Risk factors and outcomes of melanoma in children and adolescents: A retrospective multicenter study.

BACKGROUND: Pediatric melanoma presents with distinct clinical features compared to adult disease. OBJECTIVE: Characterize risk factors and negative outcomes in pediatric melanoma. METHODS: Multicenter retrospective study of patients under 20 years diagnosed with melanoma between January 1, 1995 and June 30, 2015 from 11 academic medical centers. RESULTS: Melanoma was diagnosed in 317 patients, 73% of whom were diagnosed in adolescence (age ≥11). Spitzoid (31%) and superficial spreading (26%) subtypes were most common and 11% of cases arose from congenital nevi. Sentinel lymph node biopsy was performed in 68% of cases and positive in 46%. Fatality was observed in 7% of cases. Adolescent patients with melanoma were more likely to have family history of melanoma (P = .046) compared to controls. LIMITATIONS: Retrospective nature, cohort size, control selection, and potential referral bias. CONCLUSION: Pediatric melanoma has diverse clinical presentations. Better understanding of these cases and outcomes may facilitate improved risk stratification of pediatric melanoma.

Management of Drug-Induced Epidermal Necrolysis (DEN) in Pediatric Patients: Moving from Drug-Induced Stevens-Johnson Syndrome, Overlap and Toxic Epidermal Necrolysis to a Single Unifying Diagnosis of DEN.

Pediatric Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare but life-threating blistering diseases triggered by medications that affect the skin and mucosae. Drug-induced epidermal necrolysis is a better term for medication-triggered cases because there is a spectrum of disease severity that otherwise is divided into the separate entities of SJS, overlap SJS/TEN, and TEN. This manuscript reviews the management of drug-induced epidermal necrolysis (DEN), including diagnosis, investigations to exclude differential diagnoses, and treatment. Diagnosis of DEN relies on clinical features and a detailed medication history. The primary differential diagnosis is reactive infectious mucocutaneous eruption, which can be clinically distinguished by its disproportionate mucous membrane involvement relative to (sparse or absent) skin lesions. Identification and discontinuation of culprit medications is the mainstay of treatment of DEN. Early initiation of immunomodulatory therapy may prevent progression, reducing maximal disease severity and the risk of sequelae. A checklist approach to detailed management of DEN is proposed.

A retrospective multicenter study of fatal pediatric melanoma.

BACKGROUND: Pediatric melanoma is rare and diagnostically challenging. OBJECTIVE: To characterize clinical and histopathologic features of fatal pediatric melanomas. METHODS: Multicenter retrospective study of fatal melanoma cases in patients younger than 20 years diagnosed between 1994 and 2017. RESULTS: Of 38 cases of fatal pediatric melanoma identified, 57% presented in white patients and 19% in Hispanic patients. The average age at diagnosis was 12.7 years (range, 0.0-19.9 y), and the average age at death was 15.6 years (range, 1.2-26.2 y). Among cases with known identifiable subtypes, 50% were nodular (8/16), 31% were superficial spreading (5/16), and 19% were spitzoid melanoma (3/16). One fourth (10/38) of melanomas arose in association with congenital melanocytic nevi. LIMITATIONS: Retrospective nature, cohort size, and potential referral bias. CONCLUSIONS: Pediatric melanoma can be fatal in diverse clinical presentations, including a striking prevalence of Hispanic patients compared to adult disease, and with a range of clinical subtypes, although no fatal cases of spitzoid melanoma were diagnosed during childhood.

Risk Factors and Outcomes of Nonmelanoma Skin Cancer in Children and Young Adults.

OBJECTIVE: To identify risk factors associated with nonmelanoma skin cancer (NMSC) occurrence and survival in children. STUDY DESIGN: This was a multicenter, retrospective, case-control study of patients <20 years of age diagnosed with NMSC between 1995 and 2015 from 11 academic medical centers. The primary outcome measure was frequency of cases and controls with predisposing genetic conditions and/or iatrogenic exposures, including chemotherapy, radiation, systemic immunosuppression, and voriconazole. RESULTS: Of the 124 children with NMSC (40 with basal cell carcinoma, 90 with squamous cell carcinoma), 70% had at least 1 identifiable risk factor. Forty-four percent of the cases had a predisposing genetic condition or skin lesion, and 29% had 1 or more iatrogenic exposures of prolonged immunosuppression, radiation therapy, chemotherapy, and/or voriconazole use. Prolonged immunosuppression and voriconazole use were associated with squamous cell carcinoma occurrence (cases vs controls; 30% vs 0%, P = .0002, and 15% vs 0%, P = .03, respectively), and radiation therapy and chemotherapy were associated with basal cell carcinoma occurrence (both 20% vs 1%, P < .0001). Forty-eight percent of initial skin cancers had been present for >12 months prior to diagnosis and 49% of patients were diagnosed with ≥2 skin cancers. At last follow-up, 5% (6 of 124) of patients with NMSC died. Voriconazole exposure was noted in 7 cases and associated with worse 3-year overall survival (P = .001). CONCLUSIONS: NMSC in children and young adults is often associated with a predisposing condition or iatrogenic exposure. High-risk patients should be identified early to provide appropriate counseling and management.

Percutaneous Intervention to Treat Platypnea-Orthodeoxia Syndrome: The Toronto Experience.

OBJECTIVES: This study reviewed a series of patients treated with transcatheter closure of septal defect to treat platypnea-orthodeoxia syndrome, with specific attention to septal characteristics and device choice. BACKGROUND: Platypnea-orthodeoxia syndrome is an uncommon condition characterized by positional dyspnea and hypoxemia due to intracardiac right-to-left shunting through a patent foramen ovale (PFO), an atrial septal defect, or pulmonary arteriovenous malformations. Percutaneous closure of such defects is the treatment of choice. METHODS: In this single-center series, 52 patients were treated with percutaneous closure of an interatrial communication after presentation between January 1997 and July 2015. Septal morphology, clinical, procedural, and outcomes data were analyzed. RESULTS: All patients had a PFO; however, nearly one-quarter required a non-PFO device (11 Amplatzer Septal Occluder and 1 post-infarct muscular VSD), as opposed to a dedicated PFO device to achieve shunt occlusion. These patients were characterized by an aneurysmal septum, shorter primum septum overlap with the secundum septum, and greater septal angulation from the midline. After closure, all demonstrated acute improvements in oxygen saturation (pre-procedure: 81 ± 8%; post-procedure: 95.1 ± 0.5% on room air). Each patient was treated with a single device and no one required re-intervention. CONCLUSIONS: Patients presenting with platypnea-orthodeoxia syndrome can be treated successfully with a percutaneous intervention often requiring a variety of devices. Those requiring a non-PFO-type device had a greater prevalence of an aneurysmal septum, shorter primum septal overlap with the secundum septum, and greater septal angulation with the midline.