Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027819 (neuroblastoma)
 27,800 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Neurogenic tumors of the neck occur in children and adults. Important parameters to aid in the differential diagnosis are age at presentation, location, and a history of NF or multiple endocrine neoplasia. Schwannoma is the most common solitary neurogenic tumor in the neck and is usually seen in patients between 20 and 50 years of age. The plexiform neurofibroma and multiple localized neurofibromas are characteristic of NF1. MPNSTs are uncommon aggressive lesions that can arise de novo in patients with NF (10% to 30%) and postirradiation. Neuroblastic tumors consist of neuroblastoma, ganglioneuroblastoma, and ganglioneuroma. These tumors typically arise in the chest and abdomen but occasionally present as a primary neck mass. A neck mass with a histologic diagnosis of neuroblastoma is, however, more commonly metastatic from an abdominal neuroblastoma.
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PMID:Neurogenic tumors of the neck. 1105 70

While pediatric orbital tumors are most often managed in tertiary care centers, clinicians should be aware of the signs of intraocular and orbital neoplasms. In the pediatric population, a delay in diagnosis of orbital and intraocular lesions, even if benign, can lead to vision loss and deformity. Intraocular lesions reviewed are retinoblastoma, medulloepithelioma, and retinal astrocytic hamartoma. Orbital neoplasms reviewed are rhabdomyosarcoma, neuroblastoma metastases, optic pathway glioma, plexiform neurofibroma, leukemia, lymphoprolipherative disease, orbital inflammatory syndrome, dermoid and epidermoid inclusion cysts, and Langerhans' cell histiocytosis. Vascular lesions reviewed are infantile hemangioma and venous lymphatic malformation. In conjunction with clinical examination, high-resolution ophthalmic imaging and radiologic imaging play an important role in making a diagnosis and differentiating between benign and likely malignant processes. The radiologic imaging characteristics of these lesions will be discussed to facilitate prompt diagnosis and treatment. The current treatment modalities and management of tumors will also be reviewed.
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PMID:A clinical update and radiologic review of pediatric orbital and ocular tumors. 2357 29

A 15-month-old girl was initially referred for endocrine evaluation for clitoromegaly and subsequently found to have an adrenal mass that tracked along the paravertebral region and was associated with increased vascular markings along the skin. Neurologic examination was normal. Magnetic resonance imaging of the chest mass demonstrated a serpiginous lesion along the intercostal margins. Initial differential diagnosis included neuroblastoma, ganglioneuroblastoma, vascular lesion, or nerve sheath tumor. Biopsy was consistent with plexiform neurofibroma. Subsequent examination revealed features consistent with neurofibromatosis type 1. A review of clinical features of NF1 is provided in the case report.
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PMID:A 15-Month-Old Girl Presenting With Clitoromegaly and a Chest Mass. 2996 6