Chondrosarcoma Of Orbit Research Articles

Orbito-cranial mesenchymal chondrosarcoma in a young female: A rare case report

Mesenchymal chondrosarcoma (MCS) of the head and neck is highly aggressive malignant small round cell neoplasm with cartilaginous differentiation, often with a pericytomatous vascular pattern. It represents approximately 0.1% of all head and neck neoplasms. Chondrosarcoma of orbit is extremely rare. We report a case of orbito-cranial MCS in a 24-year-old female who presented with exophthalmos. Imaging revealed a large heterogenous lesion arising from right orbit with intracranial extension. Patient underwent right fronto-temporal craniotomy with tumor decompression from intracranial part and right orbit. Histopathologic examination and immunohistochemistry was suggestive of MCS. Postoperative residual disease was treated with chemotherapy showing partial response.

Orbital chondrosarcoma arising from paranasal sinuses

Orbital chondrosarcoma arising from paranasal sinuses or from the nasal cavity with orbital extension is highly unusual. The aim of this report is to describe our multidisciplinary experience in the treatment of three patients with extensive sino-orbital chondrosarcomas. The patients were aged 43, 75 and 63years, and the tumors originated from the maxillary, sphenoidal, and ethmoidal sinuses, respectively. Magnetic resonance imaging studies showed homogeneously hypointense lesions on T1-weighted scans, which were hyperintense on T2-weighted images. Intranasal endoscopic surgery alone or in combination with other conventional surgical approaches was the main therapeutic choice. Two patients had recurrences treated with wider surgical removal. Fractionated stereotactic radiotherapy was used in two cases. Two patients had histopathological grade I tumor and one had grade III chondrosarcoma. No patient had regional spread or distant metastasis. All patients were disease-free at 4, 2, and 3years, respectively, following their last therapeutic interventions. Endoscopic surgery results in lesser morbidity for chondrosarcomas arising from paranasal sinuses and extending into posterior parts of the orbit. However, because control of surgical margins may not be complete, recurrences may occur justifying more radical approaches.

Primary Orbital Mesenchymal Chondrosarcoma: Case Report and Review of the Literature

Orbital mesenchymal chondrosarcoma is a very uncommon lesion of the bone and extraskeletal tissue. To our knowledge, approximately 30 cases have been described. We present the case of a 52-year-old male who presented with a history of progressive proptosis and chemosis of the right eye caused by an orbital tumor. He underwent exenteration of the right orbit, and the histological examination revealed a mesenchymal orbital chondrosarcoma. This paper attempts to describe a rare entity that should be considered in the differential diagnosis of calcified orbital lesions, especially in young adults. Complete removal of the tumor is the mainstay of treatment, but adjuvant radiation therapy and chemotherapy should be considered.

A case of primary orbital chondrosarcoma

The case of a twenty-year-old male with orbital chondrosarcoma is reported. He visited National Defense Medical College Hospital because of reduced vision in the right eye since two months previously. His corrected visual acuity was 8/20 in the right eye and 20/20 in the left eye. Fifteen degrees lateral displacement of the right globe and limitation of right ocular movement were recognized. Right fundus examination revealed optic disc edema and protuberant nasal fundus. Orbital computed tomography (CT) demonstrated a high density area between the inner part of the right orbit and the ethmoid sinus. Magnetic resonance imaging (MRI) showed a smoothly outlined and low intensity (T1) space occupying lesion. This lesion was irregularly enhanced by gadolinium-diethylene triamine pentaacetic acid (Gd-DTPA). This orbital tumor was removed by an anterior approach. Histopathological examination revealed well-differentiated chondrosarcoma (grade 1) as determined by small prominent chondromatous cell projection into the collagen fibrous stroma, and existence of binucleate cells in the hypercellular region. After the operation the disc edema disappeared and his corrected right visual acuity improved to 20/20.

CT and MRI diagnosis of chondrosarcoma in sinonasal and orbital region

目的 探讨鼻眶部软骨肉瘤的CT和MRI表现,提高其诊断准确性.方法回顾性分析12例经病理证实的鼻眶部软骨肉瘤的影像学资料.结果12例中起源于鼻腔4例,鼻窦5例,眼眶3例.普通型软骨肉瘤8例,去分化型和间叶型软骨肉瘤各2例.CT表现:肿瘤呈卵圆形2例,分叶形6例,不规则形4例;内见散在点、环、结节、斑片状或不定型钙化;边界清楚9例,模糊3例;3例增强后呈不均匀低度强化.7例鼻软骨肉瘤出现骨质破坏,并侵犯周围结构;眼眶软骨肉瘤骨质破坏1例,并侵犯同侧额部.MRI表现:普通型和去分化型软骨肉瘤在T1WI上表现为低信号6例(与脑组织比较,以下同),等信号4例,T2WI表现为高信号8例,等信号2例,信号不均匀,内见散在低信号区,增强后呈轻到中度不均匀强化,其中5例普通型软骨肉瘤边缘及间隔呈明显强化,内部不强化,呈斑驳状3例,蜂窝状2例;间叶型软骨肉瘤在T1、T2WI均呈等信号,增强后呈均匀和不均匀显著强化各1例.结论CT是鼻眶部软骨肉瘤诊断和鉴别的主要影像检查方法;典型间隔及边缘强化的MRI表现也能够提示诊断,MRI可更清楚、准确地显示病变侵犯的范围,为治疗提供可靠依据。

Orbital chondrosarcoma developing in a patient with Paget disease

PURPOSE: To describe the radiologic, histopathologic, and cytogenetic features of an orbital chondrosarcoma developing in a patient with Paget disease. METHODS: A 64-year-old woman presented with rapidly progressive proptosis of her right eye. Computed tomographic scans, histopathologic examination, and cytogenetic analysis were performed. RESULTS: Computed tomographic scans disclosed osseous changes of the temporal and frontal bones, with areas of high density consistent with Paget disease. A soft-tissue tumor in the right lateral orbital wall was consistent with Paget sarcoma. On histology, a chondrosarcoma was diagnosed, which was confirmed by fluorescent in situ hybridization. CONCLUSION: This is a unique case of orbital chondrosarcoma developing in a patient with Paget disease.