Divisions of Pediatric Surgery and Anatomic Pathology, Children's National Medical Center, Washington, DC 20010, USA.
J Pediatr Surg. 2012 Feb;47(2):408-11. doi: 10.1016/j.jpedsurg.2011.11.041.
Giant congenital melanocytic nevi (CMN) are considered to be premalignant with the highest risk for conversion to melanoma. Solid tumors within giant CMN presenting at birth are relatively unusual, and a variety of malignancies have been reported within these masses. However, the frequency of malignancy in these tumors is unknown. We report the unusual case of a female neonate who was born with 2 large, perianal masses within a giant CMN over her trunk. We resected the larger of the 2 masses because of discomfort and bleeding. The mass consisted of an outgrowth of melanocytes with benign characteristics. The smaller of the 2 masses was managed expectantly and has subsequently decreased in size. We propose that pathologically benign masses within giant CMNs may not require immediate resection. We will discuss the management and pathologic findings of this case and review the literature regarding management of CMN-associated neoplasms arising at birth.
先天性巨大黑素细胞痣（CMN）被认为具有潜在恶变风险，尤其是发生黑色素瘤的风险最高。出生时即存在于先天性巨大 CMN 内的实性肿瘤相对少见，这些肿块内已经报道了多种恶性肿瘤。然而，这些肿瘤的恶性程度尚不清楚。我们报告了一例罕见病例，一名女性新生儿出生时在躯干上的巨大 CMN 内有 2 个大的肛周肿块。由于不适和出血，我们切除了较大的肿块。该肿块由具有良性特征的黑素细胞外生而成。较小的肿块采取了期待治疗，并且随后体积有所减小。我们提出，先天性巨大 CMN 内的病理性良性肿块可能不需要立即切除。我们将讨论该病例的处理方法和病理发现，并回顾有关出生时发生的与 CMN 相关的肿瘤的处理的文献。
