颅内血管周细胞瘤的影像学特点及显微外科手术治疗

目的探讨颅内血管周细胞瘤(HPC)的影像学特点及显微外科手术方法。方法回顾性分析显微外科手术治疗的26例颅内HPC患者的临床资料;包括影像学检查、手术方法及手术疗效。结果16例颅内HPC患者术前影像学诊断与术后病理诊断一致,6例患者术前被误诊为脑膜瘤,4例患者误诊为血管母细胞瘤。手术效果:肿瘤全切除者17例,次全切除5例,部分切除4例,无死亡患者。全部患者术后均行局部放疗。术后随访6~42个月,3例患者肿瘤复发。结论颅内HPC的影像学表现具有一定特点,仔细分析其影像学特点可提高术前诊断率。根据肿瘤的影像学表现制定手术方案,以及娴熟的显微外科技术,术中控制好出血,是提高颅内HPC全切除率和提高手术疗效的关键。     

脑膜血管周细胞瘤临床病理分析

目的:探讨脑膜血管周细胞瘤(hemangiopericytoma,HPC)的临床病理特点及鉴别诊断.方法:对9例脑膜HPC患临床病理资料进行回顾性分析和随访15~95个月,并复习相关文献.结果:脑膜HPC常见症状为头痛头昏、肿瘤压迫和颅内高压;CT/MRI检查见肿瘤与脑膜相连,增强扫描明显强化.光镜下肿瘤细胞丰富,核圆形或卵圆形,核仁不明显;间质富含"鹿角状"血管,网状纤维包绕单个瘤细胞.免疫标记瘤细胞CD34局灶阳性,PR阴性,1例EMA局灶弱阳性.7例随访15~95个月,4例术后复发,复发病例中1例术后35月腰椎转移.结论:脑膜HPC是一种少见肿瘤,确诊需依靠病理学检查.脑膜HPC影像学上与脑膜瘤相似,但其生物学行为、病理组织学及预后不同于脑膜瘤,易复发和远处转移,术后需长期随访.     

枕骨大孔-第二颈段血管周细胞瘤1例报道并文献复习

目的:探讨血管周细胞瘤的临床病理学特点及诊断要点.方法:对1例血管周细胞瘤进行临床资料、病理形态学及免疫组化观察,并结合文献对其诊断及鉴别诊断进行探讨.结果:本例镜下观肿瘤细胞为梭形,血管丰富,形成弥漫的网状结构,鹿角状的血管将肿瘤细胞分割小叶状,瘤细胞体积较大,胞浆丰富,边界不清,核呈圆形、卵圆形,病理性核分裂相多.免疫组化染色结果显示肿瘤细胞Vimentin(+)、CD34(+)、FVIII(+)、S-100(?)、CEA(?)和GFAP(?).结论:血管周细胞瘤,非常少见,因此缺乏对其认识,从而易与其他肿瘤混淆导致误诊.提高对血管周细胞瘤的认识,对避免误诊是至关重要的.     

Low-grade Sarcomas with CD34-Positive Fibroblasts and Low-Grade Myofibroblastic Sarcomas

A subset of low-grade fibrosarcomas is composed of CD34-positive spindle cells. These include dermatofibrosarcoma, its morphologic variants, and its associated fibrosarcoma, solitary fibrous tumor, hemangiopericytoma and their malignant counterparts, and some cases of myxoinflammatory fibroblastic sarcoma. Dermatofibrosarcoma and related lesions are characterized by a t(17;22)(q22;q13) rearrangement resulting in fusion of the genes COL1A (17q21-22) and PDGFB1 (22q13). Solitary fibrous tumor displays varying cellularity and fibrosis and a peripheral hemangiopericytomatous pattern; most tumors formerly called hemangiopericytoma are now subsumed into the category of solitary fibrous tumor, although a few strictly defined examples are recognized; however, these are probably not composed of pericytes. Myofibroblastic malignancies are best identified by electron microscopy, with which varying degrees of differentiation, including the presence of fibronexus junctions, can be identified. Low-grade sarcomas showing myofibroblastic differentiation include myofibrosarcomas and inflammatory myofibroblastic tumors. Myofibrosarcomas are spindle cell neoplasms that occur in children or adults in the head and neck, trunk, and extremities as infiltrative neoplasms and that display a fascicular or fasciitis-like pattern with focal nuclear atypia and variable expression of myoid antigens. These sarcomas are prone to recurrence and a small number metastasize. Inflammatory myofibroblastic tumor (synonymous with inflammatory fibrosarcoma) is a neoplasm arising predominantly in childhood, and frequently in intraabdominal locations. It has spindle cells in fascicular, fasciitis-like and sclerosing patterns, with heavy chronic inflammation including abundant plasma cells. Many IMT have clonal chromosomal abnormalities involving 2p22-24, and fusion of the ALK gene with tropomyosin 3 (TPM3-ALK) or tropomyosin 4 (TPM4-ALK) is found in a subset.     

Liver Recurrence of a Subcutaneous Temporal Hemangiopericytoma: The Index Case

Introduction  Hemangiopericytoma is an uncommon soft tissue vascular neoplasm. Intraperitoneal hemangiopericytomas such as primary or secondary liver location have been exceptionally described. Its natural history is mostly benign, but recurrences may occur and determining if these late-discovered tumors are distant metastases or synchronous slow and silent-growing locations is sometimes challenging. The histopathological diagnosis and definition of hemangiopericytoma is based on its distinction with solitary fibrous tumors. Liver resection to treat liver hemangiopericytoma seems to be supported by various published experiences. Case presentation  We herein report the first case of liver metastases from a subcutaneous temporal hemangiopericytoma. The patient was treated by a liver resection. CD34 Immunostaining was negative, but strong expression of Bcl2 and CD99 was found in the neoplastic cells. After 1 year of follow-up, the patient is alive without recurrence. Conclusion  To date, published data, including the case herein reported, support the need for a prolonged follow-up and the role of liver resection to treat liver metastases of hemangiopericytomas. Complete resection of all gross disease appears to be the most significant prognostic factor.     

Mediastinal hemangiopericytoma

Mediastinal hemangiopericytoma (HPC) was diagnosed in a 3‐year‐old female. The incidence of this tumor is rare in children, and few data are available to guide clinical management. The surgical resection was incomplete and she received adjuvant radiation therapy and chemotherapy. The patient is alive without adverse events 6 years after diagnosis. Pediatr Blood Cancer 2009;53:206–207. © 2009 Wiley‐Liss, Inc.     

Retroperitoneal Sarcoma with Features Suggestive of Alveolar Soft Part Sarcoma

A 72-year-old man presented with a 15 × 12 × 5 cm cystic and necrotic tumor involving the superior region of the stomach and the retroperitoneum down to the transverse colon and extending laterally from the left lobe of the liver to the spleen without involving either organ. Light microscopy showed the tumor cells to be arranged in an organoid pattern. Immunocytochemistry showed a strong reaction for desmin and vimentin. Electron microscopy demonstrated features suggestive of an alveolar soft part sarcoma.     

下颌骨恶性血管内皮与外皮瘤的临床病理观察(附4例报告)

本文根据作者经治发生于下颌骨的4个恶性血管内皮与外皮瘤病例，结合文献复习，探讨其临床病理特点、诊断治疗与预后等问题。发现该病发生于颌骨者十分罕见，临床易发生误诊，因此，临床医生遇有颌骨肿块生长迅速，疼痛呈持续性且逐渐加重，X线片提示有溶骨性破坏，诊断也应考虑患本病的可能，尽早手术治疗，并于术中行冰冻活检协诊。一般认为本病预后差，但如组织学形态分化较好，治疗及时，切除范围适当，也可获得较佳疗效。