Shenoy Vijendra S, Sinha Ananya, Dhawan Saksham, Rao Sneha, K V Apoorva, Chandy Nithya
Dept of ENT and Head & neck surgery, Kasturba Medical College, Mangalore Manipal Academy of Higher Education, Manipal, India.
Dept of Pathology, Kasturba Medical College, Mangalore Manipal Academy of Higher Education, Manipal, India.
Indian J Otolaryngol Head Neck Surg. 2024 Dec;76(6):5872-5878. doi: 10.1007/s12070-024-04951-x. Epub 2024 Aug 24.
Embryonal rhabdomyosarcoma is the malignant mesenchymal proliferation of undifferentiated skeletal muscle. It is the most common soft tissue tumour found in children. Head and neck location accounts for 40% of the cases with the most common variants being of embryonal Nasopharyngeal RMS is a type of parameningeal RMS (arising from tissues adjacent to the meninges) having propensity for CNS and known for having a poorer prognosis, early recurrence, rapid growth and invasion to adjacent structures. Other types of RMS include orbital and non-orbital/non-parameningeal. Here, we encountered a rare case of a 5 year old boy presenting with complaints of epistaxis. On anterior rhinoscopy of the nose, no apparent mass could be visualised. However, on oral examination, a right sided oropharyngeal bulge was noted. Clinical evidence was corroborated radiologically with MRI neck findings which suggested a hyperintense lesion with heterogeneous enhancement in the region of right side of nasopharynx extending laterally into parapharyngeal region and carotid space, encasing the right internal carotid artery with narrowing of nasopharyngeal airway. Hence, biopsy was taken from the nasopharynx and a diagnosis of embryonal rhabdomyosarcoma - botryoid type was made which was confirmed with IHC markers.
胚胎性横纹肌肉瘤是未分化骨骼肌的恶性间叶组织增殖。它是儿童中最常见的软组织肿瘤。头颈部发病占病例的40%,最常见的类型为胚胎型。鼻咽部横纹肌肉瘤是一种脑膜旁横纹肌肉瘤(起源于脑膜附近组织),易侵犯中枢神经系统,预后较差,早期复发,生长迅速且易侵犯相邻结构。其他类型的横纹肌肉瘤包括眼眶型和非眼眶/非脑膜旁型。在此,我们遇到一例罕见病例,一名5岁男孩因鼻出血前来就诊。前鼻镜检查未发现明显肿物。然而,口腔检查时发现右侧口咽有隆起。颈部MRI检查结果在影像学上证实了临床所见,提示鼻咽右侧区域有一高强度病变,强化不均匀,向外侧延伸至咽旁区域和颈动脉间隙,包绕右侧颈内动脉,导致鼻咽气道狭窄。因此,从鼻咽部取组织进行活检,诊断为葡萄状胚胎性横纹肌肉瘤,免疫组化标记物证实了该诊断。