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颅面区域的巨细胞骨病变:诊断和治疗挑战。

Giant cell bone lesions in the craniofacial region: a diagnostic and therapeutic challenge.

机构信息

University of São Paulo Medical School, Clinics Hospital USP, São Paulo, Brazil.

出版信息

Int Forum Allergy Rhinol. 2012 Nov;2(6):501-6. doi: 10.1002/alr.21050. Epub 2012 May 7.

Abstract

BACKGROUND

Giant cell tumors of bone (GCTs) are common in the long bones, but rare in the craniofacial region, with only 1% of cases occurring in the latter. Clinical, radiological, and anatomical diagnosis of this locally aggressive disease, which occurs in response to trauma or neoplastic transformation, poses a major challenge in clinical practice.

METHODS

The present study describes a series of 4 cases and highlights the main features of the differential diagnosis and treatment of these lesions: GCT, giant cell reparative granuloma (GCRG), and the brown tumor of hyperparathyroidism.

RESULTS

GCT presents as a benign neoplasm, most typically affecting the knees, and rarely in the temporal and sphenoid bones. It is radiologically indistinguishable from GCRG due to its lytic, poorly defined appearance. The distinction can only be made microscopically, as the presence of multinucleated giant cells scattered throughout the stroma and the absence of a history of trauma favor a diagnosis of GCT. The brown tumor of hyperparathyroidism occurs with rapid, localized osteoclast activity secondary to the effects of increased parathyroid hormone (PTH) levels; parathyroid examination is indispensable.

CONCLUSION

The diagnosis and treatment of these lesions poses a major challenge due to their similar clinical presentation and radiological appearance. Accurate diagnosis is essential for definition of appropriate management, as complete resection is the goal in GCT and GCRG to avoid recurrence, whereas the brown tumor often yields to treatment of the underlying hyperparathyroidism.

摘要

背景

骨巨细胞瘤(GCT)常见于长骨,但在颅面区域罕见,仅有 1%的病例发生在后者。这种局部侵袭性疾病,发生于创伤或肿瘤转化后,其临床表现、影像学和解剖学诊断在临床实践中构成了重大挑战。

方法

本研究描述了一系列 4 例病例,并强调了这些病变的鉴别诊断和治疗的主要特征:GCT、巨细胞修复性肉芽肿(GCRG)和甲状旁腺功能亢进的棕色瘤。

结果

GCT 表现为良性肿瘤,最常影响膝关节,很少发生在颞骨和蝶骨。由于其溶骨性、边界不清的表现,GCT 在影像学上与 GCRG 无法区分。只有通过显微镜才能做出区分,因为存在散布在基质中的多核巨细胞且无创伤史提示 GCT 的诊断。甲状旁腺功能亢进的棕色瘤是由于甲状旁腺激素(PTH)水平升高导致破骨细胞快速、局部性活性增加而发生的;甲状旁腺检查是必不可少的。

结论

由于这些病变的临床表现和影像学表现相似,因此诊断和治疗构成了重大挑战。准确的诊断对于确定适当的治疗方案至关重要,因为在 GCT 和 GCRG 中,完全切除是避免复发的目标,而棕色瘤通常可以通过治疗潜在的甲状旁腺功能亢进得到缓解。

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