MULTIPLE GRANULAR CELL TUMORS IN A CHILD WITH NOONAN SYNDROME
MULTIPLE GRANULAR CELL TUMORS IN A CHILD WITH NOONAN SYNDROME
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DOI：
10.1111/j.1525-1470.2010.01111.x
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发表时间：
2010-03-01
影响因子：
1.5
通讯作者：
Dinulos, James G. H.
中科院分区：
文献类型：
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作者：
Ramaswamy, Preethi V.;Storm, Craig A.;Dinulos, James G. H.
颗粒细胞瘤是良性神经源性肿瘤，累及皮肤和皮下组织，通常为孤立性病变。多发性颗粒细胞瘤发生在10%的受影响的个体中，但在儿童中。有潜在的躯体和遗传综合征的儿童，包括神经纤维瘤病和努南综合征，发生多发性颗粒细胞瘤的风险更高。皮肤活检有助于诊断，因为颗粒细胞瘤与其他皮肤结节具有相似的外观。疼痛或快速生长的颗粒细胞瘤应切除，可观察到无症状的非生长颗粒细胞瘤。患有多发性颗粒细胞瘤的儿童应该进行全面的体格检查，以排除潜在的遗传综合征。
Granular cell tumors are benign neurally derived neoplasms, involving cutaneous and subcutaneous tissues; and typically occur as solitary lesions. Multiple granular cell tumors occur in 10% of affected individuals, but are in children. Children with underlying somatic and genetic syndromes, including neurofibromatosis and Noonan syndrome, appear to be at higher risk of developing multiple granular cell tumors. Skin biopsy assists in diagnosis, since granular cell tumors have a similar appearance to other cutaneous nodules. Painful or rapidly growing granular cell tumors should be excised and asymptomatic non-growing granular cell tumors may be observed. Children with multiple granular cell tumors should have a complete physical examination to rule out an underlying genetic syndrome.