Departments of Pathology, University of Illinois, Chicago 60612, USA.
Arch Pathol Lab Med. 2010 Feb;134(2):276-8. doi: 10.5858/134.2.276.
Abstract Sinus histiocytosis with massive lymphadenopathy (SHML), also called Rosai-Dorfman disease, is a rare entity. Its etiology and pathogenesis are still essentially unclear. The histologic hallmark of this disease is proliferation of distinctive histiocytes within lymph node sinuses and in extranodal sites. Approximately 23% of patients with SHML, documented in the SHML Registry, presented with disease primarily in extranodal sites, and very few cases of SHML (<1%) involving the gastrointestinal system have been described in the literature. We report an unusual case of primary pancreatic SHML with infiltration of the process into peripancreatic, perinephric, and perisplenic adipose tissue, simulating malignancy.
摘要 伴有巨大淋巴结病的窦组织细胞增生症（SHML），又称罗-道氏病，是一种罕见疾病。其病因和发病机制仍基本不清楚。该病的组织学特征是在淋巴结窦内和结外部位增生独特的组织细胞。在 SHML 登记处记录的约 23%的 SHML 患者主要表现为结外疾病，文献中很少有涉及胃肠道的 SHML（<1%）病例。我们报告了一例罕见的原发性胰腺 SHML 病例，其浸润过程累及胰腺周围、肾周和脾周脂肪组织，模拟恶性肿瘤。
