Retrospective Multicentric Study on Non-Optic CNS Tumors in Children and Adolescents with Neurofibromatosis Type 1

Santoro, Claudia; Picariello, Stefania; Palladino, Federica; Spennato, Pietro; Melis, Daniela; Roth, Jonathan; Cirillo, Mario; Quaglietta, Lucia; D’Amico, Alessandra; Gaudino, Giuseppina; Meucci, Maria Chiara; Ferrara, Ursula; Constantini, Shlomi; Perrotta, Silverio; Cinalli, Giuseppe

doi:10.3390/cancers12061426

Open AccessArticle

Retrospective Multicentric Study on Non-Optic CNS Tumors in Children and Adolescents with Neurofibromatosis Type 1

by

Claudia Santoro

^1,2,*

,

Stefania Picariello

^1,3

,

Federica Palladino

¹

,

Pietro Spennato

⁴

,

Daniela Melis

⁵,

Jonathan Roth

⁶,

Mario Cirillo

⁷

,

Lucia Quaglietta

⁸,

Alessandra D’Amico

⁹,

Giuseppina Gaudino

¹,

Maria Chiara Meucci

⁴

,

Ursula Ferrara

¹⁰,

Shlomi Constantini

⁶,

Silverio Perrotta

¹

and

Giuseppe Cinalli

⁴

¹

Neurofibromatosis Referral Center, Department of Women’s and Children’s Health, and General and Specialized Surgery, “Luigi Vanvitelli” University of Campania, Via Luigi de Crecchio 2, 80138 Naples, Italy

²

Clinic of Child and Adolescent Neuropsychiatry, Department of Mental and Physical Health, and Preventive Medicine, “Luigi Vanvitelli” University of Campania, Largo Madonna delle Grazie 1, 80138 Naples, Italy

³

Department of Advanced Medical and Surgical Sciences, “Luigi Vanvitelli” University of Campania, P.zza L. Miraglia 2, 80138 Naples, Italy

⁴

Department of Pediatric Neurosurgery, Santobono-Pausilipon Children’s Hospital, Via Mario Fiore 6, 80129 Naples, Italy

⁵

Department of Medicine, Surgery and Dentistry, “Scuola Medica Salernitana”, Via Salvador Allende, Baronissi, 84081 Salerno, Italy

⁶

Department of Pediatric Neurosurgery, Dana Children’s Hospital, Tel Aviv Sourasky Medical Center, 6 Weizmann St., Tel Aviv 6423906, Israel

⁷

Department of Medicine, Surgery, Neurology, Metabolism and Geriatrics, “Luigi Vanvitelli” University of Campania, Piazza Luigi Miraglia 2, 80138 Naples, Italy

⁸

Department of Pediatric Oncology, Santobono-Pausilipon Children’s Hospital, Via Mario Fiore 6, 80129 Naples, Italy

⁹

Department of Advanced Biomedical Sciences, “Federico II” University of Naples, Via Sergio Pansini 5, 80100 Naples, Italy

¹⁰

Section of Pediatrics, Department of Translational Medical Science, “Federico II” University of Naples, Via Sergio Pansini 5, 80100 Naples, Italy

^*

Author to whom correspondence should be addressed.

Cancers 2020, 12(6), 1426; https://doi.org/10.3390/cancers12061426

Submission received: 27 April 2020 / Revised: 22 May 2020 / Accepted: 28 May 2020 / Published: 31 May 2020

(This article belongs to the Special Issue Clinical and Genetic Findings in Patients with Neurofibromatosis Type 1)

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Abstract

The natural history of non-optic central nervous system (CNS) tumors in neurofibromatosis type 1 (NF1) is largely unknown. Here, we describe prevalence, clinical presentation, treatment, and outcome of 49 non-optic CNS tumors observed in 35 pediatric patients (0–18 years). Patient- and tumor-related data were recorded. Overall survival (OS) and progression-free survival (PFS) were evaluated. Eighteen patients (51%) harbored an optic pathway glioma (OPG) and eight (23%) had multiple non-optic CNS lesions. The majority of lesions (37/49) were managed with a wait-and-see strategy, with one regression and five reductions observed. Twenty-one lesions (42.9%) required surgical treatment. Five-year OS was 85.3%. Twenty-four patients progressed with a 5-year PFS of 41.4%. Patients with multiple low-grade gliomas progressed earlier and had a lower 5-year PFS than those with one lesion only (14.3% vs. 57.9%), irrespective of OPG co-presence. Non-optic CNS tumors are common in young patients with NF1. Neither age and symptoms at diagnosis nor tumor location influenced time to progression in our series. Patients with multiple lesions tended to have a lower age at onset and to progress earlier, but with a good OS.

Keywords: neurofibromatosis type 1; children; CNS; brain tumors; low-grade glioma; NF1

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MDPI and ACS Style

Santoro, C.; Picariello, S.; Palladino, F.; Spennato, P.; Melis, D.; Roth, J.; Cirillo, M.; Quaglietta, L.; D’Amico, A.; Gaudino, G.; et al. Retrospective Multicentric Study on Non-Optic CNS Tumors in Children and Adolescents with Neurofibromatosis Type 1. Cancers 2020, 12, 1426. https://doi.org/10.3390/cancers12061426

AMA Style

Santoro C, Picariello S, Palladino F, Spennato P, Melis D, Roth J, Cirillo M, Quaglietta L, D’Amico A, Gaudino G, et al. Retrospective Multicentric Study on Non-Optic CNS Tumors in Children and Adolescents with Neurofibromatosis Type 1. Cancers. 2020; 12(6):1426. https://doi.org/10.3390/cancers12061426

Chicago/Turabian Style

Santoro, Claudia, Stefania Picariello, Federica Palladino, Pietro Spennato, Daniela Melis, Jonathan Roth, Mario Cirillo, Lucia Quaglietta, Alessandra D’Amico, Giuseppina Gaudino, and et al. 2020. "Retrospective Multicentric Study on Non-Optic CNS Tumors in Children and Adolescents with Neurofibromatosis Type 1" Cancers 12, no. 6: 1426. https://doi.org/10.3390/cancers12061426

APA Style

Santoro, C., Picariello, S., Palladino, F., Spennato, P., Melis, D., Roth, J., Cirillo, M., Quaglietta, L., D’Amico, A., Gaudino, G., Meucci, M. C., Ferrara, U., Constantini, S., Perrotta, S., & Cinalli, G. (2020). Retrospective Multicentric Study on Non-Optic CNS Tumors in Children and Adolescents with Neurofibromatosis Type 1. Cancers, 12(6), 1426. https://doi.org/10.3390/cancers12061426

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Retrospective Multicentric Study on Non-Optic CNS Tumors in Children and Adolescents with Neurofibromatosis Type 1

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