Ijichi Kei, Muto Masahiro, Masaki Ayako, Murakami Shingo
Department of Otolaryngology-Head and Neck Surgery, Nagoya City University Graduate School of Medical Sciences, 467-8601 Nagoya, Japan.
Department of Radiology, Nagoya City University Graduate School of Medical Sciences, 467-8601 Nagoya, Japan.
Auris Nasus Larynx. 2018 Apr;45(2):358-361. doi: 10.1016/j.anl.2017.04.002. Epub 2017 May 6.
Plexiform schwannoma (PS) is a rare variety of benign nerve sheath tumor characterized by a multinodular plexiform growth pattern. PS is usually confined to the head and neck or skin. The pre-operative diagnosis of PS is difficult, and this has lead to a common misdiagnosis as a schwannoma. In addition, studies have indicated that an incomplete resection of PS often results in tumor recurrence. Here we describe a rare case of PS presented in the parapharyngeal space. Our case involved a 36-year-old man with swelling of the pharynx, who presented with a soft cervical mass. MRI revealed a multinodular mass in the left parapharyngeal space, and further pathological diagnosis by the referral hospital indicated schwannoma. A cervical approach was taken and the tumor was removed with preservation of the nerve sheath by intracapsular resection. The tumor recurred within one year after the first surgery in the same lesion of the left parapharyngeal space. The second surgical approach was a combination of a facial dismasking flap and trans-pterygopalatine fossa. The mass was resected completely, and the diagnosis of PS was confirmed by histopathology. While schwannoma commonly occurs in the head and neck, parapharyngeal space PS is rare, and pre-operative pathological diagnosis of PS is difficult. MRI studies of PS revealed distinctive features that we found useful in pre-operative diagnosis. Intracapsular resection of PS with nerve preservation has a very high recurrence rate of the tumor. Therefore, if MRI findings suggest PS we recommend removing the tumor completely without nerve preservation will offer the most curative outcome.
丛状神经鞘瘤(PS)是一种罕见的良性神经鞘瘤,其特征为多结节丛状生长模式。PS通常局限于头颈部或皮肤。PS的术前诊断困难,这导致其常被误诊为神经鞘瘤。此外,研究表明PS切除不完全常导致肿瘤复发。在此,我们描述一例发生于咽旁间隙的罕见PS病例。我们的病例是一名36岁男性,咽部肿胀,伴有颈部软组织肿块。磁共振成像(MRI)显示左侧咽旁间隙有一多结节肿块,转诊医院的进一步病理诊断为神经鞘瘤。采用颈部入路,通过囊内切除保留神经鞘完整的情况下切除肿瘤。首次手术后一年内,肿瘤在左侧咽旁间隙同一部位复发。第二次手术采用面部分离皮瓣联合经翼腭窝入路。肿块被完全切除,组织病理学确诊为PS。虽然神经鞘瘤常见于头颈部,但咽旁间隙PS罕见,且PS的术前病理诊断困难。PS的MRI研究显示出一些独特特征,我们发现这些特征对术前诊断很有用。保留神经进行PS囊内切除肿瘤复发率非常高。因此,如果MRI表现提示PS,我们建议不保留神经完整切除肿瘤将提供最佳治疗效果。