Global Neuroscience Initiative Foundation, Los Angeles, CA, USA.
J Hematol Oncol. 2008 May 28;1:3. doi: 10.1186/1756-8722-1-3.
Desmoid fibromatoses are benign, slow growing fibroblastic neoplasms, arising from musculoaponeurotic stromal elements. Desmoids are characterized by local invasion, with a high rate of local recurrence and a tendency to destroy adjacent structures and organs. Desmoid fibromatoses are rare in children, and though they may occur in the head and neck region, are extremely rare in the paranasal sinuses. Here we report a case of extraabdominal desmoid fibromatosis in a seven-year-old boy involving the sphenoid sinus, one of only six published reports of desmoid fibromatosis of the paranasal sinuses. The expansile soft tissue mass eroded the walls of the sphenoid sinus as well as the posterior ethmoid air cells extending cephalad through the base of the skull. We discuss the clinicopathologic features of this lesion, including structural and ultrastructural characteristics, and we review the literature regarding treatment and outcome.
硬纤维瘤病是一种良性、生长缓慢的纤维母细胞瘤，起源于肌-腱膜的基质细胞。硬纤维瘤病的特点是局部侵袭，局部复发率高，并有破坏邻近结构和器官的趋势。硬纤维瘤病在儿童中罕见，虽然可能发生在头颈部，但在鼻窦中极为罕见。我们报告了一例 7 岁男孩发生在蝶窦的腹外型硬纤维瘤病，这是仅有的 6 例报告的鼻窦硬纤维瘤病之一。扩张的软组织肿块侵蚀了蝶窦的壁以及后筛气房，向颅底上方延伸。我们讨论了该病变的临床病理特征，包括结构和超微结构特征，并复习了关于治疗和预后的文献。
