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
- Pathologist
The skin is sent to a lab for a pathologist to examine. This is called a biopsy. and doctors who read the biopsies are called pathologists. Experienced pathologists can usually tell the difference between melanoma and a Spitz nevus. [chw.org]
Sophie Spitz, a pathologist, identified a separate class of noncancerous moles, which became known as Spitz nevi. This distinction between the mole types was important. [healthline.com]
Pathology of Melanocytic Nevi and Malignant Melanoma is the essential reference for every practicing dermatopathologist, pathologist, dermatologist, and cancer research scientist today. [books.google.com]
- Swelling
You should see a doctor right away if you have a mole that: changes size, shape, or color looks different from other moles on your skin has an irregular border causes itching or pain isn’t symmetrical spreads to areas around it causes redness or swelling [healthline.com]
Plexiform neurofibromas are soft swellings with indistinct borders located underneath the skin that can infiltrate the orbit and temporal regions or the eyelids. Eyelids with neurofibromas typically feel like a ‘bag of worms’ when palpitated. [reviewofoptometry.com]
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]
- Surgical Procedure
Medical Treatment Surgical Procedures Physical Modalities Not applicable Excision Not applicable Optimal Therapeutic Approach for this Disease Although controversial, many experts recommend complete excision of all Spitz nevi. [clinicaladvisor.com]
- Lymphadenopathy
ウィルス性リンパ節炎:viral lymphadenitis 風疹,EBV(伝染性単核球症:infectious mononucleosis) 薬剤性リンパ節症:drug-induced lymphadenopathy ◎亜急性壊死性リンパ節炎:subacute necrotizing lymphadenitis, Kikuchi's lymphadenitis ◎Piringerリンパ節炎:toxoplasmic lymphadenitis ◎木村氏病:angiolymphoid hyperplasia [medical-e.net]
- 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
- Freckles
It begins as a circumscribed macular patch of mottled pigmentation, showing shades of dark brown, tan, or black ( lentigo maligna or melanotic freckle of Hutchinson ), and enlarges by lateral growth before dermal invasion occurs. [medical-dictionary.thefreedictionary.com]
On DermNet NZ Spitz naevi – pathology Spitzoid melanoma Birthmarks (naevi) Moles (melanocytic naevi) Congenital melanocytic naevi Blue naevi Halo naevi Freckles Melanoma Other websites Spitz nevus — Medscape Reference Books about skin diseases See the [dermnetnz.org]
Clinical features of NF1 include skin fold freckles, neurofibromas, optic pathway gliomas, Lisch nodules and the most common finding associated with the disease: café au lait spots. [reviewofoptometry.com]
Risk factors for the development of AMN include fair skin type (often with red hair, blue eyes, and inability to tan), propensity to sunburn, freckling, and sun exposure. 2 Acquired melanocytic nevi may be flat (junctional melanocytic nevus) or raised [practicaldermatology.com]
Health (NIH) consensus conference, 1 a diagnosis of NF1 in children is established by the presence of at least 2 of 7 clinical criteria: 6 or more café au lait macules larger than 0.5 cm, 2 neurofibromas or 1 plexiform neurofibroma, axillary or inguinal freckling [jamanetwork.com]
- Skin Disease
Warfarin necrosis Cholesterol embolism Necrobiotic xanthogranuloma Polychondritis Subcutaneous granuloma annulare Calciphylaxis Subcutaneous thrombotic vasculopathy Cutis laxa Alopecia Infectious skin diseases For more information visit our conference [dermatopathologyconsultations.com]
Anyone who finds unusual moles or other skin growths around the eye or elsewhere on the body should see a dermatologist, a doctor specializing in skin diseases. This is especially important if there is a family history of melanoma. [cancer.net]
On DermNet NZ Spitz naevi – pathology Spitzoid melanoma Birthmarks (naevi) Moles (melanocytic naevi) Congenital melanocytic naevi Blue naevi Halo naevi Freckles Melanoma Other websites Spitz nevus — Medscape Reference Books about skin diseases See the [dermnetnz.org]
- Cutis Laxa
laxa Alopecia Infectious skin diseases For more information visit our conference pages on Dermpedia: Dr. [dermatopathologyconsultations.com]
Psychiatrical
- Fear
The mother appears to have been inordinately fearful of having cancer but her fears were not groundless since, after coming to Tucson to be examined by the former family physician it was discovered that she did have a uterine cancer requiring surgery. [leagle.com]
Strouse JJ, Fears TR, Tucker MA, Wayne AS. Pediatric melanoma: risk factor and survival analysis of the surveillance, epidemiology and end results database. J Clin Oncol. 2005; 23(21):4735-41. Lewis KG. [practicaldermatology.com]
Neurologic
- Confusion
Abstract Medical literature is full of involved and Confusing reports on the genesis, incidence, and development of metastatic melanoma in children. [pediatrics.aappublications.org]
Differential diagnosis : Cellular hemangiomas of infancy are benign tumours whose dense cellularity may lead to confusion with soft tissue sarcomas. [histopathology-india.net]
In addition, incompletely excised Spitz nevi, such as can be seen when the lesion is initially biopsied via a shave procedure, can recur and create diagnostic confusion with an evolving melanoma. [clinicaladvisor.com]
In fact, her "confusion" was more likely precipitated by the conduct of others who did not approve of the child's "life-style." [leagle.com]
- Irritability
However, inflammatory linear verrucous epidermal naevus lesions may be inflamed and irritated; naevus comedonicus lesions may become infected or inflamed. In ENS, neurological involvement may include: Epilepsy or infantile spasms. [patient.info]
Chemical sunscreen agents have the potential to cause more irritation and, rarely, allergic contact dermatitis; therefore, sunscreen that contains only physical sunscreen agents is often recommended for infants and young children and those children with [practicaldermatology.com]
Five of the 10 Spitz nevi with BRAF mutations were altered by more than usual cytologic atypia and/or architectural atypia overlapping with dysplastic nevi, or irritation/inflammation; one desmoplastic Spitz nevus had a BRAF mutation. [thedoctorsdoctor.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]