El Zein Mohamad, Vali Reza, Charron Martin, Manson David, Perlman Kusiel, Shammas Amer
Department of Diagnostic Imaging, Division of Nuclear Medicine, The Hospital for Sick Children, University of Toronto, Toronto, ON, Canada.
J Pediatr Hematol Oncol. 2014 Jan;36(1):e1-4. doi: 10.1097/MPH.0b013e318286d2ba.
A rare case of possible primary ectopic adrenocorticotropic hormone (ACTH)-producing tumor in the liver mimicking a liver hemangioma is reported. A 9-year-old boy, with Cushing syndrome, was referred for the assessment of ectopic ACTH-producing tumor. Ultrasound, CT scan, and MRI of the abdomen revealed a liver lesion suggestive of a hemangioma. (111)In-octreotide scintigraphy revealed focal activity in the liver, indicative of a somatostatin-positive lesion. (99m)Tc-labeled RBC scintigraphy was negative for hemangioma. After surgical resection of the tumor, the cortisol level converted to a normal range indicative of a rare possible primary ACTH-producing tumor in the liver, which was confirmed by histopathology.
报告了一例罕见的可能起源于肝脏的原发性促肾上腺皮质激素（ACTH）分泌肿瘤，该肿瘤酷似肝血管瘤。一名患有库欣综合征的9岁男孩因评估异位ACTH分泌肿瘤前来就诊。腹部超声、CT扫描和MRI检查发现肝脏有一个提示为血管瘤的病变。（111）铟奥曲肽闪烁扫描显示肝脏有局灶性放射性，提示为生长抑素阳性病变。（99m）锝标记红细胞闪烁扫描对血管瘤呈阴性。肿瘤手术切除后，皮质醇水平恢复正常范围，提示肝脏中可能存在罕见的原发性ACTH分泌肿瘤，这一点经组织病理学证实。
