Radiology & Biomedical Imaging, Yale University School of Medicine, New Haven, CT, USA.
Department of Pathology, Yale University School of Medicine, New Haven, CT, USA.
Yale J Biol Med. 2021 Dec 29;94(4):593-597. eCollection 2021 Dec.
: Glomangiopericytoma (GPC) is a rare tumor in the nasal cavity or paranasal sinuses with low malignant potential. Initially deemed a hemangiopericytoma, in 2005 it was classified as a distinct entity by the World Health Organization (WHO). : A male patient in his early 60s presented with new-onset right arm and leg weakness/numbness, who was incidentally found to have a left ethmoid sinus mass with extension in the olfactory fossa. On CT and MRI, the mass enhanced with well-defined borders and eroded the bone, but without dural enhancement. The mass was surgically excised, and pathology confirmed the diagnosis of glomangiopericytoma by microscopic appearance and staining. : Glomangiopericytoma has less than 0.5% incidence of all neoplasms of the sinonasal cavity, making it rare. Most diagnosed patients are in their 6th or 7th decade of age, with a slight female predominance. Treatment is complete surgical excision, with excellent prognosis, although there is up to 17% local recurrence. Despite the non-specific appearance on CT and MRI, imaging can help provide differential diagnosis, tumor extent, size, and reassuring non-aggressive characteristics of the tumor prior to surgery. GPC tumors are relatively resistant to radiation and chemotherapy. : It is important to recognize glomangiopericytoma in the differential of masses of the nasal cavities or paranasal sinuses, as they rarely warrant aggressive treatment beyond local excision. Each reported case of glomangiopericytoma helps to build guidance for imaging and treatment since GPC is rare and not well-represented in the medical literature.
: 血管外皮细胞瘤(GPC)是一种罕见的鼻腔或鼻窦肿瘤,恶性潜能低。最初被认为是血管外皮细胞瘤,2005 年被世界卫生组织(WHO)分类为一种独特的实体肿瘤。 : 一位 60 多岁的男性患者出现新发右侧手臂和腿部无力/麻木,偶然发现左侧筛窦肿块并延伸至嗅沟。在 CT 和 MRI 上,肿块边界清晰增强,并侵蚀骨组织,但无硬脑膜增强。肿块通过手术切除,病理通过显微镜观察和染色证实了血管外皮细胞瘤的诊断。 : 血管外皮细胞瘤在鼻腔鼻窦的所有肿瘤中发病率低于 0.5%,非常罕见。大多数诊断患者年龄在 60 或 70 岁左右,女性略占优势。治疗方法是完全手术切除,预后良好,尽管局部复发率高达 17%。尽管 CT 和 MRI 上的表现不具有特异性,但影像学检查可以帮助提供鉴别诊断、肿瘤范围、大小,并在手术前提供肿瘤非侵袭性特征的保证。GPC 肿瘤对放疗和化疗具有一定的耐药性。 : 在鼻腔或鼻窦肿块的鉴别诊断中,认识血管外皮细胞瘤非常重要,因为它们很少需要除局部切除以外的积极治疗。由于 GPC 罕见且在医学文献中代表性不足,因此每例报道的血管外皮细胞瘤病例都有助于为影像学和治疗提供指导。