Fetsch John F, Vinh Tuyethoa N, Remotti Fabrizio, Walker Eric A, Murphey Mark D, Sweet Donald E
Department of Soft Tissue, Armed Forces Institute of Pathology, Washington, DC 20306-6000, USA.
Am J Surg Pathol. 2003 Sep;27(9):1260-8. doi: 10.1097/00000478-200309000-00010.
Tenosynovial chondromatosis is a multinodular cartilaginous proliferation that arises from the tenosynovial membranes. This report describes the clinical, radiologic, and histopathologic findings in 37 cases of this uncommon entity. There were 17 males and 20 females, ranging in age from 20 to 86 years (mean and median age, 46 years). The process involved tenosynovium of the fingers (n = 19), feet (n = 8), wrists (n = 4), ankles (n = 2), hand, not otherwise specified, or palm (n = 2), knee (n = 1), and forearm (n = 1). Signs of disease or symptoms were present for 5 weeks to 18 years (median duration, approximately 2 years) before surgical excision. The two most common complaints were a painless mass and a mass that was mildly tender with pressure. None of the tumors had clinical, radiologic, or histopathologic evidence of articular or bone involvement. Histologically, all tumors consisted of a multinodular cartilaginous proliferation involving tenosynovium and/or subsynovial connective tissue. Mild or moderate atypia, as encountered in chondroma of soft parts and synovial chondromatosis, was a frequent finding. Follow-up information was available for 16 patients (43%). Only two patients with follow-up information remained disease free after their initial surgical procedure. Seven patients had one recurrence and seven patients had two or more recurrences. Tenosynovial chondromatosis appears to be an extraarticular counterpart of synovial (intraarticular) chondromatosis. Our review indicates this process is often confused with chondroma of soft parts, in part, because both entities have a predilection for the hands and feet. Diagnosis of this underrecognized entity is of clinical importance because of the high local recurrence rate.
腱鞘软骨瘤病是一种起源于腱鞘膜的多结节性软骨增生。本报告描述了37例这种罕见疾病的临床、放射学和组织病理学表现。患者中男性17例,女性20例,年龄在20至86岁之间(平均年龄和中位数年龄为46岁)。病变累及手指腱鞘(19例)、足部腱鞘(8例)、腕部腱鞘(4例)、踝部腱鞘(2例)、未明确部位的手部或手掌腱鞘(2例)、膝关节腱鞘(1例)和前臂腱鞘(1例)。在手术切除前,疾病体征或症状出现的时间为5周至18年(中位数病程约为2年)。最常见的两个主诉是无痛性肿块和轻压时有轻度压痛的肿块。所有肿瘤均无关节或骨受累的临床、放射学或组织病理学证据。组织学上,所有肿瘤均由累及腱鞘和/或滑膜下结缔组织的多结节性软骨增生组成。在软组织软骨瘤和滑膜软骨瘤病中常见的轻度或中度异型性是常见表现。16例患者(43%)有随访信息。仅2例有随访信息的患者在初次手术后无疾病复发。7例患者复发1次,7例患者复发2次或更多次。腱鞘软骨瘤病似乎是滑膜(关节内)软骨瘤病的关节外对应物。我们的综述表明,这个过程常与软组织软骨瘤相混淆,部分原因是这两种疾病都好发于手和脚。由于局部复发率高,对这种未被充分认识的疾病进行诊断具有临床重要性。