Clinical and Pathological Characteristics of Extrapleural Solitary Fibrous Tumor
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摘要:
目的 探讨胸膜外孤立性纤维性肿瘤的临床病理特征及鉴别诊断。 方法 对26例胸膜外孤立性纤维性肿瘤样本进行HE及免疫组织化学染色,并结合临床及影像学资料进行综合分析。 结果 孤立性纤维性肿瘤的组织学形态多样,典型者可见卵圆形或梭形细胞不规则排列,间质中可见粗大的胶原带、鹿角状血管及管周透明变性;免疫组化染色STAT-6、CD34、Bcl-2、CD99、SMA阳性率分别为96.2%、92.3%、92.3%、76.9%、26.9%。 结论 SFT常见发生于胸膜,亦可发生于其它部位,其组织学特征和免疫组化表型有助于明确诊断,但当其发生于不常见的解剖部位时,应当对重点需要鉴别或排除的疾病加以认真细致的鉴别并综合判断其临床预后。 -
关键词:
- 胸膜外孤立性纤维性肿瘤 /
- 组织学形态 /
- 生物学行为 /
- 临床预后
Abstract:Objective To investigate the clinical and pathological characteristics of the extrapleural solitary fibrous tumor and the relevant differential diagnosis. Methods HE and immunohistochemical staining were performed on 26 samples of isolated fibrous tumors outside the pleura, and a comprehensive analysis was conducted based on the clinical and imaging data. Results The histological morphology of isolated fibrous tumors was diverse, with typical cases showing the irregular arrangement of oval or spindle shaped cells. In the stroma, thick collagen bands, antler like blood vessels, and peritubular transparent degeneration could be seen. The immunohistochemical staining demonstrated that the positive rates of STAT-6、CD34、Bcl-2、CD99、SMA were 96.2%、92.3%、92.3%、76.9% and 26.9%, respectively. Conclusion SFT commonly occurs in the pleura and can also occur in other areas. Its histological characteristics and immunohistochemical phenotype help to clarify the diagnosis. However, when it occurs in uncommon anatomical sites, it is necessary to carefully and meticulously distinguish and comprehensively judge the clinical prognosis of key diseases that need to be distinguished or excluded.. -
表 1 病例基本情况
Table 1. Basic information of patients
性别 n 年龄(岁) 肿瘤大小(cm) 男 15 >55(n = 6) >5(n = 14) 女 11 ≤55(n = 20) ≤5(n = 12) 表 2 胸膜外孤立性纤维性肿瘤的病理学特征
Table 2. Pathological characteristics of ESFT
病理特征 n 占比(%) 组织学形态 “鹿角状”薄壁血管 13 50.0 血管周透明变性 6 23.1 细胞丰富、有异型性 1 3.8 小灶坏死 1 3.8 间质黏液变性 6 23.1 微囊性变 3 11.5 小出血灶 3 11.5 成熟脂肪细胞 2 7.7 淋巴细胞浸润 2 7.7 免疫表型 STAT-6 25 96.2 CD34 24 92.3 Bcl-2 24 92.3 Vimentin 26 100.0 CD99 20 76.9 SMA 7 26.9 S-100 6 23.1 EMA 13 50.0 Desmin 1 3.8 CD117 0 0 -
[1] Joon H C,Jae Y R. The 2020 WHO Classification of tumors of soft tissue: Selected changes and new entities[J]. Adv Anat Pathol,2021,28(1):44-58. [2] Papathanassiou Z G,Alberghini M,Picci P,et al. Solitary fibrous tumors of the soft tissues: Imaging features with histopathologic correlations[J]. Clin Sarcoma Res,2013,3(1):1. doi: 10.1186/2045-3329-3-1 [3] Erdag G,Qureshi H S,Patterson J W,et al. Solitary fibrous tumors of the skin: A clinicopathologic study of 10 cases and review of the literature[J]. J Cutan Pathol,2007,34(11):844-850. doi: 10.1111/j.1600-0560.2006.00728.x [4] Liu Y Q,Yue J Q. Intramural solitary fibrous tumor of the ileum: A case report and review of the literature[J]. J Cancer Res Ther,2013,9(4):724-726. doi: 10.4103/0973-1482.126469 [5] Ronchi A,La Mantia E,Gigantino V,et al. A rare case of malignant solitary fibrous tumor in prostate with review of the literature[J]. Diagn Pathol,2017,12(1):50. doi: 10.1186/s13000-017-0640-5 [6] Dungarwalla M M,Barrett A W,Gulati A. Solitary fibrous tumour of the tongue: A series of four cases[J]. J Laryngol Otol,2017,131(9):838-841. doi: 10.1017/S0022215117001396 [7] Papi G,Corrado S,Uberti E D,et al. Solitary fibrous tumor of the thyroid gland[J]. Thyroid,2007,17(2):119-126. doi: 10.1089/thy.2005.0202 [8] Bauer J L,Miklos A Z,Thompson L D. Parotid gland solitary fibrous tumor: A case report and clinicopathologic review of 22 cases from the literature[J]. Head Neck Pathol,2012,6(1):21-31. doi: 10.1007/s12105-011-0305-8 [9] Tanaka K,Yano H,Hayashi H,et al. Total resection combined with osteotomy is more effective for orbital solitary fibrous tumor excision: A report of three cases[J]. Int Ophthalmol,2018,38(1):345-351. doi: 10.1007/s10792-016-0421-2 [10] Insabato L,Siano M,Somma A,et al. Extrapleural solitary fibrous tumor: A clinicopathologic study of 19 cases[J]. Int J Surg Pathol,2009,17(3):250-254. doi: 10.1177/1066896909333779 [11] Chang S K,Yoon D Y,Choi C S,et al. CT,MR,and angiography findings of a solitary fibrous tumor of the larynx: A case report[J]. Korean J Radiol,2008,9(6):568-571. doi: 10.3348/kjr.2008.9.6.568 [12] Shim Y S,Choi S J,Kim H S,et al. Solitary fibrous tumor of the trachea: CT findings with a pathological correlation[J]. Korean J Radiol,2008,9(3):286-289. doi: 10.3348/kjr.2008.9.3.286 [13] Placide N,Robert S. Solitary fibrous tumor of the vagina: A case report with review of the literature[J]. Int J Surg Pathol,2012,20(1):101-104. doi: 10.1177/1066896911413577 [14] Bouvier C,M é tellus P,De Paula A M,et al. Solitary fibrous tumors and hemangiopericytomas of the meninges: Overlapping pathological features and common prognostic factors suggest the same spectrum of tumors[J]. Brain Pathol,2012,22(4):511-521. doi: 10.1111/j.1750-3639.2011.00552.x [15] Fine S W,McCarthy D M,Chan T Y,et al. Malignant solitary fibrous tumor of the kidney: Report of a case and comprehensive review of the literature[J]. Arch Pathol Lab Med,2006,130(6):857-861. doi: 10.5858/2006-130-857-MSFTOT [16] Li X M,Reng J,Zhou P,et al. Solitary fibrous tumors in abdomen and pelvis: Imaging characteristics and radiologic-pathologic correlation[J]. World J Gastroenterol,2014,20(17):5066-5073. doi: 10.3748/wjg.v20.i17.5066 [17] Zhang H,Yu S,Wang W,et al. Primary mesenchymal tumors of the pancreas in a single center over 15 years[J]. Oncol Lett,2016,12(5):4027-4034. doi: 10.3892/ol.2016.5155 [18] Feng L H,Dong H,Zhu Y Y,et al. An update on primary hepatic solitary fibrous tumor: An examination of the clinical and pathological features of four case studies and a literature review[J]. Pathol Res Pract,2015,211(12):911-917. doi: 10.1016/j.prp.2015.09.004 [19] Rao N,Colby T V,Falconieri G,et al. Intrapulmonary solitary fibrous tumors: Clinicopathologic and immunohistochemical study of 24 cases[J]. Am J Surg Pathol,2013,37(2):155-166. doi: 10.1097/PAS.0b013e31826a92f5 [20] Oike N,Kawashima H,Ogose A,et al. A malignant solitary fibrous tumour arising from the first lumbar vertebra and mimicking an osteosarcoma: A case report[J]. World J Surg Oncol,2017,15(1):100. doi: 10.1186/s12957-017-1161-0 [21] Cranshaw I M,Gikas P D,Fisher C,et al. Clinical outcomes of extra-thoracic solitary fibrous tumours[J]. Eur J Surg Oncol,2009,35(9):994-998. doi: 10.1016/j.ejso.2009.02.015 [22] Lee J Y,Park S E,Shin S J,et al. Solitary fibrous tumor with myxoid stromal change[J]. Am J Dermatopathol,2015,37(7):570-573. doi: 10.1097/DAD.0000000000000154 [23] Guillou L,Gebhard S,Coindre J M. Lipomatous hemangiopericytoma: A fat-containing variant of solitary fibrous tumor? Clinicopathologic,immunohistochemical,and ultrastructural analysis of a series in favor of a unifying concept[J]. Hum Pathol,2000,31(9):1108-1115. doi: 10.1053/hupa.2000.9777 [24] Jing H B,Meng Q D,Tai Y H. Lipomatous hemangiopericytoma of the stomach: A case report and a review of literature[J]. World J Gastroenterol,2011,17(43):4835-4838. doi: 10.3748/wjg.v17.i43.4835 [25] Barazani Y,Tareen B. Rare case of paratesticular solitary fibrous tumour (lipomatous hemangiopericytoma)[J]. Can Urol Assoc J,2012,6(3):E131-133. doi: 10.5489/cuaj.11092 [26] Davies P E,Davis G J,Dodd T,et al. Orbital lipomatous haemangiopericytoma: An unusual variant[J]. Clin Exp Ophthalmol,2002,30(4):281-283. doi: 10.1046/j.1442-9071.2002.00542.x [27] Dei Tos A P,Seregard S,Calonje E,et al. Giant cell angiofibroma. A distinctive orbital tumor in adults[J]. Am J Surg Pathol,1995,19(11):1286-1293. doi: 10.1097/00000478-199511000-00009 [28] Guillou L,Gebhard S,Coindre J M. Orbital and extraorbital giant cell angiofibroma: A giant cell-rich variant of solitary fibrous tumor? Clinicopathologic and immunohistochemical analysis of a series in favor of a unifying concept[J]. Am J Surg Pathol,2000,24(7):971-979. doi: 10.1097/00000478-200007000-00008 [29] Sigel J E,Fisher C,Vogt D,et al. Giant cell angiofibroma of the inguinal region[J]. Ann Diagn Pathol,2000,4(4):240-244. doi: 10.1053/adpa.2000.8129 [30] Mosquera J M,Fletcher C D. Expanding the spectrum of malignant progression in solitary fibrous tumors: A study of 8 cases with a discrete anaplastic component-is this dedifferentiated SFT?[J]. Am J Surg Pathol,2009,33(9):1314-1321. doi: 10.1097/PAS.0b013e3181a6cd33 [31] Thway K,Hayes A,Ieremia E,et al. Heterologous osteosarcomatous and rhabdomyosarcomatous elements in dedifferentiated solitary fibrous tumor: Further support for the concept of dedifferentiation in solitary fibrous tumor[J]. Ann Diagn Pathol,2013,17(5):457-463. doi: 10.1016/j.anndiagpath.2012.08.006 [32] Miettinen M. Immunohistochemistry of soft tissue tumours - review with emphasis on 10 markers[J]. Histopathology,2014,64(1):101-118. doi: 10.1111/his.12298 [33] Schweizer L,Koelsche C,Sahm F,et al. Meningeal hemangiopericytoma and solitary fibrous tumors carry the NAB2-STAT6 fusion and can be diagnosed by nuclear expression of STAT6 protein[J]. Acta Neuropathol,2013,125(5):651-658. doi: 10.1007/s00401-013-1117-6 [34] Mohajeri A,Tayebwa J,Collin A,et al. Comprehensive genetic analysis identifies a pathognomonic NAB2/STAT6 fusion gene,nonrandom secondary genomic imbalances,and a characteristic gene expression profile in solitary fibrous tumor[J]. Genes Chromosomes Cancer,2013,52(10):873-886. doi: 10.1002/gcc.22083 [35] Vivero M,Doyle L A,Fletcher C D,et al. GRIA2 is a novel diagnostic marker for solitary fibrous tumour identified through gene expression profiling[J]. Histopathology,2014,65(1):71-80. doi: 10.1111/his.12377 [36] Demicco E G,Harms P W,Patel R M,et al. Extensive survey of STAT6 expression in a large series of mesenchymal tumors[J]. Am J Clin Pathol,2015,143(5):672-682. doi: 10.1309/AJCPN25NJTOUNPNF [37] Ouladan S,Trautmann M,Orouji E,et al. Differential diagnosis of solitary fibrous tumors: A study of 454 soft tissue tumors indicating the diagnostic value of nuclear STAT6 relocation and ALDH1 expression combined with in situ proximity ligation assay[J]. Int J Oncol,2015,46(6):2595-2605. doi: 10.3892/ijo.2015.2975 [38] Lecoutere E,Creytens D. Multifocal cytokeratin expression in pleural and abdominal malignant solitary fibrous tumors: An unusual diagnostic pitfall[J]. Virchows Arch,2015,467(1):119-121. doi: 10.1007/s00428-015-1768-x [39] Doyle L A,Vivero M,Fletcher C D,et al. Nuclear expression of STAT6 distinguishes solitary fibrous tumor from histologic mimics[J]. Mod Pathol,2014,27(3):390-395. doi: 10.1038/modpathol.2013.164 [40] Thway K,Jordan S,Fisher C,et al. Updates in the approach to intrathoracic sarcomas[J]. Histopathology,2015,67(6):755-770. doi: 10.1111/his.12771 [41] Musyoki F N,Nahal A,Powell T I. Solitary fibrous tumor: An update on the spectrum of extrapleural manifestations[J]. Skeletal Radiol,2012,41(1):5-13. doi: 10.1007/s00256-010-1032-z [42] Fukasawa Y,Takada A,Tateno M,et al. Solitary fibrous tumor of the pleura causing recurrent hypoglycemia by secretion of insulin-like growth factor II[J]. Pathol Int,1998,48(1):47-52. doi: 10.1111/j.1440-1827.1998.tb03827.x [43] Steigen S E,Schaeffer D F,West R B,et al. Expression of insulin-like growth factor 2 in mesenchymal neoplasms[J]. Mod Pathol,2009,22(7):914-921. doi: 10.1038/modpathol.2009.48 [44] Demicco E G,Park M S,Araujo D M,et al. Solitary fibrous tumor: A clinicopathological study of 110 cases and proposed risk assessment model[J]. Mod Pathol,2012,25(9):1298-1306. doi: 10.1038/modpathol.2012.83 [45] Cardillo G,Carbone L,Carleo F,et al. Solitary fibrous tumors of the pleura: An analysis of 110 patients treated in a single institution[J]. Ann Thorac Surg,2009,88(5):1632-1637. doi: 10.1016/j.athoracsur.2009.07.026 [46] Ito H,Fukuda M,Imamura Y,et al. A malignant solitary fibrous tumor in the retroperitoneum[J]. Int J Clin Oncol,2008,13(2):173-175. doi: 10.1007/s10147-007-0712-8 [47] Bishop A J,Zagars G K,Demicco E G,et al. Soft tissue solitary fibrous tumor: Combined surgery and radiation therapy results in excellent local control[J]. Am J Clin Oncol,2018,41(1):81-85. doi: 10.1097/COC.0000000000000218 [48] Gold J S,Antonescu C R,Hajdu C,et al. Clinicopathologic correlates of solitary fibrous tumors[J]. Cancer,2002,94(4):1057-1068. doi: 10.1002/cncr.10328 [49] Moran C A,Suster S,Koss M N. The spectrum of histologic growth patterns in benign and malignant fibrous tumors of the pleura[J]. Semin Diagn Pathol,1992,9(2):169-180. [50] Briselli M,Mark E J,Dickersin G R. Solitary fibrous tumors of the pleura: Eight new cases and review of 360 cases in the literature[J]. Cancer,1981,47(11):2678-2689. doi: 10.1002/1097-0142(19810601)47:11<2678::AID-CNCR2820471126>3.0.CO;2-9 [51] Vallat-Decouvelaere A V,Dry S M,Fletcher C D. Atypical and malignant solitary fibrous tumors in extrathoracic locations: Evidence of their comparability to intra-thoracic tumors[J]. Am J Surg Pathol,1998,22(12):1501-1511. doi: 10.1097/00000478-199812000-00007