Perinatal Neurofibromatosis: Two Case Reports and Review of the Literature

Isaacs, Hart

doi:10.1055/s-0029-1241737

Cited by 18 publications

(9 citation statements)

References 33 publications

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“…The tumor produces a dumbbell-shaped mass with cyst formations in both intraorbital and intracranial segments of the nerve. There is a significant association between optic pathway pilocytic astrocytoma and neurofibromatosis type 1 approaching 50 % in some series [9, 30, 34, 67]. This association foretells a poor prognosis.…”

Section: Resultsmentioning

confidence: 99%

Perinatal (fetal and neonatal) astrocytoma: a review

Isaacs

2016

Childs Nerv Syst

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Introduction The purpose of this review is to document the various types of astrocytoma that occur in the fetus and neonate, their locations, initial findings, pathology, and outcome. Data are presented that show which patients are likely to survive or benefit from treatment compared with those who are unlikely to respond. Materials and methods One hundred one fetal and neonatal tumors were collected from the literature for study. Results Macrocephaly and an intracranial mass were the most common initial findings. Overall, hydrocephalus and intracranial hemorrhage were next. Glioblastoma (GBM) was the most common neoplasm followed in order by subependymal giant cell astrocytoma (SEGA), low-grade astrocytoma, anaplastic astrocytoma, and desmoplastic infantile astrocytoma (DIA). Tumors were detected most often toward the end of the third trimester of pregnancy. Conclusion A number of patients were considered inoperable since their tumor occupied much of the intracranial cavity involving large areas of the brain. High-grade astrocytomas were more common than low-grade ones in this review. Fetuses and neonates with astrocytoma have a mixed prognosis ranging from as low as 20 % (GBM) to a high of 90 %. The overall survival was 47/101 or 46 %.

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Section: Resultsmentioning

confidence: 99%

Perinatal (fetal and neonatal) astrocytoma: a review

Isaacs

2016

Childs Nerv Syst

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“…Survivors were reported to develop serious sequelae (e.g. progressive growth of neurofibromas within neck and mediastinum leading to increasing airway obstruction and death; an enlarging proptotic and glaucomatous eye; and occurrence of brain and malignant nerve sheath tumors) especially when there was a strong family history of NF1 [14].…”

Section: Discussionmentioning

confidence: 99%

Pregnancy with Elephantiasis Neurofibromatosa—A Rare Presentation

Gupta¹,

Gupta²,

Sapre³

et al. 2014

OALib

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Neurofibromatosis (NF1) is an autosomal dominant disorder primarily affecting the development and growth of nerve cell tissues. We report a rare case of pregnancy with giant plexiform neurofibroma involving the genital tract with adverse perinatal outcome. A 26-year-old female presented at term in labour with NF1 and a huge plexiform neurofibroma arising from sacral region, gluteal region, pelvis and involving left labium majus. She delivered by cesarean section and the baby had congenital NF1. The baby expired on day 4 of life. To the best of our knowledge, this large size neurofibroma involving this site in pregnancy has never been reported in English literature. Both pregnancy and NF1 have reciprocal adverse effects on each other. Congenital neurofibromatosis can be fatal. Hence, all pregnancies associated with NF1 should be considered as high risk and monitored carefully.

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“…When carefully examined, approximately half of affected children meet these criteria by the end of the 1st year of life; nearly all patients are diagnosable by 5 years of age [132,136]. Rarely, the syndrome may manifest in utero or at birth, which usually portends a poor prognosis [137]. The earliest clinical signs (café-au-lait macules, axillary freckling, and Lisch nodules) are indicative of melanocytic hyperplasia and/or abnormal melanosome synthesis [124,128,129,132,133,136,138,139], Plexiform neurofibromas, which affect up to 50% of NF1 patients, often come to clinical attention before 5 years of age; occasionally they are multiple or congenital [122,124,133,139–141].…”

Section: Benign Neoplasmsmentioning

confidence: 99%

Neurogenic Tumors of Soft Tissue

Cates

Coffin

2012

Pediatr Dev Pathol

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Neurogenic tumors are an uncommon yet important category of soft tissue tumors in children and adolescents because of their frequent association with various genetic syndromes. The heterogeneous cellular composition of the peripheral nerve and the wide metaplastic capacity of the neural crest and its derivatives generate a variety of neoplasms with neurogenic differentiation. This article reviews the clinicopathologic features and differential diagnosis of neurogenic tumors in the first two decades of life, and highlights use of selected ancillary methods for diagnosis.

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Perinatal Neurofibromatosis: Two Case Reports and Review of the Literature

Cited by 18 publications

References 33 publications

Perinatal (fetal and neonatal) astrocytoma: a review

Perinatal (fetal and neonatal) astrocytoma: a review

Pregnancy with Elephantiasis Neurofibromatosa—A Rare Presentation

Neurogenic Tumors of Soft Tissue

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