Pediatric Surgery Department. Valladolid Clinical Hospital (Spain)..
Pediatric Surgery Department. Valladolid Clinical Hospital (Spain).
Cir Pediatr. 2021 Apr 1;34(2):90-94.
Carotid glomus is an exceptional extra-adrenal paraganglioma in childhood originating at the carotid body. Only 3% of paragangliomas occur in the head and the neck. Familial forms, associated with Succinate Dehydrogenase (SDH) gene mutations, account for 10% of cases, the proportion being higher in childhood. They are benign in 95% of patients, but they can extend to both carotids. Treatment is surgical with or without previous embolization. Metastasis is rare and associated with malignant cases, which are limited.
8-year-old patient with a cervical mass originating 4 months ago and normal serum levels. Regarding family history, she had an aunt who underwent cervical surgery. Ultrasound examination demonstrated a greatly vascularized hypoechoic mass most likely related to carotid glomus. Full surgical resection without embolization was decided upon, which proved uneventful. The genetic study was positive for SDH gene mutation.
Carotid glomus in childhood should be considered as a differential diagnosis in cervical masses. Surgical treatment without previous embolization represents a safe therapeutic option in selected cases.
颈动脉球是一种罕见的起源于颈动脉体的儿童期肾上腺外副神经节瘤。仅 3%的副神经节瘤发生在头部和颈部。家族性形式与琥珀酸脱氢酶(SDH)基因突变相关,占病例的 10%,在儿童中比例更高。95%的患者为良性,但可向两侧颈动脉扩展。治疗方法是手术治疗,可选择或不选择栓塞。转移罕见,与恶性病例相关,且较为局限。
8 岁患者,4 个月前出现颈部长肿块,血清水平正常。家族史方面,患者有一位曾接受过颈部手术的阿姨。超声检查显示一个高度血管化的低回声肿块,最可能与颈动脉球有关。决定行完整的无栓塞手术切除,结果顺利。基因研究显示 SDH 基因突变阳性。
儿童期颈动脉球应作为颈部长肿块的鉴别诊断。在选择的病例中,无栓塞的手术治疗是一种安全的治疗选择。