Dedifferentiated Primary Parosteal Osteosarcoma of the Temporal Bone in a 19-Year-Old Patient: A Case Report.

Andro Košec, Domagoj Šarić, Hrvoje Čupić, Jakov Ajduk
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Abstract

Osteosarcoma is the most common primary malignant tumor affecting the bone but is a rare occurrence in the head and neck region. Complete surgical resection with wide surgical margins is currently the main treatment strategy for osteosarcoma but can be hard to achieve due to the complex anatomy of the head and neck. We report the first case of primary high-grade dedifferentiated parosteal osteosarcoma arising from the temporal bone in published literature. The 19-year-old patient presented with a left retroauricular lesion measuring 3 cm in diameter. Radiographic imaging and biopsy suggested the diagnosis of intermediate-grade chondrosarcoma, but definitive histopathology confirmed a diagnosis of dedifferentiated parosteal osteosarcoma. The tumor was resected with wide margins, removing the underlying temporal bone, periosteum and overlying soft tissue through a lateral temporal bone resection. The middle ear was reconstructed with cartilage grafting, and the dura of the posterior and middle cranial fossa was covered using temporal fascia grafts and local transpositional flaps. The patient is recurrence free 10 months after treatment. This report was assembled following CARE [The CARE guidelines (for Case Reports)] guidelines and describes clinical, histological, and radiological manifestations of our patient's rare clinical entity and may provide more data in treating patients with osteosarcoma affecting the anatomically complex head-and-neck region.

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一例19岁患者的原发性颞骨去分化腮腺骨肉瘤:病例报告。
骨肉瘤是影响骨骼的最常见的原发性恶性肿瘤,但在头颈部罕见。宽手术切缘的完全手术切除是目前骨肉瘤的主要治疗策略,但由于头颈部解剖结构复杂,很难实现。在已发表的文献中,我们报告了第一例由颞骨引起的原发性高级别去分化骨旁骨肉瘤。这名19岁的患者表现为左耳后病变,直径3厘米。影像学和活检提示诊断为中度软骨肉瘤,但最终的组织病理学证实诊断为去分化的骨旁骨肉瘤。肿瘤被切除,边缘较宽,通过颞侧骨切除术去除了下方的颞骨、骨膜和上方的软组织。用软骨移植重建中耳,用颞筋膜移植和局部移位皮瓣覆盖后中颅窝硬脑膜。患者在治疗后10个月内无复发。本报告是根据CARE[CARE指南(病例报告)]指南汇编的,描述了我们患者罕见临床实体的临床、组织学和放射学表现,并可能为治疗影响解剖复杂头颈部的骨肉瘤患者提供更多数据。
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