作者投稿
专家审稿
编辑办公
邮件订阅
RSS
-
摘要: 低度恶性肌纤维母细胞肉瘤(low-grade myofibroblastic sarcoma，LGMS)是一种极其罕见的肌成纤维细胞恶性肿瘤，好发于头颈部，口腔内常见于舌部，发生于颌骨的病例较少见，目前仅有12例报道。LGMS常发生于成年男性，儿童少见。本文报道1例罕见的下颌骨LGMS儿童病例，患儿病变累及下颌骨，行手术切除，组织病理学结果显示有梭形细胞增生并向周边横纹肌侵袭性生长；免疫组化结果显示Vimentin、Calponin、SMA和Actin呈阳性表达，支持其肌纤维母细胞和平滑肌分化的特点，诊断为LGMS。本文通过相关文献复习对颌骨LGMS的免疫学特点、病理学特征、治疗和预后进行讨论，以丰富临床医生对该病的认识，提高诊断水平。
-
关键词:
- 低度恶性肌纤维母细胞肉瘤 /
- 下颌骨 /
- 颌骨 /
- 免疫组化
Abstract: Low-grade myofibroblastic sarcoma(LGMS) is an extremely rare malignant tumor of myofibroblastic cells that primarily affects the head and neck region. LGMS occurring in the oral cavity is commonly found on the tongue, and those occurring in the jawbone are less common, with only 12 reported cases to date. LGMS is often in adult males but rare in children. This report describes a rare case of LGMS in the mandible of a child who underwent surgical resection. Histopathological examination revealed spindle cell proliferation with invasive growth into surrounding skeletal muscle. Immunohistochemical staining showed positive expression of Vimentin, Calponin, SMA, and Actin, supporting myofibroblastic and smooth muscle differentiation of the tumor and the diagnosis of LGMS. This article, based on this case and relevant literature, discusses the immunological and pathological characteristics, treatment and prognosis of LGMS in the jawbone, in order to enrich clinicians' understanding and improve the diagnosis of this disease.-
Key words:
- low-grade myofibroblastic sarcoma /
- mandible /
- jaw /
- immunohistochemistry
作者贡献：蓝育明负责资料收集及论文撰写；王芳负责文献整理及论文修订；章杰负责论文审校。利益冲突：所有作者均声明不存在利益冲突注：本研究发表已获得患儿家属知情同意。 -
图 1 患儿术后病理诊断符合下颌低度恶性肌纤维母细胞肉瘤
A.切除肿物大体观；B.肿瘤细胞呈长梭形，HE染色(×100)可见核分裂象；C.Vimentin染色(+)；D.Desmin染色(+)；E.SMA染色(+)；F.Calponin染色(+)
表 1 13例颌骨LGMS患者临床资料
病例 发表年份(年) 第一作者 性别 年龄(岁) 病变部位 治疗方案 随访结果 1 2001 Bisceglia[5] 男 24 上颌骨 扩大切除术 术后40个月无复发 2 2005 仇晓菲[6] 女 31 上颌骨 局部切除术 术后5个月复发 3 2009 Niedzielska[7] 男 54 下颌骨 局部切除术 － 4 2009 Demarosi[8] 男 51 下颌骨 扩大切除术 术后12个月无复发 5 2011 雷丽[9] 女 67 上颌骨 扩大切除术 － 6 2014 Qiu[10] 男 45 上颌骨 扩大切除术 术后30个月无复发 7 2014 Qiu[10] 女 29 上颌骨 局部扩大切除术 术后6个月复发 8 2016 Yu[11] 女 8 下颌骨 扩大切除术，放疗 术后6个月复发，24个月死亡 9 2016 Cai[12] 男 31 下颌骨 局部扩大切除术，放疗 术后18个月无复发 10 2019 Miyazaki[13] 男 1 下颌骨 化疗，局部切除术，放化疗 术后144个月无复发 11 2021 Jayasooriya[14] 女 7 下颌骨 局部扩大切除术 术后12个月无复发 12 2022 Emanuelli[15] 男 11 下颌骨 扩大切除术 术后6个月无复发 13 2023 蓝育明 男 10 下颌骨 局部切除术 术后18个月无复发 注：病例13为本文报道病例；LGMS: 低度恶性肌纤维母细胞肉瘤；-：未提及 
下载: 导出CSV
表 2 13例颌骨LGMS患者免疫组化结果
病例 Vimentin SMA/α-SMA MSA Fibronectin Desmin Calponin Actin Cytokeratin h-caldesmon S-100 CD34 CD10 1 + + + 2 + + 3 + + - + + - - 4 + + - - - 5 + + - - 6 + + + 7 + + + + - 8 + + - - - 9 + + + + - 10 + + + - - 11 + + - - - 12 + - - - 13 + + + + + + - + MSA：肌肉特异性肌动蛋白；Fibronectin：纤维连接蛋白；Actin：肌动蛋白；Cytokeratin：细胞角蛋白；h-caldesmon：重型钙调蛋白结合蛋白；S-100：钙蛋白家族；CD34：血管内皮细胞标志蛋白；CD10：锌依赖金属蛋白酶；Vimentin、SMA、Desmin、Calponin：同图 1；LGMS: 同表 1
下载: 导出CSV
-
[1] Fletcher CD, Unni KK, Mertens F. World Health Organisation Classification of tumors[M]. IARC Press, 2002: 48. [2] Qiu X, Montgomery E, Sun B. Inflammatory myofibroblastic tumor and Low-grade myofibroblastic sarcoma: a comparative study of clinicopathologic features and further observations on the immunohistochemical profile of myofibroblasts[J]. Hum Pathol, 2008, 39: 846-856. doi: 10.1016/j.humpath.2007.10.010 [3] Mentzel T, Dry S, Katenkamp D, et al. Low-grade myofibroblastic sarcoma: analysis of 18 cases in the spectrum of myofibroblastic tumors[J]. Am J Surg Pathol, 1998, 22: 1228-1238. doi: 10.1097/00000478-199810000-00008 [4] Watanabe K, Ogura G, Tajino T, et al. Myofibrosarcoma of the bone: a clinicopathologic study[J]. Am J Surg Pathol, 2001, 25: 1501-1507. doi: 10.1097/00000478-200112000-00005 [5] Bisceglia M, Tricarico N, Minenna P, et al. Myofibrosar-coma of the upper jawbones: a clinicopathologic and ultrastructural study of two cases[J]. Ultrastruct Pathol, 2001, 25: 385-397. doi: 10.1080/019131201317101261 [6] 仇晓菲, 孙保存, 张立华, 等. 9例低度恶性肌纤维母细胞肉瘤的临床病理观察[J]. 中国肿瘤临床, 2005, 32: 582-584, 591. doi: 10.3969/j.issn.1000-8179.2005.10.013 [7] Niedzielska I, Janic T, Mrowiec B. Low-grade myofibro-blastic sarcoma of the mandible: a case report[J]. J Med Case Rep, 2009, 3: 8458. doi: 10.4076/1752-1947-3-8458 [8] Demarosi F, Bay A, Moneghini L, et al. Low-grade myofibroblastic sarcoma of the oral cavity[J]. Oral Surg Oral Med Oral Pathol Oral Radiol Endod, 2009, 108: 248-254. doi: 10.1016/j.tripleo.2009.03.031 [9] 雷丽. 上颌骨低度恶性肌纤维母细胞肉瘤1例报道[J]. 中国当代医药, 2011, 18: 139-140. https://www.cnki.com.cn/Article/CJFDTOTAL-ZGUD201134089.htm [10] Qiu JY, Liu P, Shi C, et al. Low-grade myofibroblastic sarcomas of the maxilla[J]. Oncol Lett, 2015, 9: 619-625. doi: 10.3892/ol.2014.2790 [11] Yu Y, Xiao J, Wang L, et al. Low-Grade Myofibroblastic Sarcoma in the Mandibular Canal: A Case Report[J]. J Oral Maxillofac Surg, 2016, 74: 1501-1505. http://www.onacademic.com/detail/journal_1000038770494510_1d71.html [12] Cai ZG, Pan CC, Yu DH, et al. Myofibroblastic sarcomas: A clinicopathologic analysis of 15 cases and review of literature[J]. Int J Clin Exp Path, 2016, 9: 1568-1577. http://ijcep.com/files/ijcep0012862.pdf [13] Miyazaki R, Ogawa T, Takao K, et al. Low-grade myofibroblastic sarcoma of mandible in an infant[J]. J Oral Maxillofacial Surg, Med, Pathol, 2019, 31: 406-409. doi: 10.1016/j.ajoms.2019.06.008 [14] Jayasooriya PR, Athukorala C, Attygalla M, et al. Low-Grade Myofibroblastic Sarcoma of the Oral Cavity: A Report of Three Cases Illustrating an Emerging Disease in Children[J]. Dermatopathology (Basel), 2021, 8: 1-9. doi: 10.3390/dermatopathology8010001 [15] Emanuelli E, O'Connor M, Garg RK. Management of a Highly Vascular Low-grade Myofibroblastic Sarcoma of the Mandible[J]. Plast Reconstr Surg Glob Open, 2022, 1: e4043. http://www.socolar.com/Article/Index?aid=100092676088&jid=100000008658 [16] Ross R, Everett NB, Tyler R. Wound healing and collagen formation. Ⅵ. The origin of the wound fibroblast studied in parabiosis[J]. J Cell Biol, 1970, 44: 645-654. doi: 10.1083/jcb.44.3.645 [17] Eyden BP, Christensen L. Leiomyosarcoma versus myofibrosarcoma: observations and terminology[J]. Ultrastruct Pathol, 1993, 17: 231-239. doi: 10.3109/01913129309027769 [18] Fisher C. Myofibroblastic malignancies[J]. Adv Anat Pathol, 2004, 11: 190-201. doi: 10.1097/01.pap.0000131773.16130.aa [19] Coyne JD. Low-grade myofibroblastic sarcoma of the piriform fossa: a case report with a literature review of a tumour with a predilection for the head and neck[J]. Br J Oral Maxillofac Surg, 2007, 45: 335-337. doi: 10.1016/j.bjoms.2005.10.015 [20] Myong NH, Min JW. Low-grade myofibroblastic sarcoma arising in fibroadenoma of the breast-A case report[J]. Diagn Pathol, 2016, 11: 33. doi: 10.1186/s13000-016-0480-8?site=diagnosticpathology.biomedcentral.com [21] Morii T, Mochizuki K, Sano H, et al. Occult myofibroblastic sarcoma detected on FDG-PET performed for cancer screening[J]. Ann Nucl Med, 2008, 22: 811-815. doi: 10.1007/s12149-008-0194-4 [22] Ni C, Xu YY, Zhou S H, et al. Differential diagnosis of inflammatory myofibroblastic tumour and Low-grade myofibroblastic sarcoma: two case reports with a literature review[J]. J Int Med Res, 2011, 39: 311-320. doi: 10.1177/147323001103900134 [23] Lu ZJ, Zhou SH, Yan SX, et al. Anaplastic lymphoma kinase expression and prognosis in inflammatory myofibroblastic tumours of the maxillary sinus[J]. J Int Med Res, 2009, 37: 2000-2008. doi: 10.1177/147323000903700639 [24] Laco J, Simakova E, Slezak R, et al. Low grade myofibroblastic sarcoma of tongue: a case report[J]. Cesk Patol, 2006, 42: 150-153. [25] Wu X, Guo L, Li S, et al. Low-grade myofibroblastic sarcoma with abdominal pain, a stuffy nose, hearing loss, and multiple cavity effusion: a case report and literature review[J]. J Int Med Res, 2020, 48: 1219695213. http://pubmed.ncbi.nlm.nih.gov/31975633/ [26] Meng GZ, Zhang HY, Bu H, et al. Myofibroblastic sarcomas: a clinicopathological study of 20 cases[J]. Chin Med J (Engl), 2007, 120: 363-369. doi: 10.1097/00029330-200703010-00003 [27] Kordac P, Nikolov DH, Smatanova K, et al. Low-grade myofibroblastic sarcoma of the larynx: case report and review of literature[J]. Acta Medica (Hradec Kralove), 2014, 57: 162-164. doi: 10.14712/18059694.2015.82 [28] Demarosi F, Bay A, Moneghini L, et al. Low-grade myofibroblastic sarcoma of the oral cavity[J]. Oral Surg Oral Med Oral Pathol Oral Radiol Endod, 2009, 108: 248-254. doi: 10.1016/j.tripleo.2009.03.031 [29] Yamada T, Yoshimura T, Kitamura N, et al. Low-grade myofibroblastic sarcoma of the palate[J]. Int J Oral Sci, 2012, 4: 170-173. doi: 10.1038/ijos.2012.49 [30] Keller C, Gibbs CN, Kelly SM, et al. Low-grade myofibrosarcoma of the head and neck: importance of surgical therapy[J]. J Pediatr Hematol Oncol, 2004, 26: 119-120. doi: 10.1097/00043426-200402000-00013 [31] Yu Y, Xiao J, Wang L, et al. Low-Grade Myofibroblastic Sarcoma in the Mandibular Canal: A Case Report[J]. J Oral Maxillofac Surg, 2016, 74: 1501-1505. http://www.onacademic.com/detail/journal_1000038770494510_1d71.html -
点击查看大图
图(2) / 表(2)
计量
- 文章访问数: 151
- HTML全文浏览量: 40
- PDF下载量: 32
- 被引次数: 0
