Mesenchymal Chondrosarcoma, Clinicopathological Characteristics of an Uncommon Tumor

M. Raza, Mudassar Hussain, N. Uddin, N. Akhter, Usman Hassan
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引用次数: 4

Abstract

Mesenchymal chondrosarcomas (MCS) are very rare malignant tumors. They comprise 2–10% of all chondrosarcomas. Approximately 600 cases of MCS have been published, as case reports and small series. In English language medical literature only three series include 20 or more cases have been described. This paper aims to document various histological findings, histological patterns that can be seen in MCS. In addition, demographic features, clinical and radiological findings and survival data are also incorporated to assess prognostic impact of various histological findings. This is a descriptive cross sectional study including cases of MCS diagnosed at Shaukat Khanum Memorial Cancer Hospital and Research Center, Lahore, Pakistan between 2002 and 2012. The slides were reviewed by two consultant pathologists independently and findings (pattern, cellularity, spindle cell component, necrosis, mitoses, hemangiopericytoma like vessels) were documented on a specially developed form. The clinical information was extracted from case files. Survival data was obtained by contacting the patient or their families in those cases for which contact numbers were available. Data analysis was done using SPSS 20.0. Of the total 18 cases, 10 were males (55.6%) and 8 were females (44.4%). Patients were most commonly in their second or third decades of life. Tumor locations included craniofacial bones (n=5, 28%), ribs and chest wall (n=3, 16%), spine (n=3, 16%), and the lower extremity bones (n=5, 28%). Extraskeletal sites included parapharyngeal region (n=1, 6%) and thigh (n=1, 6%). Histologically biphasic pattern was seen 16 cases (89%). Two cases showed sheets of round to spindle cells with very focal cartilaginous component (11%). Spindle cell component was seen in 14 cases. Necrosis was present in 7 cases (38.9%). Survival data was available for 11 patients (61%). Among these, n=7 (64%) patients developed metastatic disease. The mean survival for cases with metastatic disease was 14.2 months. In patients without metastatic disease, mean survival was 37 months. As the incidence of mesenchymal chondrosarcoma is extremely low, the limited number of patients discussed in most of the studies precludes statistically significant conclusions. MCS should be considered in the differential diagnosis, when pathologists encounter a bone and soft tissue tumor with biphasic pattern composed of round to spindle cell component and hyaline cartilage. Given the diagnostic pitfalls discussed, clinicians may consider obtaining larger biopsy specimens to limit sampling error or confirming the diagnosis with immunohistochemical stains or genetic analysis when a larger biopsy is not possible.
间充质软骨肉瘤:一种罕见肿瘤的临床病理特征
间充质软骨肉瘤是一种非常罕见的恶性肿瘤。它们占所有软骨肉瘤的2-10%。已经发表了大约600例MCS病例,包括病例报告和小型丛书。在英语医学文献中,只有三个系列包括20个或更多的病例被描述。本文旨在记录各种组织学发现,组织学模式,可以看到在MCS。此外,还纳入了人口统计学特征、临床和放射学发现以及生存数据,以评估各种组织学发现对预后的影响。这是一项描述性横断面研究,包括2002年至2012年在巴基斯坦拉合尔Shaukat Khanum纪念癌症医院和研究中心诊断的MCS病例。两名咨询病理学家独立检查了载片,发现(模式,细胞结构,梭形细胞成分,坏死,有丝分裂,血管外皮细胞瘤样血管)被记录在一个特殊的发展形式上。临床资料是从病例档案中提取的。生存数据是通过与有联系电话的患者或其家属联系获得的。数据分析采用SPSS 20.0软件。18例中,男性10例(55.6%),女性8例(44.4%)。患者最常见于生命的第二或第三个十年。肿瘤部位包括颅面骨(n=5, 28%)、肋骨和胸壁(n=3, 16%)、脊柱(n=3, 16%)和下肢骨(n=5, 28%)。骨外部位包括咽旁区(n= 1.6%)和大腿(n= 1.6%)。组织学双相型16例(89%)。2例显示圆形至梭形细胞片,有非常局灶性的软骨成分(11%)。梭形细胞成分14例。坏死7例(38.9%)。11例患者(61%)获得了生存数据。其中,n=7(64%)例患者发生转移性疾病。转移性疾病患者的平均生存期为14.2个月。无转移性疾病患者的平均生存期为37个月。由于间充质软骨肉瘤的发病率极低,大多数研究中讨论的患者数量有限,因此无法得出具有统计学意义的结论。当病理学家遇到由圆形到梭形细胞成分和透明软骨组成的双相骨软组织肿瘤时,应考虑MCS作为鉴别诊断的依据。鉴于所讨论的诊断缺陷,临床医生可能会考虑获得更大的活检标本以限制采样误差,或者在不可能进行更大的活检时,使用免疫组织化学染色或遗传分析来确认诊断。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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