原发性肾上腺血管肉瘤。个案报告

Patricia Castellanos Donoso, Mario Paul Sánchez Pérez, Carlos Alberto Cárdenas Aguirre, Adriana Ortiz Barbosa, Carmen M. Galván Gómez, Adrián Murube Jimenez, Severino Rey Nodar
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引用次数: 1

摘要

原发性肾上腺血管肉瘤是一种罕见的血管恶性肿瘤,文献中报道的病例不足50例,多见于中年男性,预后较差,中位生存期约为18个月,5年总生存率为30%。一例61岁的男性患者谁咨询疼痛在左肾窝放射到同侧侧腹和体质综合征。诊断性影像学检查显示左侧肾上腺大实性肿块,未见局部肿瘤扩展或其他器官的征象;实验室研究证实皮质醇的自动生成与可能的副肿瘤消耗性凝血病有关。经解剖病理诊断为原发性肾上腺上皮样血管肉瘤及肝转移,行左侧肾上腺切除术。术后立即发现手术部位出现血肿,保守治疗进展良好。随后,给予紫杉醇辅助化疗治疗,在手术后5周的诊断影像学检查中显示转移进展。死亡出口,术后三个月出现。
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Angiosarcoma suprarrenal primario. Reporte de caso
Primary adrenal angiosarcoma is a rare vascular malignancy with fewer than fifty cases reported in the literature, with a preference for middle-aged men and a poor prognosis with a median survival of around eighteen months and a five-year overall survival of 30 %. A case of a 61-year-old male patient who consulted for pain in the left renal fossa radiating to the ipsilateral flank and constitutional syndrome is reported. Diagnostic imaging tests show a large solid left adrenal mass with no signs of local tumor extension or in other organs; Laboratory studies confirm autoproduction of cortisol and association with probable paraneoplastic consumptive coagulopathy. Open left adrenalectomy was performed with an anatomopathological diagnosis of primary epithelioid angiosarcoma of the adrenal gland and liver metastases. During the immediate postoperative period, hematoma of the surgical site was evidenced, which was treated conservatively with favorable evolution. Subsequently, adjuvant chemotherapy treatment with paclitaxel was administered, presenting metastatic progression in diagnostic imaging tests five weeks after surgery. Exitus letalis, presented three months after surgery.
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