Taher Altaf, Pushpanathan Chitra
Department of Anatomical Pathology, Memorial University of Newfoundland, Health Sciences Centre Laboratory, St John's, Newfoundland and Labrador, Canada.
Arch Pathol Lab Med. 2007 Jul;131(7):1135-8. doi: 10.5858/2007-131-1135-PFTABR.
Plexiform fibrohistiocytic tumor is a rare mesenchymal neoplasm of intermediate malignancy, first reported by Enzinger and Zhang in 1988. It has a predilection for children and young adults but can occur at any age. The tumor usually involves the upper limbs as a slow-growing, painless mass. The tumor has a high local recurrence rate but metastasizes only rarely. Histologically, the tumor is characterized by poorly demarcated dermal or subcutaneous mass with multinodular plexiform growth and fibrohistiocytic cytomorphology. There are three distinct recognized growth patterns: fibrohistiocytic, fibroblastic, and mixed types. The tumor displays uniform immunoreactivity for vimentin and CD68. Ultrastructurally, the tumor cells have features of myofibroblasts and histiocyte-like cells. Complete surgical resection of the tumor, preferably with wider margins, is required to prevent local recurrence. Long-term follow-up is necessary to detect any nodal or pulmonary metastasis.
丛状纤维组织细胞瘤是一种罕见的中间型恶性间叶性肿瘤，由恩津格和张于1988年首次报道。它好发于儿童和年轻人，但可发生于任何年龄。肿瘤通常累及上肢，表现为生长缓慢、无痛性肿块。该肿瘤局部复发率高，但很少发生转移。组织学上，肿瘤的特征是边界不清的真皮或皮下肿块，呈多结节丛状生长，具有纤维组织细胞的细胞形态。有三种不同的公认生长模式：纤维组织细胞型、成纤维细胞型和混合型。肿瘤对波形蛋白和CD68显示一致的免疫反应性。超微结构上，肿瘤细胞具有肌成纤维细胞和组织细胞样细胞的特征。需要完整切除肿瘤，最好切缘更宽，以防止局部复发。需要长期随访以检测任何淋巴结或肺转移。
