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成釉细胞瘤的异常影像学表现。

Unusual radiographic appearance of adamantinoma.

作者信息

Cappuccio Michele, Montalti Maurizio, Bosco Giuseppe, Gasbarrini Alessandro, Boriani Stefano

机构信息

Department of Orthopedics and Traumatology-Spine Surgery, Ospedale Maggiore C A Pizzardi, Bologna, Italy.

出版信息

Orthopedics. 2009 Dec;32(12):926. doi: 10.3928/01477447-20091020-22.

Abstract

Adamantinoma is a rare tumor with an indolent course that occurs most commonly in the tibia. It is locally aggressive, and local recurrences are described after resection. Pain is the most common symptom. Since the lesion is typically slow growing, the pain can be present for many years before the patient seeks medical attention. Microscopically, adamantinoma consists of islands of epithelial cells in a fibrous stroma. Nuclear atypia is minimal, and mitotic figures are rare. The most common radiographic appearance is that of multiple sharply demarcated radiolucent lesions surrounded by areas of dense, sclerotic bone. This tumor most often affects the tibial diaphysis and produces lytic lesions that can cause fractures. A 31-year-old man presented with a rapidly growing lytic lesion of the distal tibia. On histological examination, many areas of epithelial cells in a fibrous stroma were identified. Diagnosis of adamantinoma was performed. The lesion was treated with en bloc resection and reconstruction with distal tibia allograft and ankle arthrodesis with retrograde nail. At 2-year follow-up, there were no clinical or radiological signs of recurrence of disease.

摘要

造釉细胞瘤是一种罕见的肿瘤,病程进展缓慢,最常发生于胫骨。它具有局部侵袭性,切除术后有局部复发的报道。疼痛是最常见的症状。由于该病变通常生长缓慢,患者在寻求医疗关注之前,疼痛可能已存在多年。在显微镜下,造釉细胞瘤由纤维基质中的上皮细胞岛组成。核异型性极小,有丝分裂象罕见。最常见的影像学表现是多个边界清晰的透光性病变,周围环绕着致密的硬化骨区域。这种肿瘤最常累及胫骨骨干,产生溶骨性病变,可导致骨折。一名31岁男性出现胫骨远端快速生长的溶骨性病变。组织学检查发现,在纤维基质中有许多上皮细胞区域。诊断为造釉细胞瘤。该病变采用整块切除,并使用异体胫骨远端移植和逆行髓内钉进行踝关节融合重建。在2年的随访中,没有疾病复发迹象的临床或影像学表现。

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