Department of Surgery and Translational Medicine, University of Florence, Florence, Italy.
Dermatopathology Section, Azienda Ospedaliera Città della Salute e della Scienza, Turin, Italy.
J Am Acad Dermatol. 2015 Jan;72(1):37-46. doi: 10.1016/j.jaad.2014.09.049. Epub 2014 Oct 25.
Diagnosis and proper management of atypical Spitz tumors in pediatric age are still controversial.
We sought to investigate the clinicopathological and molecular features of atypical Spitz tumors in patients aged 18 years or younger.
We performed a retrospective clinicopathological and fluorescence in situ hybridization study on 50 pediatric atypical Spitz tumors.
Parameters that were significantly correlated with a diagnosis of atypical Spitz tumors over Spitz nevus included asymmetry, level IV/V, lack of maturation, solid growth, nuclear pleomorphism, high nuclear-cytoplasmic ratio, atypical and deep mitoses, and more than 6 mitoses/mm(2). In the atypical Spitz tumors group, a significantly higher mitotic rate was observed in prepuberal age (P = .04). The 4-probe fluorescence in situ hybridization melanoma assay did not discriminate atypical Spitz tumors from Spitz nevi. Heterozygous 9p21 loss was found in 3 of 37 cases and homozygous 9p21 loss in 2 of 37 cases. Only 1 child experienced a fatal outcome, showing genetic abnormalities by melanoma fluorescence in situ hybridization probe and a heterozygous 9p21 deletion.
The limited number of adverse outcomes did not allow the prognostic analysis of single morphologic features.
Pediatric atypical Spitz tumors are associated with minimal lethal potential. Atypical Spitz tumors require complete excision and careful follow-up while our data do not support any clinical benefit for the sentinel lymph node biopsy procedure and completion lymphadenectomy.
儿童时期非典型 Spitz 肿瘤的诊断和恰当治疗仍存在争议。
我们旨在研究 18 岁及以下非典型 Spitz 肿瘤患者的临床病理和分子特征。
我们对 50 例儿童非典型 Spitz 肿瘤进行了回顾性临床病理和荧光原位杂交研究。
与 Spitz 痣相比,诊断为非典型 Spitz 肿瘤的显著相关参数包括不对称、水平 IV/V、缺乏成熟、实性生长、核多形性、高核浆比、不典型和深的有丝分裂,以及超过 6 个有丝分裂/mm(2)。在非典型 Spitz 肿瘤组中,青春期前年龄的有丝分裂率显著更高(P =.04)。4 探针荧光原位杂交黑色素瘤检测不能区分非典型 Spitz 肿瘤和 Spitz 痣。37 例中有 3 例存在杂合性 9p21 缺失,37 例中有 2 例存在纯合性 9p21 缺失。只有 1 例患儿出现致命结局,通过黑色素瘤荧光原位杂交探针显示遗传异常和杂合性 9p21 缺失。
不良结局数量有限,无法进行单一形态特征的预后分析。
儿童非典型 Spitz 肿瘤具有极小的致命潜能。非典型 Spitz 肿瘤需要完全切除和密切随访,而我们的数据不支持前哨淋巴结活检和完成淋巴结清扫术的任何临床获益。