Laryngeal chondrosarcoma – Ten years of experience

IntroductionLaryngeal involvement by cartilaginous tumors is rare. However, although accounting for only 1% of laryngeal tumor pathology, they are the most frequently occurring non-epithelial neoplasms. The most probable location is the endolaryngeal surface of the cricoid cartilage. Their symptoms are variable, depending on the size and location, and may include hoarseness, stridor, and dyspnea. Treatment is based on surgical excision. Some centers take into account the degree of differentiation and whether it is a case of relapse when deciding to perform a radical resection.AimTo evaluate this disease in a sample of the Portuguese population.MethodsA review of the medical records from 2002 to 2012 by assessment of clinical processes was performed. Data on demographics, clinical treatments, and outcomes were collected.ResultsSix patients were included in the study. Five of them underwent total laryngectomy, and in one case, partial excision of the thyroid cartilage was performed. None of the patients had either metastases or tumor-related death.ConclusionLaryngeal chondrosarcomas remain a rare disease of unknown etiology, with slow and insidious symptoms. The treatment is surgical, with favorable prognosis, and metastases rarely occur. The main concern regards their propensity to relapse.     

骨软骨瘤继发肉瘤变临床病理分析

目的 分析骨软骨瘤继发肉瘤变的临床、影像学、组织病理学和免疫表型特征.方法 回顾上海交通大学附属第六人民医院1991-2008年间诊断的骨软骨瘤463例,其中单发性408例,多发性55例;重点分析11例骨软骨瘤继发肉瘤变病例的临床、影像学和组织学形态特点;采用免疫组织化学EnVision二步法检测7例骨软骨瘤肉瘤变组织及lO例未发生肉瘤变组织中CK、波形蛋白、S-100蛋白、p53及c-myc蛋白表达.结果 11例骨软骨瘤恶变病例其中5例为单发性(单发性恶变率1.2%),6例为多发性(多发性恶变率10.9%).男女比例为10:1.肉瘤变的部位分别是股骨4例,胫骨3例,肱骨1例,髂骨3例,耻骨1例,其中1例是髂骨和股骨联合病变.肉瘤变患者平均就诊年龄39.8岁,未肉瘤变骨软骨瘤平均就诊年龄20.4岁.肿瘤在骨表面结节状生长,体积巨大,边界不清,软骨帽厚度＞1.5 cm,有广泛钙化.恶变成分为周围型软骨肉瘤Ⅰ～Ⅱ级,肿瘤细胞侵及周围软组织,偶可累及髓腔.免疫组织化学标记结果显示肿瘤细胞表达波形蛋白和S-100蛋白.p53阳性表达见于其中2例肉瘤变的病例(2/7),未发生肉瘤变的病例未检测到p53阳性表达.结论 骨软骨瘤肉瘤变大多继发于多发性病例,发病年龄通常在30岁以后,有明显的男性发病优势,临床病史长,肿瘤体积巨大,软骨帽厚,钙化明显,结节状生长浸润周围软组织.恶变成分主要是周围型高分化软骨肉瘤,预后较好.p53基因突变可能是恶变的分子机制之一.     

肋软骨肉瘤的X线表现(附5例报告)

目的探讨肋软骨肉瘤的X线、CT表现,为临床提供可靠的佐证。方法回顾性分析5例经病理证实的肋软骨肉瘤的X线、CT表现。结果发生在肋弓部2例,1例前支肋软骨,2例后支肋软骨。X线、CT均可见骨质破坏、消失,软组织肿块伴不同形态的钙化。结论肋软骨肉瘤具有特征性的影像学表现,术前不难明确诊断,并可为临床拟定治疗方案提供有价值的佐证资料。     

颌骨软骨肉瘤10例诊治分析

目的探讨颌骨软骨肉瘤的治疗方式和预后的关系。方法对1999至2009年收治于中国医学科学院肿瘤医院的10例颌骨软骨肉瘤患者进行回顾性分析。结果 10例患者中,4例患者行单纯手术治疗,6例患者行手术＋放疗。总体5年生存率为70.0%,局部复发率为50.0%（5/10）,远处转移率为20.0%（2/10）。单纯手术组2年无复发生存率为100%,而手术＋放疗组为40.0%,但二者差异无显著性（P=0.11）。结论根治性手术切除是颌骨软骨肉瘤的主要治疗方式,对于切除不彻底的患者,可以考虑辅助放疗,但疗效有待进一步研究证实。     

Expression of matrix metalloproteinases and tissue inhibitors of metalloproteinases in human chondrosarcomas

The aim of the present study was to characterise the ability of malignant chondrosarcomas to invade normal bone by analysing their production of matrix metalloproteinases (MMPs) and tissue inhibitors of metalloproteinases (TIMPs). For this purpose 12 chondrosarcomas were investigated for the expression of mRNAs for several MMPs and all 4 TIMPs by Northern hybridisation, and for immunohistochemical localisation of the proteins. A characteristic finding of these analyses was increased expression of MMP-13, MMP-14 and TIMP-2 mRNAs in chondrosarcomas when compared with nonmalignant control samples. Individual chondrosarcomas also exhibited elevated levels of MMP-1, MMP-7 and MMP-9 mRNAs. The results of Northern hybridisations were supported by immunohistochemical stainings of the corresponding tumour areas for MMP-2, MMP-14 and TIMP-2, further suggesting that these may have prognostic value for determining whether individual chondrosarcomas are locally aggressive or have a probability of recurrence. Another finding of the present study was a marked heterogeneity in histologic appearance and gene expression of the chondrosarcomas, emphasising the importance of analysing several areas of these tumours to get representative results. These findings suggest that analysis of MMPs could be a useful diagnostic indicator in patients with cartilaginous tumours and could help in differentiating between a low-grade malignant chondrosarcoma and a benign growing enchondroma.     

The role of surgical margins in chondrosarcoma

Introduction

Chondrosarcoma (CS) is the second most common primary bone sarcoma with no clear role for adjuvant therapy. The purpose of this study was to investigate (1) the relationship between surgical excision margins and local recurrence free survival (LRFS), and (2) the role of local recurrence (LR) in disease specific survival (DSS) in CS of the extremity and pelvis.

Postoperative spot-scanning proton radiation therapy for chordoma and chondrosarcoma in children and adolescents: initial experience at paul scherrer institute

PURPOSE: To evaluate postoperative spot-scanning proton radiation therapy (PT) and intensity-modulated PT (IMPT) for chordoma and chondrosarcoma in pediatric patients. METHODS AND MATERIALS: Between 2000 and 2005, 10 patients (six male patients, four female patients; six chordomas, four chondrosarcomas), aged 10-20 years (median, 16 years), were treated at our institute. Tumor sites were in the brain (one case), skull base (five cases), cervical (three cases), and lumbar spine (one case). Three children had complete resections. In seven children, resection was incomplete, leaving residual tumor behind (range, 2.3-46.3 mL). PT was delivered using spot scanning, with (three patients) or without (seven patients) IMPT. Total dose was 74.0 cobalt Gray equivalents (CGE) for chordoma, and 63.2-68.0 CGE for chondrosarcoma (median, 66.0), depending on histopathological grading and whether the patient had concurrent chemotherapy. RESULTS: Median follow-up time was 36 months (range, 8-77 months). Radiation treatment was well tolerated. All patients remained failure-free at their last follow-up. Late adverse events were reported in three patients and were mild (neurosensory in one patient; alopecia and hypoaccusis in one patient) to moderate (one patient, Grade 2 pituitary insufficiency). CONCLUSIONS: Postoperative spot-scanning PT, delivered in combination with and without IMPT, for chordoma and chondrosarcoma in children and adolescents was tolerated without unacceptable adverse event and initial outcome is perfectly satisfactory in this small cohort. Longer follow-up time and larger cohort are needed to more fully assess tumor control, adverse events, as well as functional and cosmetic outcome.     

骨外黏液样软骨肉瘤一例报道及文献复习

目的结合1例典型病例和文献复习,探讨骨外黏液样软骨肉瘤（EMC）的病理和临床特点。方法报道11例61岁女性左腹股沟EMC的临床表现、诊治经过、组织形态表现,复习近期国内外相关研究报道,包括临床表现、病理学、细胞遗传学、影响预后因素和治疗方法。结果该例术中冰冻病理考虑软骨肉瘤,遂行右髋关节离断术,术后常规病理证实诊断,随访10个月,无复发。结论EMC发病率低易误诊,临床应结合病理检查,目前手术是最主要的治疗方式。     

去分化软骨肉瘤的诊断及治疗进展*

去分化软骨肉瘤（DDCS）约占所有软骨肉瘤的10% ,预后极差,5 年生存率不到10% 。好发于股骨、肱骨和骨盆。DDCS是软骨肉瘤中的一个独特类型。典型的特点是分化良好的软骨样成分和高度恶性的间充质细胞来源的肉瘤成分并存、毗邻。DDCS的诊断非常复杂,需要详细的影像学和病理学检查及准确的活检。DDCS去分化成分可以是骨肉瘤、恶性纤维组织细胞瘤,甚至是任何级别的未分化肉瘤成分。约1/3 的X 光片,1/3 的MR,一多半的CT扫描,DDCS表现为典型的“双态”征。最近利用微阵列- 比较基因组杂交技术,发现反复发生的5q14.2~q21.3,6q16~q25.3,9p24.2~q12和9p21.3。染色体缺失更多见于高度恶性的软骨肉瘤（3 级和DDCS）,该差异具有统计学意义。9 号染色体的缺失是DDCS最常见的染色体缺失。早期研究发现DDCS的去分化成分有p53和p53杂合性的丢失现象,进一步研究发现同时伴随Rb基因杂合性的丢失。DDCS的两种成分可出现p16INK 4,FHIT和E-cadherin（上皮型钙黏附蛋白）甲基化的异常。手术切除包括合适足够的外科切缘或根治性的切除,是目前DDCS最主要的治疗手段。化疗效果目前仍然不确定。最近针对软骨肉瘤（包括DDCS）发现了一些新的药物靶标,有些已经进入临床Ⅱ期试验阶段,其中包括Apomab、Perifosine （哌立福新）、Dasatinib（达沙替尼）和多烯紫杉醇联合吉西他滨的联合化疗。同时几个Ⅰ期药物临床试验报告针对DDCS新的有效药物,如组蛋白去乙酰酶抑制剂和血管内皮生长因子反义寡合甘酸。DDCS患者预后极差,预后主要由DDCS中的去分化成分决定。因此,早期诊断、早手术对改善患者的预后非常关键。