Department of Plastic and Reconstructive Surgery, Seoul St. Mary's Hospital, College of Medicine, The Catholic University of Korea, Seocho-gu.
Department of Hospital Pathology, Eunpyeong St. Mary's Hospital, College of Medicine, The Catholic University of Korea, Eunpyeong-gu, Seoul, Republic of Korea.
World Neurosurg. 2020 Sep;141:8-13. doi: 10.1016/j.wneu.2020.05.189. Epub 2020 May 28.
Central nervous system (CNS) solitary fibrous tumor/hemangiopericytoma (SFT/HPC) is a soft tissue neoplasm that accounts for <1% of all intracranial tumors. Its growth will be mostly intracranial, usually along the sinuses. We have reported a rare case of direct extracranial extension of CNS SFT/HPC penetrating the frontal bone.
A 64-year-old woman had visited our institution for treatment of a forehead mass. With the impression that it was a subcutaneous mass, we had planned a simple excision with the patient under local anesthesia. However, the intraoperative findings showed extension of the mass into the skull and attachment to the dura mater. Brain magnetic resonance imaging showed a 1.8-cm solid mass with an adjacent skull defect, and examination of the biopsy specimen confirmed the diagnosis as grade II CNS SFT/HPC. Definitive excision was performed by en bloc tumor resection with a 2-cm safety margin. Adjuvant radiotherapy was performed with 60 Gy in 30 fractions. The patient showed no signs of recurrence or metastasis during 2 years of follow-up.
The present case has shown that CNS SFT/HPC can infiltrate the full thickness of the skull bone and grow extracranially, even if low grade. However, the unusual presentation of the present made early exploration and total resection possible.
中枢神经系统（CNS）孤立性纤维瘤/血管外皮细胞瘤（SFT/HPC）是一种软组织肿瘤，占所有颅内肿瘤的<1%。其生长主要位于颅内，通常沿窦腔。我们报告了一例罕见的 CNS SFT/HPC 直接颅外延伸穿透额骨的病例。
一名 64 岁女性因额部肿块就诊于我院。我们的初步印象是皮下肿块，计划在局部麻醉下进行简单切除。然而，术中发现肿块向颅骨内延伸并与硬脑膜相连。脑磁共振成像显示 1.8 厘米的实性肿块，伴有邻近颅骨缺损，活检标本检查证实诊断为 II 级 CNS SFT/HPC。通过 2 厘米安全边界的整块肿瘤切除术进行明确切除。采用 60 Gy/30 次分割的辅助放疗。在 2 年的随访中，患者无复发或转移迹象。
本病例表明 CNS SFT/HPC 可穿透颅骨全层并向颅外生长，即使是低度恶性。然而，本病例的不典型表现使得早期探查和完全切除成为可能。
