Department of Neurosurgery, All India Institute of Medical Sciences, Room No 720, CNC, New Delhi, India.
Department of Neuroradiology, All India Institute of Medical Sciences, New Delhi, India.
Childs Nerv Syst. 2024 Sep;40(9):2723-2733. doi: 10.1007/s00381-024-06452-2. Epub 2024 May 19.
Intracranial mesenchymal chondrosarcoma (IMC) is a rare malignant tumor in pediatric population. IMC can present as extra- or intra-axial lesion in pediatric patients, though the former is commoner causing raised intracranial pressure (ICP). Radiological diagnosis is a challenge in these cases, as is it difficult to differentiate these from other extra-axial neoplasms due to the wide differential diagnosis in pediatric population. We aim to systematically review the literature and present a rare case of extraskeletal intracranial mesenchymal chondrosarcoma treated with safe maximal resection.
A systematic review of literature was conducted in accordance with PRISMA guidelines. PubMed and Scopus databases were queried using the search terms, "primary intracranial chondrosarcoma", "extraskeletal mesenchymal chondrosarcoma", "mesenchymal chondrosarcoma" and "pediatric". Presentation, surgical management and outcome of a 15-year-old male with an extraskeletal IMC are also described.
The search yielded 25 articles which met the inclusion criteria. These published records consisted of 33 IMC cases with mean age at presentation of 9.81 ± 5.2 years (range 2 months to 18 years). Frontal region was the commonest locations (11, 33.3%). Most common presentation was headache (14, 42.4%). All patients underwent surgical intervention: gross total resection (20, 60.6%), subtotal resection (9, 27.3%) and no extent mentioned (4, 12.1%). No adjuvant therapy was received in 15 patients (45.5%). On latest follow-up, 11 patients (33.3%) are on remission, 5 patients (15.2%) are symptom free, 3 patients (9.1%) had recurrence, 2 patients (6.1%) had metastasis and 9 patients (27.3%) expired.
IMC is a rare entity in pediatric population with imaging findings which are non-characteristic leading to its diagnostic challenge. It can masquerade as other extra-axial intracranial neoplasm (meningioma or hemangiopericytoma). Combination of clinico-radiological and pathological examination can help in accurate diagnosis. Safe Maximal resection followed by radiotherapy is the preferred treatment strategy.
颅内间叶性软骨肉瘤（IMC）是儿科人群中罕见的恶性肿瘤。IMC 可在儿科患者中表现为颅外或颅内病变，尽管前者更常见，会导致颅内压升高（ICP）。由于儿科人群的广泛鉴别诊断，这些病例的放射学诊断具有挑战性，因为很难将其与其他颅外肿瘤区分开来。我们旨在系统地回顾文献，并介绍一例接受安全最大切除术治疗的罕见颅外骨外间叶性软骨肉瘤病例。
根据 PRISMA 指南进行了系统的文献回顾。使用搜索词“原发性颅内软骨肉瘤”、“骨外间叶性软骨肉瘤”、“间叶性软骨肉瘤”和“儿科”在 PubMed 和 Scopus 数据库中进行了检索。还描述了一名 15 岁男性的临床表现、手术治疗和结果。
检索结果得到 25 篇符合纳入标准的文章。这些发表的记录包括 33 例 IMC 病例，平均发病年龄为 9.81±5.2 岁（范围为 2 个月至 18 岁）。额区是最常见的部位（11 例，33.3%）。最常见的表现是头痛（14 例，42.4%）。所有患者均接受了手术干预：完全切除（20 例，60.6%）、次全切除（9 例，27.3%）和未提及切除范围（4 例，12.1%）。15 例患者未接受辅助治疗（45.5%）。在最新的随访中，11 例患者（33.3%）处于缓解状态，5 例患者（15.2%）无任何症状，3 例患者（9.1%）复发，2 例患者（6.1%）转移，9 例患者（27.3%）死亡。
IMC 在儿科人群中是一种罕见的实体，其影像学表现非特征性，导致诊断具有挑战性。它可能伪装成其他颅外颅内肿瘤（脑膜瘤或血管外皮细胞瘤）。临床-放射学和病理学检查的结合有助于准确诊断。安全的最大切除术加放疗是首选的治疗策略。
