原发于盆腔淋巴结的淋巴管肌瘤病临床病理特征探讨

Clinicopathological features of primary lymphangioleiomyomatosis in pelvic lymph nodes

目的探讨淋巴管肌瘤病的临床病理特点、诊断及治疗方法。方法报道1例原发于盆腔淋巴结的淋巴管肌瘤的临床资料、光镜及免疫组织化学结果,并结合文献讨论。结果组织学显示右髂内和左腹股沟淋巴结内平滑肌样细胞增生并呈束状排列,伴脂肪化生。增生的细胞形态善良,某些具有上皮样细胞特点,含丰富的嗜酸性胞浆,分裂象罕见。免疫组织化学:增生的细胞SMA(弥漫+),HMB-45(灶性+),CK(AE1/AE3)(-);内衬淋巴管内皮细胞D2-40(+),CD34(-)。结论原发盆腔淋巴结的肺外淋巴管肌瘤比较罕见,免疫组织化学对诊断有重要意义。手术切除后一般预后良好。

Objective To investigate the clinical pathologic characteristics ,diagnosis and treatment methods of lymphangioleio‐myomatosis（LAM ） .Methods The clinical data ,light microscopic and immunohistochemical results in 1 case of LAM originated from pelvic lymph nodes were reported and discussed by combining with the related literatures .Results The histology revealed that the right internal iliac and left inguinal lymph nodes had hyperplasia of smooth muscle‐like cells and which arranged in bundles accompanied by fat metaplasia .The hyperplastic cells had good morphology ,some cells had the epithelioid cell feature and contained abundant acidophilic cytoplasm .The karyokinesis figure was rare .The immunohistochemical results showed that the hyperplastic cells were positive for SMA and HMB45 ,but negative for CK（AE1/AE3） ;inner lining lymphatic endothelial cells D2‐40 were posi‐tive ,but CD34 was negative .Conclusion Primary pelvic lymph node LAM is relatively rare .Immunohistochemistry has an impor‐tant significance for its diagnosis .The general prognosis is good after surgical excision .