儿童病毒性肾上腺腺瘤

Anjali Verma, Yogender Singh Kadian, Pradeep Kajal, Md Mokarram Ali, Kusum Lata, Surender Verma
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摘要

摘要:肾上腺皮质肿瘤最常见于成年人,但儿童罕见。临床表现取决于患者的年龄和性别;约三分之二的病例以男性化为主要表现,而许多病例同时具有男性化和库欣样特征。诊断需要通过激素评估和放射学检查来确认。手术切除是治疗的主要方法。儿科患者的预后通常较好,而旧文献中报道的高死亡率可能是由于肿瘤较大、术后并发症和类固醇替代不足造成的。在此,我们介绍了三例儿童期肾上腺皮质肿瘤所致男性化特征的病例。所有患者都主要表现为男性化特征。所有患者均接受了手术治疗,尽管其中一名患者的肿瘤较大,但手术效果良好。组织学检查显示肿瘤为肾上腺腺瘤形式的良性病变。多数男性化和类库欣特征在随访(中位数为 1 年)期间消失。虽然这类肿瘤很少见,但如果儿童伴有类库欣特征、男性化或两者兼而有之,则应高度怀疑。即使肿瘤体积较大,充分的类固醇替代和术后支持性治疗也能使我们的患者获得良好的预后。
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Virilising Adrenal Adenoma in Children.

Abstract: Tumours of the adrenal cortex are most commonly seen in adults but rare in children. The clinical manifestations depend on the age and sex of patients; about two-thirds of the cases virilisation is the predominant presentation, whereas many presents with both virilisation and cushingoid features. Diagnosis is confirmed by hormonal evaluation and radiological investigations. Surgical removal is the mainstay of treatment. Usually, there is a good prognosis in paediatric patients, whereas the high mortality rate reported in older literature may have been due to big tumour size, post-operative complications and inadequate steroid replacement. Here, we are presenting a series of three cases during childhood with virilising features due to adrenocortical tumours. All of them presented predominantly with features suggestive of virilisation. All patients underwent surgery and had a good outcome despite big tumour size in one of the patients. Histology revealed a benign lesion in the form of adrenal adenoma. Most virilisation and cushingoid features disappeared in the follow-up (median - 1 year). Although these tumours are rare, a high index of suspicion should be kept in children with cushingoid features, virilisation or a combination of both of them. Even if the tumour size is big, adequate steroid replacement and supportive management postoperatively have led to good prognosis in our patients.

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