Di Martino C, Buonocore C, Marziano E, Treglia F, Papa O, Cristini C
Ospedale di Colleferro ASL Roma G. Divisione di Chirurgia.
Ann Ital Chir. 2002 May-Jun;73(3):331-4.
The authors report a case of a male 86 years old patient with peritoneal pseudomyxoma associated to cystoadenoma of the appendix. Preoperative CT scan showed the presence of a mass in the appendiceal site containing a gelatinous fluid collection and calcifications. Notwithstanding, diagnosis of pseudomyxoma was preoperatively considered less probable due to the rarity of the affection and the advanced age. At laparatomy was revealed the presence of the large mass observed at CT scan, involving the caecum and occupying the retroperitoneal space along the ascending colon. Mucinous ascites was present without invasive mucinous implants in the peritoneum. Operation consisted in a right hemicolectomy with total removal of the retroperitoneal mass and evacuation of the mucinous ascites. Histology confirmed the clinical diagnosis of pseudomyxoma peritonei associated to cystoadenoma of the appendix. The case is of paradigmatic interest as being quite correspondent to the classical Ronnett's definition of pseudomyxoma: a clinicopathological entity characterized by mucinous ascites and non-invasive mucinous implants with a characteristic distribution and containing histologically benign mucinous epithelium derived from an appendiceal mucinous adenoma and having a benign course. According to such definition, the authors stress the importance of an appropriate classification of the observed cases of pseudomyxoma, under whose term is often collected a heterogeneous group of pathological lesions, in order to better evaluate the prognosis and treatment.
作者报告了一例86岁男性患者,患有与阑尾囊腺瘤相关的腹膜假黏液瘤。术前CT扫描显示阑尾部位有一个肿块,内含胶冻样液体聚集及钙化。尽管如此,由于该疾病罕见且患者年龄较大,术前认为假黏液瘤的诊断可能性较小。剖腹探查时发现了CT扫描中观察到的巨大肿块,累及盲肠并沿升结肠占据腹膜后间隙。存在黏液性腹水,但腹膜内无侵袭性黏液种植。手术包括右半结肠切除术,完全切除腹膜后肿块并排出黏液性腹水。组织学证实了与阑尾囊腺瘤相关的腹膜假黏液瘤的临床诊断。该病例具有典型意义,因为它与经典的罗恩内特对假黏液瘤的定义相当吻合:一种临床病理实体,其特征为黏液性腹水和具有特征性分布的非侵袭性黏液种植,包含源自阑尾黏液性腺瘤的组织学良性黏液上皮,病程良性。根据这一定义,作者强调了对观察到的假黏液瘤病例进行适当分类的重要性,在“假黏液瘤”这一术语下通常收集了一组异质性的病理病变,以便更好地评估预后和治疗。