Ohe Chisato, Sakaida Noriko, Yanagimoto Yasuaki, Toyokawa Hideyoshi, Satoi Sohei, Kwon A-Hon, Tadokoro Chika, Takasu Kosho, Uemura Yoshiko
Department of Surgical Pathology, Kansai Medical University, Hirakata Hospital, 2-3-1 Shinmachi, Hirakata, Osaka 573-1191, Japan.
Med Mol Morphol. 2010 Dec;43(4):235-40. doi: 10.1007/s00795-010-0507-2. Epub 2011 Jan 26.
Primary splenic mucinous cystadenocarcinoma (MCCa) is extremely rare, and only six cases appear to have been reported previously. We present herein a case of primary splenic MCCa resulting in pseudomyxoma peritonei (PMP). A 66-year-old Japanese woman presented to a hospital with a chief complaint of upper abdominal pain and a 7-year history of splenic cyst. Cyst rupture was noted on computed tomography, and splenectomy was performed. The abdominal cavity was filled with a large amount of gelatinous ascites, with the appearance of PMP. On the cut surface, multiple cysts containing mucinous material were found within and outside the spleen. Microscopically, splenic parenchyma was occupied by large mucinous pools focally lined with mucinous epithelial cells and mesothelial cell-like cells, which were considered benign. Outside the spleen, a low-grade MCCa component was found. No ectopic pancreatic or intestinal tissue was identified. Although most PMP cases are known to be caused by low-grade mucinous appendiceal tumor, the present case represents the first report of a splenic MCCa resulting in PMP.
原发性脾脏黏液性囊腺癌（MCCa）极为罕见，此前似乎仅报道过6例。我们在此报告1例导致腹膜假黏液瘤（PMP）的原发性脾脏MCCa病例。一名66岁的日本女性因上腹部疼痛为主诉就诊，有7年脾脏囊肿病史。计算机断层扫描发现囊肿破裂，遂行脾切除术。腹腔内充满大量胶冻样腹水，呈现PMP表现。在切面可见，脾脏内外均发现多个含有黏液物质的囊肿。显微镜下，脾脏实质局部被大的黏液池占据，黏液池内衬黏液上皮细胞和间皮样细胞，这些细胞被认为是良性的。在脾脏外发现了低级别MCCa成分。未发现异位胰腺或肠道组织。尽管已知大多数PMP病例由低级别黏液性阑尾肿瘤引起，但本病例是导致PMP的脾脏MCCa的首例报告。
