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Management of cutaneous neurofibroma: current therapy and future directions.

ABSTRACT: Neurofibromatosis type 1 (NF1) is a life-long neurocutaneous disorder characterized by a predisposition to tumor development, including cutaneous neurofibroma (cNF), the hallmark of the disease. cNF is a histologically benign, multicellular tumor formed in virtually most individuals with NF1. It is considered the most burdensome feature of the disorder due to their physical discomfort, cosmetically disfiguring appearance, and psychosocial burden. Management of cNF remains a challenge in the medical field. Effective medicinal treatment for cNF does not exist at this time. Trials aimed at targeting individual components of the neoplasm such as mast cells with Ketotifen have not shown much success. Physical removal or destruction has been the mainstay of therapy. Surgical removal gives excellent cosmetic results, but risk in general anesthesia may require trained specialists. Destructive laser such as CO2 laser is effective in treating hundreds of tumors at one time but has high risk of scarring hypopigmentation or hyperpigmentation that alter cosmetic outcomes. A robust, low-risk surgical technique has been developed, which may be performed in clinic using traditional biopsy tools that may be more accessible to NF1 patients worldwide than contemporary techniques including Er:YAG or Nd:YAG laser. In this review, specific recommendations for management of cNFs are made based on symptoms, clinical expertise, and available resources. Additionally, antiproliferative agents aimed at stimulating cellular quiescence are explored.

SUBMITTER: Chamseddin BH

PROVIDER: S-EPMC7317049 | biostudies-literature | 2020 Jul

REPOSITORIES: biostudies-literature

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Management of cutaneous neurofibroma: current therapy and future directions.

Chamseddin Bahir H BH Le Lu Q LQ

Neuro-oncology advances 20191216 Suppl 1

Neurofibromatosis type 1 (NF1) is a life-long neurocutaneous disorder characterized by a predisposition to tumor development, including cutaneous neurofibroma (cNF), the hallmark of the disease. cNF is a histologically benign, multicellular tumor formed in virtually most individuals with NF1. It is considered the most burdensome feature of the disorder due to their physical discomfort, cosmetically disfiguring appearance, and psychosocial burden. Management of cNF remains a challenge in the medi ...[more]

PMID: 32642736

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Project description:BackgroundCurrent evidence-based treatments for adult anorexia nervosa (AN) have limitations, with high attrition, very poor outcomes for 20% of people, and no clearly superior manualised therapy for adults with AN. Specialist Supportive Clinical Management (SSCM) was designed as a control treatment but has evolved as a valid first line treatment. The present paper aims to provide an overview of the evidence base for SSCM and a pedagogical reconceptualization with expansion by theoretical integration (TI). BODY: A secondary meta-analysis endorses SSCM as a promising treatment. This paper positions SSCM as a manualised therapy for adult AN with six unique features, namely (1) a philosophy which is person-centred, non-prescriptive, and informed by the person's strengths and values, (2) a focus on the person through inclusion of supportive psychotherapy and problem (clinical management), within target symptoms as defined in relation to AN, (3) a flexible and responsive therapy that could be delivered by a variety of clinicians with experience treating AN (4) a commitment to reversing starvation though a directional approach and a defined yet flexible stance on dietetic intervention (5) a commitment to the therapeutic relationship within all three phases of treatment, and (6) a therapy 'uncluttered' by specific mandates. In addition, this paper positions SSCM as a treatment that may be strengthened by other modalities and may also be adapted to the treatment of other eating disorders (ED), not just AN. The level of therapist sophistication to deliver upon the supportive psychotherapy component is explored and future directions are offered.ConclusionSSCM is a unique and valid first line treatment for AN and would benefit from further expansion in line with emerging understandings of AN to strengthen it as a treatment. Speculation on aspects of potency would benefit from further testing. The proposed re-conceptualisation of SSCM in the context of its evidence may strengthen it as a treatment overall, position it as adaptable for treatment of other eating disorders and make it more accessible to clinicians.

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Management of cutaneous neurofibroma: current therapy and future directions.

Publications

Management of cutaneous neurofibroma: current therapy and future directions.

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