Hasegawa S L, Mentzel T, Fletcher C D
Department of Pathology, Brigham and Women's Hospital, Boston, Massachusetts 02115, USA.
Mod Pathol. 1997 Aug;10(8):777-84.
Approximately 45% of benign peripheral nerve sheath tumors occur in the head and neck region. Of these, schwannomas (neurilemomas) arising from the nasal cavity and paranasal sinuses account for less than 4%. Pathologic features of this subset are not well documented. We report a series of five cases of sinonasal schwannoma and one in the nasopharynx. The male-to-female ratio was equal, and the age at presentation ranged from 38 to 65 years of age (median, 52 yr). Four of the lesions were located within the nasal cavity, one arose from the maxillary sinus, and one originated in the nasopharynx, with extension into the Eustachian tube. Two cases showed local bony destruction, with intracranial extension. Presenting clinical symptoms included nasal obstruction, epistaxis, rhinorrhea, anosmia, facial swelling, headache, and serous otitis media; the two cases with intracranial spread also presented with visual disturbances. All of the six cases were treated by surgical excision. Clinical follow-up in five cases ranged from 6 to 48 months (median, 27 mo). Histologically, all of the lesions shared many cytomorphologic features common to schwannomas arising at other sites, and all of the six cases showed strong, diffuse immunoreactivity for S-100 protein. Four cases showed features of the cellular variant, and one showed focal granular cell change. An unusual and previously poorly documented histologic feature, distinct from schwannomas arising at most other anatomic sites, was a lack of encapsulation, which, when combined with hypercellularity, often raised suspicion of malignancy. Because none of the cases in this series has shown either local recurrence or postoperative metastasis to date, lack of encapsulation and locally destructive growth in an otherwise histologically typical schwannoma arising at this site should not suggest malignant potential.
约45%的良性周围神经鞘瘤发生于头颈部区域。其中,起源于鼻腔和鼻窦的施万细胞瘤(神经鞘瘤)占比不到4%。这一亚组的病理特征文献记载较少。我们报告了一系列5例鼻腔施万细胞瘤及1例鼻咽部施万细胞瘤。男女比例相同,发病年龄在38至65岁之间(中位数为52岁)。4个病灶位于鼻腔内,1个起源于上颌窦,1个起源于鼻咽部并延伸至咽鼓管。2例显示局部骨质破坏并伴有颅内扩展。主要临床症状包括鼻塞、鼻出血、流涕、嗅觉减退、面部肿胀、头痛和浆液性中耳炎;2例颅内扩散的病例还出现了视觉障碍。所有6例均通过手术切除治疗。5例的临床随访时间为6至48个月(中位数为27个月)。组织学上,所有病灶均具有许多其他部位施万细胞瘤共有的细胞形态学特征,6例均对S-100蛋白呈强弥漫性免疫反应。4例显示细胞型特征,1例显示局灶性颗粒细胞改变。一种不寻常且此前文献记载较少的组织学特征,与大多数其他解剖部位的施万细胞瘤不同,是缺乏包膜,这与细胞增多症相结合时,常引发对恶性肿瘤的怀疑。由于该系列病例至今均未显示局部复发或术后转移,因此在该部位出现的组织学典型施万细胞瘤中,缺乏包膜和局部侵袭性生长不应提示有恶性潜能。