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孤立性纤维性肿瘤:12例发生于软组织、鼻腔和鼻咽部、膀胱及前列腺的病例的临床病理、免疫组化及超微结构分析

Solitary fibrous tumour: clinicopathological, immunohistochemical, and ultrastructural analysis of 12 cases arising in soft tissues, nasal cavity and nasopharynx, urinary bladder and prostate.

作者信息

Mentzel T, Bainbridge T C, Katenkamp D

机构信息

Institute of Pathology, University of Jena, Germany.

出版信息

Virchows Arch. 1997 Jun;430(6):445-53. doi: 10.1007/s004280050054.

Abstract

The clinicopathological features of 12 extraserosal solitary fibrous tumours (SFT) are described. The age of the patients ranged from 18 to 72 years (mean: 48.2 years; median: 54 years); 5 were female patients. Seven lesions arose in soft tissue (5 in perifascial, and 1 each in subcutaneous and intramuscular tissues). They were situated in the groin (2 cases) and the neck, right buttock, left scapula, upper arm, and anterior abdominal wall (1 case each). One polypoid lesion was seen in in the nasal cavity and 1 in the nasopharynx; 2 neoplasms arose in the urinary bladder and 1 was located in the prostate and periprostatic tissue. Nine lesions were excised; in 1 patient wide excision was performed and in 2 patients, transurethral resection. Limited follow-up of 3 cases revealed a benign clinical course. The size of the neoplasms ranged from 1.7 cm to 20.0 cm (mean: 5.4 cm; median: 3.5 cm). Histologically, the neoplasms were well circumscribed and composed of cytologically bland spindle cells arranged without an obvious pattern; focally storiform or fascicular growth patterns were seen. Tumour cells were separated by thick bands of collagen demonstrating foci of keloid-like hyalinization. Prominent vascularity showing a haemangiopericytoma-like vascular pattern and vessels with thick, hyalinized vessel walls were seen in all cases. Increased mitotic activity was noted in 2 soft tissue cases (4-6 mitoses in 10 high-power fields); the other cases showed fewer than 2 mitotic figures in 10 highpower fields. Immunohistochemically, all cases tested stained positively for vimentin, CD34 and CD99, and 2 cases showed focal myofibroblastic differentiation. Two cases examined ultrastructurally showed a fibroblastic phenotype; focally pinocytic vesicles and microfilaments were identified. SFT represents a distinct neoplasm that should be included in the differential diagnosis of spindle-cell neoplasms in soft tissue, nasal cavity and nasopharynx, urinary bladder, and prostate. Strict diagnostic criteria are necessary to avoid overdiagnosis or confusion with more aggressive neoplasms in these locations.

摘要

本文描述了12例浆膜外孤立性纤维性肿瘤(SFT)的临床病理特征。患者年龄范围为18至72岁(平均48.2岁;中位数54岁);其中5例为女性患者。7个病变发生于软组织(5例位于筋膜周围,1例位于皮下组织,1例位于肌肉组织)。病变部位包括腹股沟(2例)、颈部、右臀部、左肩胛骨、上臂和前腹壁(各1例)。鼻腔内见1例息肉样病变,鼻咽部见1例;2例肿瘤发生于膀胱,1例位于前列腺及前列腺周围组织。9个病变已切除;1例患者行广泛切除,2例患者行经尿道切除术。3例患者随访时间有限,临床病程呈良性。肿瘤大小范围为1.7 cm至20.0 cm(平均5.4 cm;中位数3.5 cm)。组织学上,肿瘤边界清晰,由细胞形态温和的梭形细胞组成,排列无明显模式;局部可见席纹状或束状生长模式。肿瘤细胞被厚的胶原带分隔,可见瘢痕疙瘩样玻璃样变灶。所有病例均可见显著的血管增生,呈血管外皮细胞瘤样血管模式,血管壁增厚且玻璃样变。2例软组织病例有丝分裂活性增加(10个高倍视野中有4 - 6个有丝分裂象);其他病例在10个高倍视野中显示少于2个有丝分裂象。免疫组化方面,所有检测病例波形蛋白、CD34和CD99均呈阳性染色,2例显示局灶性肌成纤维细胞分化。2例超微结构检查显示成纤维细胞表型;局部可见吞饮小泡和微丝。SFT是一种独特的肿瘤,在软组织、鼻腔和鼻咽部、膀胱及前列腺的梭形细胞肿瘤鉴别诊断中应予以考虑。需要严格的诊断标准以避免在这些部位过度诊断或与侵袭性更强的肿瘤混淆。

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