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Juvenile Xanthogranuloma


Presentation

  • Although this benign disorder typically presents with a favorable clinical outcome, the unusual presentation as an intratesticular lesion can lead to diagnostic challenges.[ncbi.nlm.nih.gov]
Soft Tissue Swelling
  • Based on these results, juvenile xanthogranuloma should be included in the differential diagnosis of oral yellowish soft-tissue swellings in children and adolescents and should be managed through conservative surgical excision.[ncbi.nlm.nih.gov]
Lower Extremity Pain
  • A 28-year-old woman presented with back pain and worsening lower extremity pain, numbness, and weakness. Magnetic resonance imaging demonstrated an enhancing lumbar mass. However, at surgery, no discrete mass was identified.[ncbi.nlm.nih.gov]
Dentist
  • Patients with these rare intraoral lesions may present to dentists and subsequently to oral and maxillofacial surgeons. Diagnosis requires histopathological analysis and immunohistochemical staining.[ncbi.nlm.nih.gov]
Hoarseness
  • A 3-year-old girl presented with hoarseness and inspiration stridor. A bulky tumor was found in right glottic to subglottic region.[ncbi.nlm.nih.gov]
Neck Pain
  • At 3-month follow-up, he was asymptomatic except for mild neck pain. MRI scan of the cervical spine done at follow-up, revealed complete excision of tumor without any residual lesion.[ncbi.nlm.nih.gov]
Back Pain
  • A 28-year-old woman presented with back pain and worsening lower extremity pain, numbness, and weakness. Magnetic resonance imaging demonstrated an enhancing lumbar mass. However, at surgery, no discrete mass was identified.[ncbi.nlm.nih.gov]
Ptosis
  • We report a rare case of juvenile xanthogranuloma in a 16-year-old girl who presented with a neck mass and left-sided ptosis 2.5 months previously.[ncbi.nlm.nih.gov]
Blepharoptosis
  • MRI in a 2-year-old female presenting afebrile seizures and left blepharoptosis revealed multiple well-marginated round-shaped lesions, isointensity to gray matter on T1- and T2-weighted images with homogenously reduced diffusion and diffuse contrast[ncbi.nlm.nih.gov]
Cranial Nerve Involvement
  • A 3.5-year-old girl underwent subtotal resection of a tumor extending from the left Meckel's cave and invading the cavernous sinus and left orbit with extensive cranial nerve involvement.[ncbi.nlm.nih.gov]
Urinary Retention
  • A 18-year-old male presented with neck pain for 3 months, along with progressive quadriparesis and sensory loss of 2 months duration with urinary retention. Motor examination revealed spastic quadriparesis with power of 2/5 in all the 4 limbs.[ncbi.nlm.nih.gov]
Incontinence
  • METHODS: The patient is a 47-year-old woman who presented with bowel and bladder incontinence.[ncbi.nlm.nih.gov]

Workup

  • These data highlight the importance of identifying specific MAPK pathway alterations as part of the diagnostic workup for patients with histiocytic disorders rather than initiating empiric treatment with MEK inhibitors.[ncbi.nlm.nih.gov]
  • In Neurofibromatosis 1, JXGs are also more prevalent so workup for this genetic disease may be initiated with the discovery of JXG.[perridermatology.com]
  • Laboratory tests and autoimmune workup were completed in 7 patients.[docksci.com]
  • The varied diagnostic workup and clinical outcome illustrate difficulties that may be encountered with this entity.[healio.com]
Increased Bone Density
  • Author information 1 Nationwide Children's Hospital, Columbus, OH 43205, USA. saeeda.almarzooqi@uaeu.ac.ae Abstract Osteopetrosis (OP) is a clinically and genetically heterogeneous disorder characterized by increased bone density.[ncbi.nlm.nih.gov]

Treatment

  • The initial neurological symptoms resolved without any surgical or medical treatment.[ncbi.nlm.nih.gov]
  • Treatments that have been used include surgical excision, intralesional steroid injection, cryotherapy, and low dose radiotherapy. In the case of a resistant or reoccurring lesion, chemotherapy has been used as a treatment.[en.wikipedia.org]

Prognosis

  • The patient was thus expected with benign prognosis, and additional resection of the tumor including vocal fold was not indicated in the initial treatment. Six weeks later, the JXG recurred and a second procedure using CO₂ laser was needed.[ncbi.nlm.nih.gov]

Etiology

  • The etiology of juvenile xanthogranuloma is uncertain, although the occurrence in monozygotic twins may suggest genetic predisposition. 2010 Wiley Periodicals, Inc.[ncbi.nlm.nih.gov]
  • The exact etiology is unclear, but delayed-type hypersensitivity may be implicated in the pathogenesis. Complete surgical excision appears to be curative. No recurrences have been reported.[humpath.com]
  • J Otol Rhinol 6:3. doi: 10.4172/2324-8785.1000313 Abstract Juvenile xanthogranuloma (JXG) is a benign self-limiting non langerhans cell histiocytic lesion of unknown etiology and pathogenesis.[scitechnol.com]
  • Etiology and Pathophysiology The pathogenesis is unknown.[accesspediatrics.mhmedical.com]

Epidemiology

  • The epidemiology, clinical characteristics, histopathology and management of juvenile- and adult-onset corneoscleral limbus xanthogranuloma. Graefes Arch Clin Exp Ophthalmol. 2016 Mar. 254 (3):413-20. [Medline].[emedicine.com]
  • Epidemiology Uncommon ( 0.5% of pediatric tumors in one study) Usually infants (median age 5 months) with a congenital mark, although 10 - 30% occur in adults Male / female 1.4 : 1 Clinical features Skin of face or trunk, less commonly in subcutis, skeletal[pathologyoutlines.com]
  • Epidemiology This is predominantly a disorder of the first two decades of life, 45% 2 to 75% 3 of patients developing the lesions as infants, 95% by the age of 10 years 3.[e-immunohistochemistry.info]
Sex distribution
Age distribution

Pathophysiology

  • Etiology and Pathophysiology The pathogenesis is unknown.[accesspediatrics.mhmedical.com]
  • Pathophysiology The etiology of JXG is unknown. JXG is believed to result from a disordered macrophage response to a nonspecific tissue injury, resulting in a granulomatous reaction.[emedicine.com]

Prevention

  • However, a local excision was required because the mass had a risk of malignancy in terms of appearance of ulceration, prevention from his visual field as right gaze, and avoiding dacryoma.[karger.com]
  • Intraocular involvement often requires treatment with injectable or topical corticosteroids to prevent anterior chamber hemorrhage and glaucoma. Only when organ function is compromised in the context of systemic involvement is treatment indicated.[dermatologyadvisor.com]
  • Transplantation Substance Use and Addiction Surgery Surgical Innovation Surgical Pearls Teachable Moment Technology and Finance The Rational Clinical Examination Tobacco and e-Cigarettes Toxicology Trauma and Injury Treatment Adherence United States Preventive[jamanetwork.com]

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