胸腺源性巨大纵隔脂肪肉瘤1例:罕见的临床病例

Debmalya Saha, Pawan K Singh, R. Sharma, S. Naqvi, Saket Aggarwal, M. Geelani
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摘要

胸腺肉瘤是一种极为罕见的胸腺肿瘤,迄今为止报道的病例很少。本病例报告一例45岁男性罕见胸腺瘤。CT增强示纵隔血管前腔室一大块肿块,以脂肪衰减为主,浸润纵隔内脏腔室两侧,向上延伸至双侧心膈角及肋膈前角,向右心室前方延伸,伴心包脂肪面消失,右侧气管旁及AP窗少量亚厘米淋巴结,右侧肺门淋巴结钙化。提示高分化脂肪肉瘤/胸腺肉瘤。CT引导下的原位组织活检未见明确结果,再次活检显示纤维胶原组织伴坏死区域,背景灶性黏液样改变,存在梭形至卵圆形细胞,核轻度多形性,S100、细胞角蛋白、CD34、desmin阴性。在没有体外循环(CPB)的支持下,在心包完整的情况下进行了细致的解剖,切除了整个肿瘤。手术活检标本的最终组织病理学报告与局灶神经节细胞分化的去分化胸腺肉瘤一致。术后随访胸部CECT未见血管前腔室残余肿块。患者术后接受30 Gy放射治疗(PORT),术后6个月无病无症状,放疗科常规随访。
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A Case of Giant Mediastinal Liposarcoma of Thymic Origin: A Rare Clinical Entity
Thymoliposarcoma is an exceedingly rare tumor of thymus with a very few cases reported till date. This case study presents a 45-year male with rare type of thymoma. On the contrast-enhanced CT images, there was a large mass lesion of predominantly fat attenuation in the pre-vascular compartment of the mediastinum insinuating on both sides of the visceral compartment of the mediastinum, and extending upto the bilateral cardio phrenic and anterior costophrenic angles, anterior to the right ventricle with loss of fat plane with the pericardium, with few sub-centimetric lymph nodes in the right paratracheal and AP window and a calcified right hilar lymph node, suggestive of well-differentiated liposarcoma/thymoliposarcoma. Initial CT guided tru-cut tissue biopsy was inconclusive, and the repeat biopsy revealed as fibro-collagenous tissue with area of necrosis, focal myxoid changes in the background with presence of cells which are spindle to oval in shape with mild nuclear pleomorphism and negative for S100, Cytokeratin, CD34, desmin. The entire tumor was resected en masse after meticulous dissection without the support of cardiopulmonary bypass (CPB) with an intact pericardium. Final histopathology report of the surgical biopsy specimens is consistent with dedifferentiated thymoliposarcoma with focal ganglionic cell differentiation. Postoperative follow-up CECT of thorax revealed no evidence of residual mass in the pre-vascular compartment. The patient is disease-free and asymptomatic after 6-month and he is under routine follow-up under Radiotherapy department since he received 30 Gy of postoperative radiotherapy (PORT).
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