Hirsch M S, Dal Cin P, Fletcher C D M
Department of Pathology, Brigham and Women's Hospital and Harvard Medical School, Boston, MA 02115, USA.
Histopathology. 2006 Apr;48(5):569-78. doi: 10.1111/j.1365-2559.2006.02376.x.
Pseudosarcomatous myofibroblastic proliferation of the genitourinary tract is rare and may develop after trauma or spontaneously. The aim of this study was to characterize further the clinicopathological features of these lesions and to examine their relationship to inflammatory myofibroblastic tumour (IMT).
Twenty-seven cases of pseudosarcomatous myofibroblastic proliferation were analysed. There were seven males and 20 females; median age was 37 years (range 16-88). Most lesions were from the bladder (n = 21), while others were in the urethra, vulva, vagina, rectum and retrovesical space. Median tumour size was 30 mm (range 6-120 mm). Seven cases (25%) had a history of prior trauma or surgery. Three cases recurred locally but not destructively. The tumours had fasciitis-like features including bland spindle cells with evenly distributed chromatin, admixed inflammatory cells (mainly lymphocytes) and often a myxoid stroma. Immunohistochemistry showed positivity for smooth muscle actin in 14/20 cases, keratin in 8/19, desmin in 7/20 and anaplastic lymphoma kinase (ALK) in 10/21 cases. Fluorescent in situ hybridization was performed in six ALK+ cases; all were negative for ALK gene rearrangement.
Pseudosarcomatous myofibroblastic proliferations of the genitourinary tract may show ALK immunopositivity but do not show consistent ALK rearrangement. Given subtle morphological differences and more consistently benign behaviour, their relationship to inflammatory myofibroblastic tumour at other sites remains uncertain.
泌尿生殖道假肉瘤性肌成纤维细胞增生罕见,可在创伤后或自发发生。本研究的目的是进一步描述这些病变的临床病理特征,并探讨它们与炎性肌成纤维细胞瘤(IMT)的关系。
分析了27例假肉瘤性肌成纤维细胞增生病例。男性7例,女性20例;中位年龄37岁(范围16 - 88岁)。大多数病变来自膀胱(n = 21),其他病变位于尿道、外阴、阴道、直肠和膀胱后间隙。肿瘤中位大小为30 mm(范围6 - 120 mm)。7例(25%)有既往创伤或手术史。3例局部复发但无破坏性生长。肿瘤具有筋膜炎样特征,包括染色质均匀分布的温和梭形细胞、混合的炎性细胞(主要是淋巴细胞)以及常有的黏液样间质。免疫组化显示,20例中有14例平滑肌肌动蛋白阳性,19例中有8例角蛋白阳性,20例中有7例结蛋白阳性,21例中有10例间变性淋巴瘤激酶(ALK)阳性。对6例ALK阳性病例进行了荧光原位杂交;所有病例ALK基因重排均为阴性。
泌尿生殖道假肉瘤性肌成纤维细胞增生可能显示ALK免疫阳性,但未显示一致的ALK重排。鉴于其细微的形态学差异和更一致的良性行为,它们与其他部位炎性肌成纤维细胞瘤的关系仍不确定。