Cabral João José Lobato Guimarães Ferreira, Balacó Inês Maria Spencer, Alves Cristina Marta da Gama Gomes, Matos António Gabriel de Almeida
Serviço de Ortopedia Pediátrica, Centro Hospitalar e Universitário de Coimbra, Hospital Pediátrico, Coimbra, Portugal.
Rev Bras Ortop (Sao Paulo). 2020 Feb;55(1):125-129. doi: 10.1016/j.rbo.2018.02.006. Epub 2019 Dec 19.
Soft-tissue tumors are rare in the pediatric population. First described in 1969 as myxoma of the nerve sheath, the neurothekeoma is a benign tumor lesion with presumable origin in the nerve sheath. It occurs mainly in female children and presents as a mass of slow, subcutaneous growth, asymptomatic and without alteration of the local pigmentation. It is predominantly located in the head, neck, and extremities of the upper limbs. This report presents the case of a 16-year-old male with a tumor mass originating from the nerve sheath in the 4 left metacarpal, intraosseous, and relapsed after previous surgical resection 2 years before this observation. A marginal resection of the tumor mass was performed on the distal region of the fourth metacarpal, followed by curettage of the proximal phalanx and filling with structural autologous bone graft. The patient maintained a favorable postoperative clinical evolution, without local pain or range of motion limitation in his fingers. Radiologically, a progressive trabecular filling of the proximal phalanx of the fourth metacarpal was observed. At 17 months of follow-up, the patient is asymptomatic and shows no signs of relapse. The description of this case serves to increase the familiarity with this rare pathology, and aid its diagnosis and treatment.
软组织肿瘤在儿科人群中较为罕见。神经鞘黏液瘤于1969年首次被描述为神经鞘黏液瘤，是一种起源于神经鞘的良性肿瘤病变。它主要发生在女童身上，表现为缓慢生长的皮下肿块，无症状且局部色素无改变。它主要位于头、颈和上肢的四肢。本报告介绍了一例16岁男性患者的病例，该患者在观察前2年曾接受手术切除，其肿瘤肿块起源于左第4掌骨的神经鞘，为骨内肿瘤，术后复发。在第4掌骨远端区域对肿瘤肿块进行了边缘切除，随后刮除近端指骨并用自体结构性骨移植填充。患者术后临床进展良好，手指无局部疼痛或活动范围受限。影像学上，观察到第4掌骨近端指骨有逐渐的小梁填充。在随访17个月时，患者无症状，未显示复发迹象。对该病例的描述有助于增加对这种罕见病理的熟悉程度，并有助于其诊断和治疗。
