Rare Bone Tumor

罕见骨肿瘤
  • 文章类型: Case Reports
    影响手部的骨巨细胞瘤(GCT)是一种罕见的病变,通常在晚期诊断,复发率高。在目前的文献中,GCT被描述为主要是间充质来源的破骨细胞间质细胞肿瘤。它由三种细胞类型组成:肿瘤GCT基质细胞;单核细胞;和多核巨细胞。临床影像学是诊断GCT的基本。手部的这种肿瘤往往较不偏心,最常见的是中央。掌骨的GCT被认为是一个罕见的位置,发病率低至2%。与其他网站相比,现有的GCT在当地更具侵略性,增长得更快,复发率较高。一名22岁的男性患者出现左手肿胀7个月,自发发作,尺寸逐渐进步,痛苦地限制关节运动,没有跌倒或外伤史.在检查中,5×5×3cm的弥漫性肿胀触诊时触痛,限制第四掌指关节的运动.MRI扫描后的X线平片显示第4掌骨的CampanacciIII级GCT。开放式活检显示肿大的溶解性肿块,出血和坏死区域。有丝分裂图很少,肿瘤被诊断为GCT。在手术切除时,除基底外,整个第4掌骨区域均可见脆弱的肿瘤组织。患者通过手术病灶内切除肿块来管理,然后进行克氏针固定和合成骨移植重建。将切除的组织送去进行组织病理学检查。定期对病人进行随访,最初的夹板,然后在术后6周移除电线,并进行充分的物理治疗,患者耐受。在3个月的随访中,运动范围已经恢复到功能水平,具有良好的移植物吸收,没有其他并发症。手的GCT是这种疾病的罕见表现,需要细致的检查,包括全面的临床检查,血液学,放射学,和病理检查。文献中描述的GCTs的各种治疗方式是单独刮宫,刮宫和植骨,整块切除,截肢,切除和重建,但是单独刮治或植骨刮治对长骨和手部的GCT也无效,也是。这样的手术产生了骨骼空洞,因此需要进行具有挑战性的重建手术,需要使用自体移植物进行重建。同种异体移植,或硅橡胶(合成)植入物。
    Giant-cell tumor (GCT) of the bone affecting the hand is a rare lesion that is usually diagnosed at an advanced stage and has a high rate of recurrence. In the current literature, GCT is described as a predominantly osteoclastogenic stromal cell tumor of mesenchymal origin. It is composed of three cell types: the neoplastic GCT stromal cells; mononuclear monocyte cells; and multinucleated giant cells. Clinical imaging is basic for the diagnosis of a GCT. This tumor within the hand tends to be less eccentric and most often central. GCT of metacarpals is noted to be a rare location, with the incidence being as low as 2%. GCT on hand as compared to other sites is locally more aggressive, grows faster, and has a higher recurrence rate. A 22-year-old male patient presented with swelling over the left hand for 7 months, spontaneous in onset, gradually progressive in size, and painfully restricting the joint movement, with no history of fall or trauma. On examination, diffuse swelling of size 5 × 5 × 3 cm was tender on palpation, restricting the movement at the 4th metacarpophalangeal joint. A plain radiograph followed by an MRI scan revealed a Campanacci\'s Grade III GCT of the 4th metacarpal. An open biopsy showed an expanded and lytic mass with areas of hemorrhage and necrosis. There were few mitotic figures and the tumor was diagnosed to be a GCT. On surgical resection, friable tumor tissue was noted over the region of the entire 4th metacarpal except for the base. The patient was managed by surgical intralesional excision of the mass, followed by Kirschner-wire fixation and reconstruction with synthetic bone graft. The excised tissue was sent for histopathological examination. The patient was followed up at regular intervals, with initial splinting, followed by wire removal at 6-week post-op, and with adequate physiotherapy, as tolerated by the patient. On a 3-month follow-up, the range of motion had returned to a functional level, with good uptake of graft, and no other complications. GCT of the hand is a rare presentation of the disease and requires meticulous workup, including a thorough clinical exam, hematological, radiological, and pathological workup. The various treatment modalities described in the literature for GCTs are curettage alone, curettage and bone graft, en-bloc resection, amputation, and resection with reconstruction, but curettage alone or curettage with bone graft is not effective even for GCTs of long bones and hand, too. Such a procedure creates a skeletal void and hence furthers the need for a challenging reconstructive procedure requiring reconstruction using autograft, allograft, or silastic (synthetic) implant.
    导出

    更多引用

    收藏

    翻译标题摘要

    我要上传

       PDF(Pubmed)

  • 文章类型: Case Reports
    背景:促纤维化瘤(DF)是一种极为罕见的局部侵袭性骨肿瘤,占所有原发性骨肿瘤的0.11%。典型的临床表现是受影响区域上方的疼痛和肿胀。最常见的受累部位是下颌骨和长骨干meta端。组织学和生物学,促纤维增生性纤维瘤模仿软组织的腹外硬纤维瘤。
    一例27岁的男子,髂骨有DF,包括临床,放射学和组织学发现超过4年的时间在这里介绍。比较了手术干预前3年间隔进行的CT扫描与肿瘤扩展和皮质突破的关系。根据解剖学考虑,对患者进行刮治和移植治疗。18个月的后续CT扫描也记录在这里。此外,我们对271例病例进行了回顾和分析,包括本病例,重点介绍了MRI和CT的成像模式以及治疗方式和结果.
    结论:在纤维增生性纤维瘤患者中,CT是诊断骨内肿瘤扩展和评估皮质受累的首选成像技术。而MRI有利于评估骨外肿瘤的生长和术前计划。虽然肿瘤切除仍然是DF的首选治疗方法,刮治和移植被证明是一种可接受的替代治疗方式,当无法切除时,应密切随访。刮治和移植已显示出良好的临床效果,并且与长时间的无复发间隔有关。
    BACKGROUND: Desmoplastic fibroma (DF) is an extremely rare locally aggressive bone tumor with an incidence of 0.11% of all primary bone tumors. The typical clinical presentation is pain and swelling above the affected area. The most common sites of involvement are the mandible and the metaphysis of long bones. Histologically and biologically, desmoplastic fibroma mimics extra-abdominal desmoid tumor of soft tissue.
    UNASSIGNED: A case of a 27-year old man with DF in the ilium, including the clinical, radiological and histological findings over a 4-year period is presented here. CT scans performed in 3-year intervals prior to surgical intervention were compared with respect to tumor extension and cortical breakthrough. The patient was treated with curettage and grafting based on anatomical considerations. Follow-up CT scans over 18-months are also documented here. Additionally, a review and analysis of 271 cases including the presented case with particular emphasis on imaging patterns in MRI and CT as well as treatment modalities and outcomes are presented.
    CONCLUSIONS: In patients with desmoplastic fibroma, CT is the preferred imaging technique for both the diagnosis of intraosseus tumor extension and assessment of cortical involvement, whereas MRI is favored for the assessment of extraosseus tumor growth and preoperative planning. While tumor resection remains the preferred treatment for DF, curettage and grafting prove to be an acceptable alternative treatment modality with close follow-up when resection is not possible. Curettage and grafting have been shown to provide good clinical results and are associated with long recurrence free intervals.
    导出

    更多引用

    收藏

    翻译标题摘要

    我要上传

    求助全文

公众号