脾脏淋巴管瘤的临床病理观察

目的探讨脾脏淋巴管瘤的临床病理特征和鉴别诊断特点。方法结合文献回顾,对18例脾脏淋巴管瘤进行临床资料分析、病理形态学观察及免疫表型检测。结果18例淋巴管瘤患者年龄9～72岁,中位年龄40岁;男13例,女5例;可无明显临床症状,也可表现以腹痛为主的消化道症状或脾功能亢进的相关症状。18例患者随访率72．2％,随访时间从5个月到15年不等,患者均无瘤生存,无复发及转移。脾脏多有肿大,病灶大体形态多样,包括囊性（8例）、实性（5例）和蜂窝状（5例）,可单发（5例）或多发（13例）;组织形态上分为海绵状淋巴管瘤（9例）、囊状淋巴管瘤（5例）及混合型（4例）。16例（100％）均表达CD9;7例（43．8％）表达D2-40。结论发生在脾脏的淋巴管瘤非常少见,应注意与脾脏血管瘤等鉴别。

Splenic lymphangioma: clinicopathologic and immunohistochemical study of 18 cases and review of literature

OBJECTIVE: To study the clinicopathologic features and differential diagnosis of splenic lymphangioma. METHODS: Eighteen cases of splenic lymphangioma were retrieved from the pathology archives during the period between January 1990 to December 2005. The clinicopathologic features were analyzed. Immunohistochemical study was performed on the paraffin sections of 16 cases. RESULTS: The age of the patients ranged from 9 to 72 years (median = 40 years). Thirteen patients were males and 5 were females. Clinically, the tumor could be asymptomatic or present with abdominal symptoms and hypersplenism. Follow-up information was available in 13 patients (72.2%) and the duration varied from 5 months to 15 years. All 13 patients had an uneventful clinical course, with no evidence of residual disease, local recurrence or metastasis. Gross examination showed splenic enlargement. The tumor appeared as cystic (8/18), solid (5/18) or honeycomb mass (5/18), either solitary (5/18) or multifocal (13/18). Histologically, splenic lymphangioma could be subclassified as cavernous (9/18), cystic (5/18) or mixed (4/18). Immunohistochemical study showed that the positivity rates for CD9 and D2-40 were 100% and 43.8% respectively. CONCLUSIONS: Splenic lymphangioma is a rarely encountered entity that can be misdiagnosed as a splenic hemangioma. A definite diagnosis depends on pathologic examination.