Pediatric melanoma is the most common skin cancer in children and treatment relies on accurate staging. The American Academy of Dermatology recommends excisional biopsy for suspicious skin lesions, however, partial shave biopsies are often performed, the impact of which is unknown in pediatric and adolescent/young adult (AYA) patients. The aim of this retrospective case series study was to evaluate the impact of the diagnostic biopsy method on staging, treatment, and treatment-related outcomes in pediatric/AYA patients with melanoma. Among 103 pediatric/AYA patients with atypical cutaneous melanocytic lesions, the most common biopsy method was partial shave (68/103, 66.0%) followed by punch (20/103, 19.4%), excisional (14/103, 13.6%), and incisional nonshave (1/103, 1%). Over half of all biopsies yielded a positive deep margin, reflecting compromised microstaging (56/103, 55.4%), the majority occurred following partial shave (52/56, 92.9%) compared with other techniques (P < 0.001). All 11 patients with wider surgical target margins of wide local excision and 8/9 patients with sentinel lymph node biopsy performed due to positive deep margin, underwent a partial shave biopsy (P = 0.05 and 0.32, respectively). Almost half of all patients who underwent partial shave biopsy had a clinically suspected abnormal melanocytic tumor prior to biopsy (31/68, 45.6%; P = 0.03). Of 56 patients who had compromised microstaging, 17 (30.4%) had a diagnosis of melanoma (P = 0.17). Pediatric/AYA patients frequently undergo partial shave biopsy, which is associated with more invasive definitive surgical treatment due to compromised microstaging. These results may help optimize care of patients with cutaneous melanocytic tumors.

PURPOSEThe purpose of this study was to develop recommendations for the diagnostic evaluation and surgical management of cutaneous melanoma (CM) and atypical Spitz tumors (AST) and non-Spitz melanocytic tumors (melanocytomas) in pediatric (age 0-10 years) and adolescent (age 11-18 years) patients.METHODSA Children's Oncology Group-led panel with external, multidisciplinary CM specialists convened to develop recommendations on the basis of available data and expertise.RESULTSThirty-three experts from multiple specialties (cutaneous/medical/surgical oncology, dermatology, and dermatopathology) established recommendations with supporting data from 87 peer-reviewed publications.RECOMMENDATIONS(1) Excisional biopsies with 1-3 mm margins should be performed when feasible for clinically suspicious melanocytic neoplasms. (2) Definitive surgical treatment for CM, including wide local excision and sentinel lymph node biopsy (SLNB), should follow National Comprehensive Cancer Network Guidelines in the absence of data from pediatric-specific surgery trials and/or cohort studies. (3) Accurate classification of ASTs as benign or malignant is more likely with immunohistochemistry and next-generation sequencing. (4) It may not be possible to classify some ASTs as likely/definitively benign or malignant after clinicopathologic and/or molecular correlation, and these Spitz tumors of uncertain malignant potential should be excised with 5 mm margins. (5) ASTs favored to be benign should be excised with 1- to 3-mm margins if transected on biopsy. (6) Re-excision is not necessary if the AST does not extend to the biopsy margin(s) when complete/excisional biopsy was performed. (7) SLNB should not be performed for Spitz tumors unless a diagnosis of CM is favored on clinicopathologic evaluation. (8) Non-Spitz melanocytomas have a presumed increased risk for progression to CM and should be excised with 1- to 3-mm margins if transected on biopsy. (9) Re-excision of non-Spitz melanocytomas is not necessary if the lesion is completely excised on biopsy.

Background: Indocyanine green (ICG) is a fluorescent dye with increasing use for adult sentinel lymph node biopsy (SLNB). The utility of ICG in pediatric oncology remains understudied. We aim to describe our experience using ICG for SLNB in pediatrics versus standard blue dye. Methods: A retrospective review of pediatric patients with melanoma or sarcoma who underwent SLNB with technetium plus ICG or blue dye from 2014 to 2023 at a large academic children's hospital was conducted. Results: Twenty-four patients were included; 58.3% were male with median age 13 years (range 4-21 years). The majority had a melanocytic tumor (91.7%) and 8.3% had sarcoma. All patients received technetium with concomitant blue dye (62.5%) or ICG (37.5%). ICG more reliably identified radioactive SLNs, compared to blue dye (mean 100% vs 78.3 +/- 8.3%, p = 0.03). There was no significant difference in median operative time (ICG 82 min [68-203] vs blue dye 93 min [78-105], p = 0.84). Seven patients had positive SLNs (29.2%), with recurrence in 2 patients (8.3%) and 1 death (4.2%). There were no adverse events. Conclusion: ICG-directed SLNB in children is a safe and effective alternative to blue dye. Use of ICG did not add to operative time, and more often identified sentinel nodes versus blue dye. Type of Study: Original Research Article, Retrospective Comparative Study. Level of Evidence: III. (c) 2024 Elsevier Inc. All rights reserved.

While all childhood cancers are rare, tumors that are particularly infrequent or underrepresented within pediatrics are studied under the umbrella of the Children's Oncology Group Rare Tumor committee, divided into the Retinoblastoma and Infrequent Tumor subcommittees. The Infrequent Tumor subcommittee has traditionally included an emphasis on globally rare tumors such as adrenocortical carcinoma, nasopharyngeal carcinoma, or those tumors that are rare in young children, despite being common in adolescents and young adults, such as colorectal carcinoma, thyroid carcinoma, and melanoma. Pleuropulmonary blastoma, gonadal stromal tumors, pancreatic tumors including pancreatoblastoma, gastrointestinal stromal tumor, nonmelanoma skin cancers, neuroendocrine tumors, and desmoplastic small round cell tumors, as well as other carcinomas are also included under the heading of the Children's Oncology Group Rare Tumor committee. While substantial challenges exist in rare cancers, inclusion and global collaboration remain key priorities to ensure high quality research to advance care.

Overcoming replicative senescence is an essential step during oncogenesis, and the reactivation of TERT through promoter mutations is a common mechanism. TERT promoter mutations are acquired in about 75% of melanomas but are not sufficient to maintain telomeres, suggesting that additional mutations are required. We identified a cluster of variants in the promoter of ACD encoding the shelterin component TPP1. ACD promoter variants are present in about 5% of cutaneous melanoma and co-occur with TERT promoter mutations. The two most common somatic variants create or modify binding sites for E-twenty-six (ETS) transcription factors, similar to mutations in the TERT promoter. The variants increase the expression of TPP1 and function together with TERT to synergistically lengthen telomeres. Our findings suggest that TPP1 promoter variants collaborate with TERT activation to enhance telomere maintenance and immortalization in melanoma.

e21576 Background: Melanoma is the dominant skin cancer of pediatric patients, and accounts for 1-3% of pediatric malignancies. The prognosis for both adults and children with melanoma correlates with stage at diagnosis, and initial Breslow depth is a critical determinant. Kaplan-Meier melanoma-specific survival curves show survival rates ranging from 98% (T1a) to 75% (T4b) at 10 years for adult patients with stage I and II disease at diagnosis. Pediatric patients are known to present with thicker primary melanomas as compared to their adult counterparts. The preferred biopsy method of the American Academy is Dermatology for melanoma is excisional, however partial shave biopsy is most frequently performed in children, given its simplicity, efficiency, and often low clinical suspicion for cutaneous malignancy. Despite the ease of this biopsy technique, shave biopsy has a high rate of base transection, reducing accuracy of microstaging, which is crucial for therapy planning. Methods: Retrospective chart review evaluation of the potential effects of biopsy method on staging, surgical recommendations, and treatment approach for pediatric patients with melanocytic tumors. Results: Data was available for 91 pediatric patients with a spectrum of melanocytic tumors ranging from atypical with unknown malignant potential, to melanoma. Patient characteristics including gender, age at biopsy, biopsy method, status of biopsy margins, recommendations for re-excision, sentinel lymph node biopsy, and treatment plan were collected and analyzed. There were 48 females and 43 males with age range 1—22 years (mean 10 years). Sixty-eight of 91 (75%) tumors had a positive margin (deep, peripheral, or both) on diagnostic biopsy, 52/68 (76.5%) were obtained using shave method. Evaluating the deep margin specifically, 50/91 (55%) tumors had a positive deep margin, 46/50 (92%) of which were obtained using shave method. No tumor evaluated by punch had a positive deep margin (0/18). Of all shave biopsies, 77% had positive deep margin as opposed to only 13% using alternative biopsy methods. In 10/91 (11%) patients, surgical recommendations were changed based on inaccurate microstaging and positive deep biopsy margins, 9/10 (90%) of these patients had undergone shave biopsy. Most (8/10) patients had melanoma, the remaining 2/10 had highly atypical spitzoid tumors in which melanoma could not be ruled out. Conclusions: Pediatric patients with melanocytic tumors most commonly underwent shave biopsy as the initial diagnostic biopsy method. This method of biopsy was associated with higher incidence of positive margins at diagnosis and more aggressive definitive surgical management. These findings suggest that more routine use of excisional biopsy in pediatric patients with lesions being evaluated for malignancy could decrease the incidence of inaccurate microstaging and reduce the need for more aggressive definitive surgical management.

There are nearly 70,000 new cancer diagnoses made annually in adolescents and young adults (AYAs) in the United States. Historically, AYA patients with cancer, aged 15 to 39 years, have not shown the same improved survival as older or younger cohorts. This article reviews the contemporary cancer incidence and survival data through 2015 for the AYA patient population based on the National Cancer Institute's Surveillance, Epidemiology, and End Results registry program and the North American Association of Central Cancer Registries. Mortality data through 2016 from the Centers for Disease Control and Prevention's National Center for Health Statistics are also described. Encouragingly, absolute and relative increases in 5-year survival for AYA cancers have paralleled those of childhood cancers since the year 2000. There has been increasing attention to these vulnerable patients and improved partnerships and collaboration between adult and pediatric oncology; however, obstacles to the care of this population still occur at multiple levels. These vulnerabilities fall into 3 significant categories: research efforts and trial enrollment directed toward AYA malignancies, access to care and insurance coverage, and AYA-specific psychosocial support. It is critical for providers and health care delivery systems to recognize that the AYA population remains vulnerable to provider and societal complacency.