Department of a Sarcoma Oncology, Moffitt Cancer Center, Tampa, Florida 33612, USA.
J Natl Compr Canc Netw. 2013 Jun 1;11(6):679-86. doi: 10.6004/jnccn.2013.0087.
The rising incidence of melanoma in children has brought increased attention to the clinical and pathologic diagnosis of pigmented lesions in the pediatric age group. Although melanoma in infancy and early childhood is often associated with large congenital nevi, in older children and teenagers it is most often sporadic, occurring in patients with a low skin phototype and substantial sun exposure. The rarity of this potentially fatal disorder demands astute clinical attention and a high index of suspicion for atypical lesions in pediatric patients. The challenges include the difficult decision of whether to biopsy and an often equivocal pathologic diagnosis. These diagnostically challenging and equivocal lesions lead to a degree of uncertainty regarding additional workup, prognosis, potential therapy, and follow-up plans. Consultation with a specialty dermatopathologist can be very helpful, and advanced molecular diagnostic techniques may be used in selected circumstances. Although still controversial, good evidence exists to justify a role for sentinel lymph node biopsy. Patients with atypical melanocytic proliferations have a high rate of positive sentinel lymph nodes; however, their outcomes are clearly better than in similarly staged adults with conventional melanoma. With the multiple variables involved and the relative lack of prospectively derived evidence, clinical decision-making is challenging and patients and families may experience considerable stress. This article provides data and weighs the pros and cons of a rationale for decision-making in pediatric and young adult patients with diagnostically challenging melanocytic lesions.
儿童黑色素瘤发病率的上升引起了人们对儿科年龄段色素性病变临床和病理诊断的关注。虽然婴儿和幼儿期的黑色素瘤通常与大的先天性痣有关，但在年龄较大的儿童和青少年中，它通常是散发性的，发生在皮肤光型低且大量暴露于阳光下的患者中。这种潜在致命疾病的罕见性要求临床医生敏锐地注意，并对儿科患者的非典型病变保持高度怀疑。挑战包括是否进行活检的艰难决策，以及通常模棱两可的病理诊断。这些具有诊断挑战性和模棱两可的病变导致对进一步检查、预后、潜在治疗和随访计划存在一定程度的不确定性。咨询专业皮肤科病理学家非常有帮助，在某些情况下可以使用先进的分子诊断技术。尽管仍存在争议，但有充分的证据表明前哨淋巴结活检具有一定作用。具有非典型黑色素细胞增生的患者前哨淋巴结阳性率很高；然而，与具有传统黑色素瘤的类似分期的成人相比，他们的预后明显更好。由于涉及多个变量且前瞻性证据相对缺乏，临床决策具有挑战性，患者及其家属可能会经历相当大的压力。本文提供了数据，并权衡了在具有诊断挑战性的黑色素细胞病变的儿科和年轻成年患者中做出决策的合理性。
