Bada Bosch Isabel, Cañizo Agustín Del, Campos-Domínguez Minia, Ordoñez Javier, Blanco Verdú María Dolores, Fanjul María, Pérez-Egido Laura, de Agustín Juan Carlos
Department of Pediatric Surgery, Hospital General Universitario Gregorio Marañón, Madrid, Spain.
Department of Pediatric Dermatology, Hospital General Universitario Gregorio Marañón, Madrid, Spain.
European J Pediatr Surg Rep. 2022 Apr 19;10(1):e25-e29. doi: 10.1055/s-0042-1743159. eCollection 2022 Jan.
Vulvar masses in children are an unusual finding but their differential diagnosis is extensive. In case of solid masses, rhabdomyosarcoma (RMS) must always be considered due to the fact that it is the most common tumor in external genitals during childhood. However, RMS has a radiological appearance very similar to juvenile xanthogranuloma (JXG). We present a 16-month-old girl with a 2 cm solid mass on her left labia majora, with four overlying cutaneous papules. After imaging tests, an excisional biopsy was programmed due to high malignancy suspicion. Histopathology of the mass and one of the papules was diagnostic for JXG. After a 12-month follow-up, the patient shows no signs of relapse or complication. Deep JXG is an uncommon entity in childhood and exceptional in the genital area. Therefore, it must be included in the differential diagnosis of a solid vulvar mass, especially if accompanying yellowish xanthomatous cutaneous lesions are present.
儿童外阴肿物是一种不常见的表现，但其鉴别诊断范围广泛。对于实性肿物，横纹肌肉瘤（RMS）必须始终予以考虑，因为它是儿童期外生殖器最常见的肿瘤。然而，RMS的放射学表现与幼年性黄色肉芽肿（JXG）非常相似。我们报告一名16个月大的女孩，其左侧大阴唇有一个2厘米的实性肿物，肿物上有4个皮肤丘疹。经影像学检查后，由于高度怀疑恶性肿瘤，计划进行切除活检。肿物及其中一个丘疹的组织病理学检查诊断为JXG。经过12个月的随访，患者无复发或并发症迹象。深部JXG在儿童期并不常见，在生殖器区域更是罕见。因此，它必须列入实性外阴肿物的鉴别诊断中，尤其是当伴有淡黄色黄色瘤样皮肤病变时。
