Pawluś Aleksander, Szymańska Kinga, Kaczorowski Krzysztof, Sokołowska-Dąbek Dąbrówka, Olchowy Cyprian, Markiewicz Bartosz D, Zaleska-Dorobisz Urszula
Department of General and Pediatric Radiology, Independent Public Clinical Hospital No. 1, Wrocław, Poland ; Department of Radiology, Wrocław Medical University, Wrocław, Poland.
Department of General and Pediatric Radiology, Independent Public Clinical Hospital No. 1, Wrocław, Poland.
Pol J Radiol. 2015 Mar 28;80:164-7. doi: 10.12659/PJR.893002. eCollection 2015.
The term fibromatosis or desmoid tumor refers to a group of benign fibrous growths without metastatic potential but with a significant risk of local recurrence. These lesions typically present infiltrative growth pattern with local invasion of adjacent tissues. This tendency is the reason for a relatively high rate of local recurrence, even after surgical removal. Fibromatosis is a very rare condition in general population but occurs more frequently in one of the familial cancer predispositions known as familial adenomatous polyposis (FAP) or Gardner syndrome. There are two main groups of fibromatosis: superficial (small, slow-growing lesions) and deep, also known as aggressive fibromatosis (large, rapid-growing lesions).
We report a case of a 6-year-old boy suffering from an aggressive form of fibromatosis. The patient developed a large pathological mass extending from the neck to the loins. After incisional biopsy and histpoathological examination of the sample, a diagnosis of aggressive fibromatosis was established. During the whole diagnostic process, different imaging techniques including CT, MRI and sonoelastography were used. As the surgical treatment was not possible, the patient was finally qualified for chemotherapy.
Eventual diagnosis of aggressive fibromatosis is based on histopathological examination. However, it is an important condition that should be included in differential diagnosis of soft-tissue masses found in diagnostic imaging. Radiologists should be careful especially in defining the margins of infiltration in case of potential surgical treatment.
纤维瘤病或硬纤维瘤这一术语指的是一组无转移潜能但局部复发风险较高的良性纤维性生长物。这些病变通常呈现浸润性生长模式，可局部侵犯相邻组织。这种倾向就是即使手术切除后局部复发率仍相对较高的原因。纤维瘤病在普通人群中非常罕见，但在一种称为家族性腺瘤性息肉病（FAP）或加德纳综合征的家族性癌症易感性疾病中更常发生。纤维瘤病主要有两组：浅表型（小的、生长缓慢的病变）和深部型，也称为侵袭性纤维瘤病（大的、生长迅速的病变）。
我们报告一例患有侵袭性纤维瘤病的6岁男孩。患者出现一个从颈部延伸至腰部的巨大病理性肿块。在对样本进行切开活检和组织病理学检查后，确诊为侵袭性纤维瘤病。在整个诊断过程中，使用了包括CT、MRI和超声弹性成像在内的不同成像技术。由于无法进行手术治疗，患者最终接受化疗。
侵袭性纤维瘤病的最终诊断基于组织病理学检查。然而，这是一种重要的疾病，应纳入诊断成像中发现的软组织肿块的鉴别诊断。在可能进行手术治疗的情况下，放射科医生尤其应注意界定浸润边缘。
