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Clinicopathological and Molecular Features of Penile Melanoma With a Proposed Staging System.
The American Journal of Surgical Pathology ( IF 4.5 ) Pub Date : 2024-05-29 , DOI: 10.1097/pas.0000000000002247
Kristine M Cornejo 1 , Amrita Goyal 2 , Aida Valencia Guerrero 3 , Michael Astudillo 4 , Dora Dias-Santagata 1 , Matthew M Johnson 5 , Adam S Feldman 6 , Mai P Hoang 1
Affiliation  

Penile melanomas (PM) are an exceedingly rare subtype of mucosal melanoma (MM), and we reviewed the clinicopathologic features and molecular profile in 8 PMs. The patient ages ranged from 46 to 78 (mean: 62.8) years with involvement on the glans (n=5; 62.5%), penile urethra (n=2; 25%), and foreskin (n=1, 12.5%). Tumor depth ranged from 1.6 to 10.0 (mean: 5.25) mm. Most of the patients underwent partial penectomy (n=6; 75%) and sentinel lymph node (LN) biopsy N=7; 87.5%). Seven patients had metastatic disease at diagnosis, 6 involving LNs and 1 the adrenal gland, and 4 died of disease with a mean follow-up period of 40.5 (2 to 95) months. Five of 7 (71%) cases identified 15 molecular alterations within KIT, CDKN2A, NF1, PTEN, and APC (n=2 each), and NRAS, MAP3K1, CDH1, MSH6, and TERT (n=1 each). Two cases were not found to harbor genetic aberrations, and 1 case failed testing. In addition, we reviewed the English literature and included 93 cases with a reported depth of invasion and follow-up. A total of 101 PMs were analyzed for prognostic parameters, and the overall survival was significantly worse in patients with LN metastasis (P=0.0008), distant metastasis (P=0.0016), and greater depth of invasion (P=0.0222) based upon T-stage. While T4 conferred substantially worse survival, the delineation of the survival curves between T2 and T3 was less clear, and combining T2+T3 disease had a strong prognostic impact (P=0.0024). Prognostic parameters used in the staging of cutaneous melanomas may also be used in PMs. An alternative staging system expanding the inclusion criteria for T2 might provide a more accurate prognostic stratification.

中文翻译:


阴茎黑色素瘤的临床病理学和分子特征以及拟议的分期系统。



阴茎黑色素瘤 (PM) 是粘膜黑色素瘤 (MM) 的一种极其罕见的亚型,我们回顾了 8 例 PM 的临床病理特征和分子特征。患者年龄范围为 46 至 78 岁(平均:62.8)岁,受累龟头(n=5;62.5%)、阴茎尿道(n=2;25%)和包皮(n=1,12.5%)。肿瘤深度范围为 1.6 至 10.0(平均值:5.25)mm。大多数患者接受了部分阴茎切除术(n=6;75%)和前哨淋巴结(LN)活检 N=7; 87.5%)。 7 名患者在诊断时患有转移性疾病,其中 6 名涉及淋巴结,1 名涉及肾上腺,4 名患者死于疾病,平均随访时间为 40.5(2 至 95)个月。 7 例病例中的 5 例 (71%) 在 KIT、CDKN2A、NF1、PTEN 和 APC(各 n=2)以及 NRAS、MAP3K1、CDH1、MSH6 和 TERT(各 n=1)中发现了 15 个分子改变。 2例未发现基因畸变,1例检测未通过。此外,我们回顾了英文文献,纳入了 93 例报告的浸润深度和随访情况。总共分析了 101 个 PM 的预后参数,根据 T,有淋巴结转移 (P=0.0008)、远处转移 (P=0.0016) 和较大浸润深度 (P=0.0222) 的患者的总生存率显着较差。 -阶段。虽然 T4 的生存率明显较差,但 T2 和 T3 之间的生存曲线的划分不太清楚,并且 T2+T3 疾病的结合具有很强的预后影响 (P=0.0024)。用于皮肤黑色素瘤分期的预后参数也可用于 PM。扩大 T2 纳入标准的替代分期系统可能会提供更准确的预后分层。
更新日期:2024-05-29
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