CAJ | 학술논문

目的分析肾上腺原始神经外胚层肿瘤(PNET)的临床及病理特点. 方法肾上腺PNET患者4例.男2例,女2例.平均年龄24(21、22、24、30)岁.常规实验室及内分泌检查无阳性发现.B超、CT检查示肾上腺区边界欠清的巨大囊实性软组织肿块,直径平均12(8～17)cm. 结果 1例穿刺活检确诊后拒绝治疗,6个月后死亡;1例手术探查后行减瘤术,术后8个月死亡;行肿瘤根治性切除治疗2例,术后1个月出现远处转移1例,肿瘤局部复发1例,4例病理学检查光镜下可见典型的Homer-Wright菊形团,免疫组织化学染色CD99阳性.病理诊断均为PNET. 结论肾上腺PNET是一种罕见的原始神经外胚叶来源肿瘤,好发于青年,临床及影像学无特殊表现,确诊依据病理学检查.肿瘤恶性度高,进展迅速,预后极差.
목적 분석신상선원시신경외배층종류(PNET)적림상급병리특점. 방법신상선PNET환자4례.남2례,녀2례.평균년령24(21、22、24、30)세.상규실험실급내분비검사무양성발현.B초、CT검사시신상선구변계흠청적거대낭실성연조직종괴,직경평균12(8～17)cm. 결과 1례천자활검학진후거절치료,6개월후사망;1례수술탐사후행감류술,술후8개월사망;행종류근치성절제치료2례,술후1개월출현원처전이1례,종류국부복발1례,4례병이학검사광경하가견전형적Homer-Wright국형단,면역조직화학염색CD99양성.병리진단균위PNET. 결론 신상선PNET시일충한견적원시신경외배협래원종류,호발우청년,림상급영상학무특수표현,학진의거병이학검사.종류악성도고,진전신속,예후겁차.
Objective To analyze the clinical and pathological features of the adrenal primitive neuroectodermal tumors(PNET). Methods Four cases of PNET were analyzed.Of them,2 were males and 2 females,aged from 21 to 30 years old with a mean age of 24.No significant abnormal data was found in routine laboratory and endocrine examinations.The CT scan showed a soft tissue mass with unclear boundary and cystic changes in adrenal area.The tumor diameter was 8-17 cm. Results One patient refused treatment alter diagnosed by biopsy and died 6 months later.Another one received palliative operationand died after 8 months.The third patient was found distant metastasis 1 month after operation and had radiotherapy and chemotherapy.The fourth patient was found local tumor recurrence 1 month after operation,and started chemotherapy.All patients were diagnosed by pathology.At HE staining,tumor was consisted of even,uniform small round cells;the cells distributed diffusively or formed lobulated structures (Homer-Wright rosette).Immunohistochemical staining showed CD99 positive in all 4 patients. Canclusion The adrenal PNET is a rare disease originated from primitive neuroectodermal,mostly occurs between 20 and 30 years old,and has non-specific clinical and imaging findings.Histopathologieal examination is the key point for diagnosis.Rapid progression,highly malignant,poor prognosis are the characteristics of this disease.