Presentation
Either JXG or NA was present in 15 patients (24%). We found a high frequency of NA and JXG in patients with NF1. These conditions were present in more than half the children with NF1 younger than 2 years. [jamanetwork.com]
Case presentation A thirteen-year-old female patient was admitted at our institution with a bulbar juxtalimbal conjunctival pigmented lesion (Figure 1 ). [diagnosticpathology.biomedcentral.com]
A lymphocytic host response was present in 17 lesions (52%). Mitotic figures were rarely seen. None of the lesions had recurred over an average follow-up period of 34 months. [ncbi.nlm.nih.gov]
Childhood malignant melanomas frequently present as nodular melanomas with Spitzoid features. Spitz nevus and Spitzoid melanoma overlap clinically and histopathologically and there have been many attempts to differentiate between them. [scienceopen.com]
It is estimated that neurocutaneous melanosis is present in 2% to 45% of patients with giant congenital melanocytic nevi. [en.wikipedia.org]
Entire Body System
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Epilepsy
Epilepsy and intellectual impairment may be associated. [patient.info]
In rare cases, the condition may be associated with neurological conditions such as epilepsy. Additionally, tumors may form within lesions, and may be either benign or malignant. [ozarkderm.com]
Skin
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Cutaneous Manifestation
In this age group, NF1 usually manifests as isolated multiple café au lait spots, which are highly suggestive but not specific for NF1. 7 - 9 Additional cutaneous manifestations may occur in children with NF1: juvenile xanthogranulomas (JXG) and nevus [jamanetwork.com]
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Darier's Sign
Juvenile xanthogranuloma was defined as asymptomatic yellow-brown papules or nodules, without Darier sign (swelling or redness on rubbing). Nevus anemicus was defined as hypochromic macules devoid of vasodilation on friction. [jamanetwork.com]
Treatment
Some individuals advocate the use of hair removal laser for the treatment of congenital nevi. [en.wikipedia.org]
Treatment *Monitoring lesions for development of tumors is imperative *As long as nevi do not change, treatment is typically not required *Typical treatment consists of removal Nevus sebaceous is typically not treated, as they do not generally cause issues [ozarkderm.com]
Over-diagnosis of melanoma can lead to inappropriate therapy and psychologic burdens, whereas under-diagnosis can lead to inadequate treatment of a deadly cancer. [scienceopen.com]
For this reason, many physicians opt for a more aggressive treatment approach. Talk to your doctor about the best treatment options for your particular situation. [healthline.com]
Treatment: The treatment of choice is surgical excision, with a 0.5 to 1 cm margin if atypical in clinical morphology, location or patient age, narrower if the lesion is classical. [skincareguide.ca]
Prognosis
• Conjunctival nevi have excellent prognosis • PAM without atypia can recur in about 11% cases, but it does not progress to melanoma MICROSCOPIC Histologic Features • Anatomic classification (similar to skin) Junctional: Nested but sometimes also lentiginous [basicmedicalkey.com]
(Large case series of sixty-seven adults and children undergoing evaluation for atypical Spitz tumors; demonstrated the high prevalence of positive sentinel lymph node biopsies but an overall favorable prognosis.) Miteva, M, Lazova, R. [clinicaladvisor.com]
McWhorter and Woolner 13 in 1954 reviewed the subject of malignant melanoma in children and suggested that the favorable prognosis was spurious and due to the erroneous classification of spindle and epithelioid cell nevi under the former agnosis. [pediatrics.aappublications.org]
It arises from pigment- ( melanin -) producing cells and varies in course and prognosis according to type; types include superficial spreading melanoma, nodular malignant melanoma, and lentigo maligna melanoma. [medical-dictionary.thefreedictionary.com]
Prognosis The prognosis is very good. [patient.info]
Etiology
Pathophysiology The etiology of juvenile xanthogranuloma (JXG) is not fully known. The papules and nodules of JXG represent collections of differentiated non–Langerhans cell histiocytes. [emedicine.medscape.com]
May 2019: Iranian Journal of Otorhinolaryngology # 5 Jaime Doody, Eelam A Adil, Cameron C Trenor, Michael J Cunningham OBJECTIVE: Juvenile nasopharyngeal angiofibroma (JNA) is a rare vascular tumor of unknown etiology. [read.qxmd.com]
CPERH has been identified in a one-year-old child who was diagnosed with NF2 six years later. 20 Epiretinal membranes (ERMs) can co-occur with CPERH in 78% of cases. 20 Adults presenting with ERM are typically older than 50, and etiology is typically [reviewofoptometry.com]
The etiology is unknown. Some have postulated that JXG represents a reactive histiocytic process to an as yet unidentified stimulus. Systemic Implications and Complications JXGs may affect multiple organ systems. [dermatologyadvisor.com]
Epidemiology
[…] found commonly in dark-skinned individuals and shows bilateral involvement Definitions • Benign melanocytic proliferation arising on conjunctiva • Most common tumors of conjunctiva, accounting for up to 53% of all conjunctival neoplasms CLINICAL ISSUES Epidemiology [basicmedicalkey.com]
You can help by adding to it. ( February 2018 ) Treatment [ edit ] Surgical removal is usually performed, even though it is benign. [ citation needed ] Epidemiology [ edit ] Spitz nevi are uncommon. [en.wikipedia.org]
Melanoma incidence rises for children and adolescents: an epidemiologic review of pediatric melanoma in the United States. J Pediatr Surg 2013 ; 48 ( 11 ): 2207–13. 35. Paradela, S, et al. Melanoma in children. [cambridge.org]
The latter term is generally no longer used as it is misleading: it is not a melanoma, it is a benign lesion; it can also occur in adults, not only in children. [ 4 ] Epidemiology Spitz nevi are uncommon. [en.academic.ru]
The epidemiology of acquired melanocytic nevi. A brief review. Dermatol Clin. 1995; 13(3):595-603. Clarke LE. Dysplastic nevi. Clin Lab Med. 2011; 31(2):255-65. Haley JC, Hood AF, Chuang TY, Rasmussen J. [practicaldermatology.com]
Pathophysiology
Pathophysiology The etiology of juvenile xanthogranuloma (JXG) is not fully known. The papules and nodules of JXG represent collections of differentiated non–Langerhans cell histiocytes. [emedicine.medscape.com]
Pathophysiology The pathophysiology of Spitz nevi and Spitzoid tumors remains unknown. [clinicaladvisor.com]
Prevention
There is no known method of prevention. Benign congenital nevi can have histological characteristics resembling melanomas, often breaking most if not all of the ABCDE rules. [en.wikipedia.org]
Hospital for Sick Children Health A-Z Search a complete list of child health articles expand_more View All Drug A-Z Search a list of articles about medications expand_more View All Learning Hubs Browse a complete list of content groups Healthy Living & Prevention [aboutkidshealth.ca]
How can I prevent this type of skin lesion? Look at your child's moles every month for any changes. [chw.org]
Inadequate attention has been given instead to the obligations borne by States to prevent ... The Equal Protection Doctrine In The Age Of Trump: The Example Of Unaccompanied Immigrant Children, Rebecca A. [network.bepress.com]
[…] describe a separate class of benign melanocytic neoplasms in children that were previously diagnosed and treated as melanoma. 2 Prior to this discovery, the standard of care was to remove all suspicious pigmented lesions in children prior to adulthood to prevent [mdedge.com]