Mehta Aanand, Yadav Manish, Shilpakar Sushil K, Bohara Sandip, Yadav Digraj
Department of General Surgery.
Maharajgunj Medical Campus, Tribhuvan University, Nepal.
Ann Med Surg (Lond). 2024 Jan 18;86(3):1669-1675. doi: 10.1097/MS9.0000000000001713. eCollection 2024 Mar.
Medulloblastoma, a highly malignant embryonal tumor predominantly found in the pediatric population, typically arises within the cerebellum. This case report holds particular importance due to the rarity of medulloblastoma within the cerebellopontine angle (CPA). The distinct anatomical challenge posed by the CPA complex neurovascular structures, along with the absence of pathognomonic clinical or radiographic features, highlights the unique diagnostic and management challenge of this case.
A 5-year-old boy presented with mild, progressively worsening headaches on CT/MRI imaging, which revealed a solid mass in the left CPA. Radiologically, the lesion closely resembled a CPA meningioma. The patient underwent a left retrosigmoid suboccipital craniectomy, utilizing a modified park bench position and careful burrhole creation. Intraoperatively, the tumor exhibited well-defined margins, firm adherence to cranial nerves, and complex tissue characteristics. Postoperatively, histopathological analysis identified nodular medulloblastoma, WHO grade IV, with immunohistochemical markers confirming its subtype.
This case highlights the critical role of surgical intervention in addressing rare tumors, emphasizing the need for multidisciplinary collaboration in both diagnosis and management to achieve a favorable outcome. Uncommon tumor locations, such as the CPA, require tailored approaches, and the utilization of advanced diagnostic techniques, including immunohistochemistry, aids in accurate subtype classification.
This case highlights the critical role of surgical intervention in addressing rare tumors, emphasizing the need for multidisciplinary collaboration in both diagnosis and management to achieve a favorable outcome.
髓母细胞瘤是一种高度恶性的胚胎性肿瘤,主要见于儿童群体,通常起源于小脑。由于小脑桥脑角(CPA)内髓母细胞瘤罕见,本病例报告具有特殊重要性。CPA复杂的神经血管结构带来独特的解剖学挑战,且缺乏特征性的临床或影像学表现,凸显了该病例独特的诊断和管理挑战。
一名5岁男孩因CT/MRI成像显示轻度、逐渐加重的头痛就诊,结果发现左CPA有一个实性肿块。影像学上,该病变与CPA脑膜瘤极为相似。患者采用改良的公园长椅体位并小心钻孔,接受了左乙状窦后枕下开颅手术。术中,肿瘤边界清晰,与颅神经紧密粘连,组织特征复杂。术后,组织病理学分析确定为结节性髓母细胞瘤,WHO四级,免疫组化标记物证实了其亚型。
本病例突出了手术干预在处理罕见肿瘤中的关键作用,强调在诊断和管理中多学科协作以取得良好结果的必要性。如CPA这样不常见的肿瘤位置需要量身定制的方法,使用包括免疫组化在内的先进诊断技术有助于准确的亚型分类。
本病例突出了手术干预在处理罕见肿瘤中的关键作用,强调在诊断和管理中多学科协作以取得良好结果的必要性。