Presentation
The most common morphology is the large nodular form, which presents as either a solitary nodule or a few nodules. The small nodular form is the next most common variant presenting with multiple smaller papules. These two forms often coexist. [dermatologyadvisor.com]
The ocular presentation of neurofibromatosis 2. Eye. 1997;11:12-18. [reviewofoptometry.com]
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]
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
- Hodgkin Lymphoma
Proliferations Related to Down Syndrome Juvenile Myelomonocytic Leukemia Myeloid Neoplasms With Germline Predisposition Mastocytosis Childhood Myelodysplastic Syndrome Nodular Lymphocyte-Predominant Hodgkin Lymphoma Classic Hodgkin Lymphoma [elsevier.com]
Mutations in BRAF have also been associated with various cancers, including melanoma, thyroid carcinoma, colorectal cancer, non-Hodgkin lymphoma, and adenocarcinoma of lung. [atlasgeneticsoncology.org]
- Pallor
[…] patients with optic nerve gliomas are NF1 patients; NF1-associated OPGs are typically less aggressive than non-associated NF1 OPGs. 10 Many patients with OPGs are asymptomatic; however, OPGs may cause unilateral, bilateral or color vision loss; optic nerve pallor [reviewofoptometry.com]
Skin
- Ulcer
Spitz nevi may grow rapidly for several months and may rarely ulcerate; ulceration is most often seen in nevi that are greater than 1cm in diameter. [clinicaladvisor.com]
If ulcerated, wound care with topical antibiotics or emollients is often adequate. [dermatologyadvisor.com]
The 5 major factors were age, size, presence of ulceration, involvement of subcutaneous fat, and mitotic activity. [mdedge.com]
Certain atypical features of the Spitz nevus such as a diameter greater than 1 cm, asymmetry, or ulceration warrant a wider margin of normal skin during the initial excision. [aocd.org]
- Macula
Part B, Biochemistry & molecular biology 141(3) 314-322 Jul 2005 [Refereed] Novel macula-like virus identified in Bombyx mori cultured cells. [researchmap.jp]
Retinal astrocytic hamartomas are benign tumors of the retinal nerve fiber layer that can present bilaterally involving the optic nerve and posterior pole, with multiple peripheral lesions extending to the anterior retina. 8,9 If the optic nerve or macula [reviewofoptometry.com]
- 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]
Treatment
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]
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]
The management of the Spitz nevus is controversial as some dermatologists recommend conservative treatment with a partial biopsy as they are benign. [aocd.org]
What is the treatment? Most often the nevus is surgically removed at the same time the biopsy is done. Your doctor will talk about the different treatments to use depending on the severity of the lesion. [chw.org]
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]
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]
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]
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
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]
[…] 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]
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]
How can I prevent this type of skin lesion? Look at your child's moles every month for any changes. [chw.org]
Pediatricians play an important role in the prevention and early diagnosis of these lesions. [analesdepediatria.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]