, but the primary lesion was difficult to determine. The patient was referred to our center for further treatment and underwent cytoreductive surgery (CRS) combined with hyperthermic intraperitoneal chemotherapy (HIPEC) under general anesthesia. The intraoperative peritoneal cancer index was 30. The surgery lasted 8 hours, with a blood loss of about 600 mL. A complete cytoreduction (CCR0) was achieved. No serious complications occurred after surgery, and the patient's condition was good during the telephone follow-up. Postoperative pathology confirmed the diagnosis of small intestinal mucinous adenocarcinoma at proximal jejunum, which was complicated by high-grade PMP.
PMP originating from small intestine is an exceptionally rare entity that exhibits non-specific clinical features. The preferred treatment is CRS + HIPEC.
腹膜假黏液瘤（PMP）是一种独特的腹膜恶性肿瘤，其特征为腹腔内弥漫性胶冻样腹水，估计发病率为每100万人中有1 - 3例。PMP主要继发于阑尾黏液性肿瘤，少见的起源包括卵巢、结肠和脐尿管。然而，起源于小肠的PMP极为罕见。
一名60岁男性患者出现厌食和腹胀。计算机断层扫描显示存在腹盆腔积液和多个腹腔占位性病变。超声引导下穿刺表明吸出的组织为黏液性。 exploratory laparoscopy（此处可能有误，推测为“腹腔镜探查”）和组织活检发现腹膜、大网膜、盆腔区域、肠壁和肠系膜中有弥漫性肿瘤结节。对切除肿瘤的组织病理学分析证实存在黏液腺癌，但原发灶难以确定。患者被转诊至我们中心接受进一步治疗，并在全身麻醉下接受了减瘤手术（CRS）联合腹腔内热灌注化疗（HIPEC）。术中腹膜癌指数为30。手术持续8小时，失血约600毫升。实现了完全减瘤（CCR0）。术后未发生严重并发症，电话随访期间患者情况良好。术后病理证实为近端空肠小肠黏液腺癌，并发高级别PMP。
起源于小肠的PMP是一种极其罕见的疾病，表现出非特异性临床特征。首选治疗方法是CRS + HIPEC。
