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Registry offers insights into pediatric melanoma outcomes
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Tumor thickness, lymph node status, ulceration and disease stage significantly predicted survival among pediatric patients with melanoma, according to results of a retrospective review.
Bruce J. Averbook, MD, of the division of surgical oncology at MetroHealth Medical Center and associate professor at Case Western Reserve University, and colleagues also observed a trend toward increased survival among the youngest patients.
Bruce J. Averbook
Clinicians frequently base decisions about pediatric melanoma on data from adult patients, even though there are considerable differences between the two populations.
Averbook and colleagues analyzed clinicopathologic and outcomes data from an international registry of pediatric melanoma patients culled from 12 participating institutions. The analysis included 365 eligible children who were diagnosed with invasive disease between 1953 and 2008.
Patients were aged 1 to 21 years (mean age, 16 years).
Ten-year OS for the entire cohort was 80.6%. Ten-year OS was 100% among children aged birth to 10 years; 69.7% for those aged >10 years to 15 years; and 79.5% for those aged >15 years to 20 years (P=.147).
Ten-year OS was 97% among patients with tumors measuring 1 mm; 70% among patients with tumors measuring >1 mm to 2 mm; 78% for those with tumors >2 mm to 4 mm; and 80% for those with tumors >4 mm (P=.0077).
Patients with ulcerated tumors (P=.022) and those with lymph node metastasis (P=.017) demonstrated poorer survival.
Ten-year OS was 94.1% among patients with American Joint Committee on Cancer stage I disease; 79.6% for those with stage II disease; and 77.1% among those with stage III disease (P<.001).
“We anticipate that refinement of the international database, establishment of central pathology review and increased institutional participation will better define the behavior of pediatric melanoma and improve our ability to care for patients with this disease,” Averbook and colleagues wrote. “Future work is planned to improve the data collection, add prospective data entry for pigmented atypical melanocytic neoplasms and potentially expand data collection into the young adult population.”
The 100% survival rate in the youngest cohort was encouraging, but there were only 16 patients in this vulnerable group, Daniel G. Coit, MD, of the department of surgery at Memorial Sloan-Kettering Cancer Center, and colleagues wrote in an accompanying editorial.
“The confidence limits of the predicted outcome are broad,” they wrote.
Coit and colleagues suggested that some of the diagnoses may have been misclassified.
“This may also explain why there was poor correlation between Breslow thickness and survival outcome,” they wrote. “The association between outcome and ulceration suggests that, in contrast to adult melanoma, this may be better than tumor thickness as a predictor of biologic aggressiveness in the pediatric population. Nine of the 16 patients had positive lymph node disease, again raising the question of whether regional lymph node metastases are clinically relevant in the very young.”
The development of a database for pediatric melanoma and pediatric atypical melanocytic nevi is essential to ensure appropriate therapeutic approaches in these children, Coit and colleagues wrote.
“The authors readily acknowledge the potential for substantial diagnostic drift over this 55-year period, both between and within contributing institutions,” Coit and colleagues wrote. “Acknowledging the understandable absence of central pathology review, Averbook et al do not describe the specific histopathologic criteria for the diagnosis of [pediatric melanoma], criteria that might be retrospectively applied to potentially eligible cases to help refine a uniform study cohort.”
Exclusion criteria for this data set were not clearly outlined, and diagnostic features of the tumors were uncertain, they added.
“Retrospective series of pediatric patients with ‘melanomas’ — regardless of whether they are single-centered, multicentered or derived from large national databases — all suffer from the same fatal flaw: a lack of consistent definition as to what histology is associated with more aggressive biologic behavior,” Coit and colleagues wrote.
“In the absence of this clear-cut definition, clinicians will be hard pressed to describe the prognostic significance of either the primary tumor or the presence of tumor cells in a sentinel lymph node,” they wrote. “In the absence of this clarity, clinicians will continue to struggle to achieve consensus on the optimal care of these young children and their very worried parents.”
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Disclosure: Averbook offered expert testimony in a melanoma case for the law firm of Jeffries, Kube, Forrest and Monteleone. Other researchers report relationships with Bristol-Myers Squibb, Celgene, GlaxoSmithKline, Merck, Novartis and other companies. Coit reports consultant roles with Bristol-Myers Squibb and McKesson Health, and payment from the National Comprehensive Cancer Network for the development of educational presentations.
Perspective
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Michael Wong, MD, PhD, FRCPC
Are children simply small adults? Can the treatment “rules” established for adults apply to children? This publication from a 12-institution international registry of pediatric melanomas is an attempt to shed light on this rare cancer. Pediatric melanoma is rare, and to complicate matters, these cases resemble pigmented atypical melanocytic neoplasms (PAMN). The stakes are high because curative therapy is known to be possible in both the advanced and adjuvant settings, yet the risk categorization and long-term outcome of therapy is not well understood.
The data for this database cover almost a half century, from 1953-2008, encompassing only 365 patients with survival information. Just as in adults, Breslow thickness, ulceration and lymph node metastatic status significantly correlated with survival. Thin melanomas (≤1 mm) possess a 97% 10-year survival rate, and prognosis correlated with stage. No new prognostic factors were identified, so the answer to the first question is: Yes, children are simply like small adults — as far as the three major prognostic factors were concerned. However, a trend toward improved survival in those aged younger than 10 years is interesting and may be a clue to the biology of this disease in children.
The paper is silent as for the second question. There simply isn’t enough lymph node-positive patients (30%) or stage IV metastatic patients (4%) in the database. In addition, I was personally most anticipating guidance about PAMN, because there is a disproportionate intellectual effort and all-around anxiety about the management of an entity that straddles the line between malignant and benign. Although there were 208 PAMN patients in the database, follow up was “poor,” so the authors promise a second publication on this topic.
The almost half century timeline of this study spans major changes in surgical therapy and staging and supportive care, as well as changes in definitions and several renditions of the staging system. Molecular data are a more recent addition to the management of these tumors, so this will necessarily have to await maturation of more recent data. Further analysis of pediatric factors such as the association between puberty and melanoma behavior and prognosis will be important.
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