Owens Christopher L, Sharma Rajni, Ali Syed Z
Department of Pathology, The Johns Hopkins Hospital, Baltimore, MD 21287, USA.
Cancer. 2007 Jun 25;111(3):166-72. doi: 10.1002/cncr.22689.
Deep fibromatosis or desmoid tumor (DF/DT) is a low-grade, soft tissue lesion that is notable for its infiltration and local recurrence and its inability to metastasize. Although the histologic features of DF/DT are well described, there is a paucity of literature regarding cytologic findings.
The surgical pathology files of The Johns Hopkins Hospital revealed 17 patients with a diagnosis of DF/DT with prior cytology in a 16-year period (1989-2005). The clinicoradiologic findings were noted, and the fine-needle aspiration (FNA) slides were available for review in 8 patients. In patients with archived tissue, an immunohistochemical panel was performed that included beta-catenin, desmin, CD-34, and c-kit.
There was a wide age range and a wide range of anatomic distribution for DF/DT in this series. Eleven patients (65%) had a prior history of surgery at or near the site of DF/DT. Radiologically, 5 of 11 patients (45%) who had in-house studies available and no history of DF/DT were diagnosed as suspicious for malignancy. Predominantly bland spindled cells with long, fusiform nuclei and metachromatic matrix material were present in most tumors. The tumor cells were present both singly and as fragments embedded in the matrix. Nine patients had paraffin-embedded tissue samples available for immunohistochemical staining. Six of those samples demonstrated nuclear beta-catenin reactivity, and all 9 samples were negative for desmin, CD-34, and c-kit.
The current results indicated that clinical history in patients with suspected DF/DT is important. Because of the infiltrative nature of DF/DT, the radiographic impression often is over-called as suspicious for malignancy. The cytomorphology is nonspecific, often resulting in descriptive diagnoses. Immunohistochemical stains increase the yield of FNA.
深部纤维瘤病或韧带样瘤(DF/DT)是一种低度恶性的软组织病变,以浸润性生长、局部复发且无转移能力为显著特点。尽管DF/DT的组织学特征已有详尽描述,但关于其细胞学表现的文献却较为匮乏。
约翰霍普金斯医院的外科病理档案显示,在16年期间(1989 - 2005年)有17例诊断为DF/DT且之前有过细胞学检查的患者。记录临床放射学检查结果,8例患者的细针穿刺抽吸(FNA)玻片可供复查。对于有存档组织的患者,进行了免疫组织化学检测,检测项目包括β-连环蛋白、结蛋白、CD - 34和c - 试剂盒。
本系列中DF/DT患者的年龄范围和解剖分布范围均较广。11例患者(65%)在DF/DT部位或其附近有手术史。放射学检查方面,11例进行了院内检查且无DF/DT病史的患者中有5例(45%)被诊断为可疑恶性肿瘤。大多数肿瘤主要由形态温和的梭形细胞组成,细胞核长且呈梭形,基质材料呈异染性。肿瘤细胞既可见单个存在,也可见呈碎片状嵌入基质中。9例患者有石蜡包埋的组织样本可用于免疫组织化学染色。其中6个样本显示β-连环蛋白核反应阳性,所有9个样本的结蛋白、CD - 34和c - 试剂盒检测均为阴性。
目前的结果表明,疑似DF/DT患者的临床病史很重要。由于DF/DT具有浸润性,影像学表现常被过度解读为可疑恶性肿瘤。细胞形态学表现不具有特异性,常导致描述性诊断。免疫组织化学染色可提高FNA的诊断率。