To the Editor:
Melanoma is an aggressive skin cancer, accounting for only ~1% of skin malignancies, yet representing the second most diagnosed cancer in 15 to 29-year-olds and the most skin cancer-related deaths in the United States1–3. Melanoma occurring in the childhood, adolescent, and young adult (CAYA) group is often subcategorized as conventional, arising within a congenital nevus, or Spitz/Spitzoid4. Despite subgrouping, prediction of biologic behavior is challenging, with pediatric melanoma differing from adult melanoma in unique ways, likely related to differences in the driving genomic events5–7. Furthermore, despite advances in molecular characterization, for the large group of atypical Spitz tumors and melanocytic tumors of uncertain malignant potential (MELTUMP), it remains difficult to classify these lesions as unequivocally benign or malignant, and standard staging methods do not provide the same clear prognostic information as in conventional melanoma8 –11. As such, there remains diagnostic uncertainty regarding the identification of which tumors will behave aggressively leading to widely varying treatment patterns, including wide local excision (WLE) surgical margins, indications for sentinel lymph node biopsy (SLNB), and systemic therapies. Furthermore, referral patterns are complex, involving providers from a variety of subspecialties12 –15. These factors pose substantial management challenges for CAYA patients, ultimately leading to delays in therapy, treatment variability, distress among providers, caregivers, and patients, and potentially decreased overall survival16,17.
In 2021, within the context of the Children’s Oncology Group (COG) Rare Tumor Committee, a Melanoma Task Force developed including subspecialty providers from pediatric oncology, medical oncology, pediatric surgery, pediatric dermatology, and dermatopathology. In this report, we provide feasibility survey data reflecting multidisciplinary provider practices and referral patterns, as well as general interest regarding involvement in a novel, collaborative clinical trial for pediatric patients with melanoma and atypical melanocytic tumors. This study was institutional review board exempt and full surveys are available in supplemental materials (S1–S6).
Pediatric oncology provider survey data
Seventy (32.1%) completed surveys (REDCap) were received from 218 COG site principal investigators. Overall, the total number of melanocytic tumor referrals estimated for the 70 responding sites was approximately 240 new patients annually (Table 1). Most commonly, referrals came from pediatric dermatology (48/70; 68.6%) and general dermatology (36/70; 51.4%), followed by primary care (26/70; 37.1%) and pediatric surgery (24/70; 34.3%). Most clinical scenarios prompting referral to pediatric oncology included a pathology report indicating malignancy, atypia, and/or positive histological margins on a diagnostic biopsy, followed by a family history of melanoma (Fig. 1). For definitive surgical management, over half of the respondents indicated referral to pediatric surgery (36/69; 52.2%) followed by pediatric plastic surgery (28/69; 40.6%). Over 88% of respondents indicated potential interest in a COG-led prospective surgical management clinical trial for pediatric patients with melanoma or an atypical melanocytic tumor (Fig. 2).
TABLE 1.
COG site estimated new melanoma/melanocytic tumors per year
| Program size by new oncology diagnoses per year, N = 70 (%) |
||||
|---|---|---|---|---|
| Survey Item | <50 (15.7%) | 50 – 99 (44.3%) | 100 – 149 (10.0%) | ≥ 150 (30.0%) |
|
| ||||
|
Are patients ≤ 21 years old with atypical and/or malignant pigmented cutaneous tumors
(i.e. atypical Spitz tumors / melanocytomas / melanomas) care for at your site? |
||||
| Yes | 6 (54.5%) | 31 (80.6%) | 7 (100.0%) | 20 (95.2%) |
| No | 5 (45.5%) | 6 (19.4%) | 0 (0.0% | 1 (4.8%) |
|
| ||||
| Over one year, how many new patient referrals (≤ 21 years old) do you estimate your pediatric oncology group receives for pigmented cutaneous tumors (i.e. atypical Spitz tumors /melanocytomas /melanomas)? | ||||
| Total (range, per institution) | 28 (0 – 20*) | 57 (0 – 8) | 17 (1 – 5) | 137 (0 – 30) |
Range for 10/11 institutions was 0 – 2, with one outlier responding 20 per year
FIGURE 1.
Clinical Scenarios Prompting Referral to or Consult with Another Medical Specialty.
FIGURE 2.
Interest in a Prospective Surgical Management Trial for Melanoma and Atypical Melanocytic Tumors, by Medical Specialty Surveyed.
General and pediatric dermatology provider survey data
Dermatology providers were surveyed through the American Academy of Dermatology (AAD; via Qualtrics per AAD survey policy). Ninety-four responses were received, representing a <5% response rate from the 4035 randomly selected (per AAD survey policy) AAD members. A separate survey (REDCap) was also distributed through the Society for Pediatric Dermatology (SPD) resulting in 27 responses from 150 members. Pediatric dermatology respondents most referred CAYA patients with atypical melanocytic tumors and/or melanoma to pediatric oncology (17/27, 63.0% of respondents) followed by pediatric plastic surgery (15/27, 55.6% of respondents) (Fig.3). If there were a national cooperative trial led by the COG prospectively evaluating definitive surgical management for CAYA patients with atypical melanocytic tumors or melanoma, a majority of general and pediatric dermatologists (88% and 92%, respectively) who responded answered ‘yes’ or ‘maybe’ regarding interest in referring to a pediatric oncology center for trial participation (Fig. 2).
FIGURE 3.
Proportion of Providers Consulting with Pediatric Oncology.
Pediatric surgery provider survey data
A survey (REDCap) was distributed to 1788 members of the American Pediatric Surgical Association (APSA) with 93 completed surveys returned. Ninety percent of surveyed pediatric surgeons stated they would usually (58/93, 62.4%) or sometimes (26/93, 28.0%) involve other specialties in the care of children and adolescents with malignant or atypical melanocytic cutaneous lesions. Reasons prompting these surgeons to refer to and/or consult with other surgical or medical specialties included a diagnosis of melanoma (83/93, 89.2%), atypical Spitz tumor (54/93, 58.1%), atypical non-Spitz melanocytic tumor (52/93, 55.9%), for positive margins (any histology) (47/93, 50.5%), and 16.1% (15/93) for a family history of melanoma (Fig. 1). When asked about interest in participating in a national cooperative clinical trial prospectively evaluating definitive surgical management for atypical and/or malignant melanocytic lesions in children and adolescents, half (46/93,49.5%) of responding pediatric surgeons answered ‘yes’, and an additional 32.3% (30/93) indicated ‘maybe’ (Fig. 2).
In conclusion, pediatric melanoma and atypical melanocytic tumors pose significant challenges for the many subspecialty providers who care for these patients, both in diagnosis and clinical management. While recognizing the limitations of this study inherent to survey-based research including non-response bias, the results indicate that despite potential barriers including challenges with tumor classification and patient accrual due to complex referral patterns, pediatric patients with these tumors are often referred to pediatric oncology, and there appears to be interest in a cooperative trial across disciplines. These results support consideration for development of a prospective surgical management clinical trial for pediatric patients with melanoma and atypical melanocytic neoplasms, the first of its kind led by COG.
Supplementary Material
S3. COG Melanoma Task Force American Academy of Dermatology (AAD) Survey.
S2. Launch email script for COG Site Melanoma and Atypical Melanocytic Tumors Management Practices Survey.
S1. COG Site Melanoma and Atypical Melanocytic Tumors Management Practices Survey.
S4. Launch email script for American Academy of Dermatology (AAD) Melanoma and Atypical Melanocytic Tumors Management Practices Survey.
S5. Society for Pediatric Dermatology (SPD) Pediatric Dermatology Providers: Atypical and/or Malignant Pigmented Skin Lesion Management Practices Survey.
S6. American Pediatric Surgical Association (APSA) Pediatric Surgery Providers: Atypical and/or Malignant Pigmented Skin Lesion Management Practices Survey.
Acknowledgements:
Children’s Oncology Group Communications and Publications, American Academy of Dermatology Senior Leadership Team, American Pediatric Surgical Association Cancer Committee and Survey Subcommittee, Society for Pediatric Dermatology for assistance with survey approvals and dissemination.
Funding:
This work was funded by Children’s Oncology Group NCTN Operations Center, Grant Number: U10CA180886, NCTN Statistics and Data Center, Grant Number: U10CA180899, and St. Baldrick’s Foundation.
Abbreviations key:
- CAYA
children, adolescent, and young adult
- MELTUMP
melanocytic tumors of uncertain malignant potential
- WLE
wide local excision
- SLNB
sentinel lymph node biopsy
- COG
Children’s Oncology Group
- AAD
American Academy of Dermatology
- SPD
Society for Pediatric Dermatology
- APSA
American Pediatric Surgical Association
Footnotes
Conflict of Interest Statement: None to disclose.
Disclaimer: The content is solely the responsibility of the authors and does not necessarily represent the official views of the National Institutes of Health.
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Associated Data
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Supplementary Materials
S3. COG Melanoma Task Force American Academy of Dermatology (AAD) Survey.
S2. Launch email script for COG Site Melanoma and Atypical Melanocytic Tumors Management Practices Survey.
S1. COG Site Melanoma and Atypical Melanocytic Tumors Management Practices Survey.
S4. Launch email script for American Academy of Dermatology (AAD) Melanoma and Atypical Melanocytic Tumors Management Practices Survey.
S5. Society for Pediatric Dermatology (SPD) Pediatric Dermatology Providers: Atypical and/or Malignant Pigmented Skin Lesion Management Practices Survey.
S6. American Pediatric Surgical Association (APSA) Pediatric Surgery Providers: Atypical and/or Malignant Pigmented Skin Lesion Management Practices Survey.