Division of Imaging and Cancer, University Medical Centre Utrecht, Room G.04.2.28, PO Box 85500, 3508 GA, Utrecht, The Netherlands.
Department of Surgery, St. Antonius Hospital, 3435 CM, Nieuwegein, The Netherlands.
World J Surg Oncol. 2022 May 10;20(1):147. doi: 10.1186/s12957-022-02613-2.
Pseudomyxoma peritonei (PMP) is an uncommon clinical condition characterized by the presence of mucinous ascites, mainly induced by perforated appendiceal mucinous neoplasms (AMN). The peritoneal surface of the small bowel is usually spared from disease manifestation due to peristaltic movements. Mucinous tumours can disseminate as PMP on the entire peritoneum, but are rarely intraluminal. For the first time in literature, we report a case of intraluminal PMP involving the ileum.
A 75-year-old male was treated for perforated AMN and disseminated PMP with cytoreductive surgery and hyperthermic intraperitoneal chemotherapy. During follow-up, the patient developed intraperitoneal recurrence together with intraluminal depositions in the ileum, both disease manifestations with identical KRAS and SMAD4 mutations. Hereafter, the patient was treated with palliative care.
This case illustrates the variation in the biological and clinical behaviour of this rare disease. Clinicians should be aware of unusual tumour distribution patterns of PMP, including the presence of mucinous tumour within the small bowel.
假性黏液瘤腹膜病（PMP）是一种罕见的临床病症，其特征为存在黏液性腹水，主要由穿孔性阑尾黏液性肿瘤（AMN）引起。由于蠕动运动，小肠的腹膜表面通常不会出现疾病表现。黏液性肿瘤可能以 PMP 的形式在整个腹膜上扩散，但很少在肠腔内。我们首次在文献中报告了一例累及回肠的肠腔内 PMP。
一名 75 岁男性因穿孔性 AMN 接受了细胞减灭术和腹腔热灌注化疗治疗，随后发生播散性 PMP。在随访过程中，患者出现了腹腔内复发和回肠内的肠腔内沉积，这两种疾病表现均具有相同的 KRAS 和 SMAD4 突变。此后，患者接受姑息治疗。
本病例说明了这种罕见疾病的生物学和临床行为的变化。临床医生应注意 PMP 的不常见肿瘤分布模式，包括小肠内存在黏液性肿瘤。
