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广泛侵袭性蝶窦、海绵窦及垂体窝孤立性纤维瘤。

Widely invasive solitary fibrous tumor of the sphenoid sinus, cavernous sinus, and pituitary fossa.

作者信息

Cassarino David S, Auerbach Aaron, Rushing Elisabeth J

机构信息

Laboratory of Pathology, National Cancer Institute, National Institute of Health, Bethesda, MD 20892, USA.

出版信息

Ann Diagn Pathol. 2003 Jun;7(3):169-73. doi: 10.1016/s1092-9134(03)00016-9.

DOI:10.1016/s1092-9134(03)00016-9
PMID:12808569
Abstract

Solitary fibrous tumor is a spindle cell tumor first described in the pleura, but also found in multiple extrathoracic sites including the meninges, orbit, nasal and paranasal sinuses. No cases have been previously reported in the cavernous sinus or pituitary fossa. We present the case of a 54-year-old woman who presented with progressive amaurosis. On imaging studies, a widely infiltrative lesion involving the pituitary fossa, sphenoid sinus, cavernous sinus, carotid artery, medial temporal, ethmoid, and pterygoid bones, and extending into the nasopharynx was discovered; impression was a malignant tumor originating in the pituitary fossa. At surgery, the tumor was only partially resectable because of extensive bony invasion and encasement of the carotid artery, and was found to compress but not invade the pituitary gland. Histology showed a spindle cell proliferation with a dense, hyalinized collagenous stroma and dilated vascular spaces, some showing a staghorn-like appearance. Areas of cellular pleomorphism and increased cellularity were present, but few mitoses were identified. Immunohistochemistry showed strong positivity with CD34, factor XIIIa, CD99, and Bcl-2. There was scattered cyclin D1, mib-1, and p53 positivity. Muscle, epithelial, vascular, and melanocytic markers were negative. These results led to the diagnosis of solitary fibrous tumor. The size, extensive invasion, and bony destruction indicated a tumor with at least low malignant potential. The occurrence of solitary fibrous tumors in the pituitary fossa and sinuses of the head and neck is rare, but must be considered in the differential diagnosis of spindle cell lesions in these regions.

摘要

孤立性纤维瘤是一种梭形细胞瘤,最初在胸膜中被描述,但也见于多个胸外部位,包括脑膜、眼眶、鼻腔和鼻窦。此前尚无在海绵窦或垂体窝发现此类病例的报道。我们报告一例54岁女性患者,该患者出现进行性黑蒙。影像学检查发现一个广泛浸润性病变,累及垂体窝、蝶窦、海绵窦、颈动脉、颞内侧、筛骨和翼状骨,并延伸至鼻咽部;初步诊断为起源于垂体窝的恶性肿瘤。手术中,由于肿瘤广泛侵犯骨质并包绕颈动脉,仅能部分切除,且发现肿瘤压迫但未侵犯垂体。组织学检查显示梭形细胞增生,伴有致密、玻璃样变的胶原性间质和扩张的血管间隙,部分呈鹿角样外观。存在细胞多形性区域和细胞增多现象,但仅见少数核分裂象。免疫组化显示CD34、因子ⅩⅢa、CD99和Bcl-2呈强阳性。有散在的细胞周期蛋白D1、mib-1和p53阳性。肌肉、上皮、血管和黑素细胞标记物均为阴性。这些结果诊断为孤立性纤维瘤。肿瘤的大小、广泛侵犯和骨质破坏提示其至少具有低恶性潜能。孤立性纤维瘤发生于垂体窝及头颈部鼻窦罕见,但在这些区域梭形细胞病变的鉴别诊断中必须予以考虑。

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