Congenital Gliosarcoma: Detailed Clinicopathologic Documentation of a Rare Neoplasm

Hocwald, Ori; McFadden, Deborah E.; Osiovich, Horacio; Dunham, Christopher

doi:10.2350/09-01-0592-oa.1

Cited by 8 publications

(1 citation statement)

References 12 publications

(13 reference statements)

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“…18 Notably, paediatric cases of gliosarcoma are uncommon and congenital cases of gliosarcoma are very rare. [19][20][21] Pathologically, the areas of high-grade astrocytoma and sarcoma in 21 The sarcomatous component of gliosarcoma may also take on the appearance of malignant fibrous histiocytoma, chondrosarcoma or osteosarcoma. 23 Rarely, epithelial areas may be seen, suggesting carciomatous differentiation.…”

Section: Gliosarcoma -What Can Be Learned From Adult Cases?mentioning

confidence: 99%

Gliosarcoma – Clinico-pathology, Genetics and a Review of Rare Congenital Cases

Dunham¹

2009

European Oncology &Amp; Haematology

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Congenital brain tumours are rare. In general, they are preferentially located in the supratentorial compartment, and despite the occurrence of low-grade entities, these tumours are associated with a very poor prognosis. When strictly defined, the most common forms of congenital neoplasia are teratomas and astrocytomas. Among the astrocytomas, all grades (World Health Organization [WHO] I–IV) and many of the different subtypes are represented. This includes the most prognostically worrisome ‘diffusely infiltrating’ astrocytomas. Gliosarcomas are a variant of glioblastoma (i.e. WHO grade IV astrocytoma) that exhibits both malignant astrocytic (i.e. glioblastoma) and mesenchymal (i.e. sarcoma) components. Despite their bi-phasic histology, genetic analyses of adult gliosarcoma cases suggest not only a molecular profile similar to glioblastoma, but also a monoclonal histogenesis for the glial and sarcomatous elements. Congenital gliosarcomas are extremely rare, with only a handful of cases being described in the literature. Not surprisingly, therefore, detailed clinical, pathological and genetic data are limited. However, based on a recent analysis of congenital glioblastomas, congenital gliosarcomas may constitute an entity that is genetically and prognostically distinct from adult cases.

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Section: Gliosarcoma -What Can Be Learned From Adult Cases?mentioning

confidence: 99%

Gliosarcoma – Clinico-pathology, Genetics and a Review of Rare Congenital Cases

Dunham¹

2009

European Oncology &Amp; Haematology

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Congenital tumors of the central nervous system

et al. 2010

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Congenital tumors of the central nervous system (CNS) are often arbitrarily divided into "definitely congenital" (present or producing symptoms at birth), "probably congenital" (present or producing symptoms within the first week of life), and "possibly congenital" (present or producing symptoms within the first 6 months of life). They represent less than 2% of all childhood brain tumors. The clinical features of newborns include an enlarged head circumference, associated hydrocephalus, and asymmetric skull growth. At birth, a large head or a tense fontanel is the presenting sign in up to 85% of patients. Neurological symptoms as initial symptoms are comparatively rare. The prenatal diagnosis of congenital CNS tumors, while based on ultrasonography, has significantly benefited from the introduction of prenatal magnetic resonance imaging studies. Teratomas constitute about one third to one half of these tumors and are the most common neonatal brain tumor. They are often immature because of primitive neural elements and, rarely, a component of mixed malignant germ cell tumors. Other tumors include astrocytomas, choroid plexus papilloma, primitive neuroectodermal tumors, atypical teratoid/rhabdoid tumors, and medulloblastomas. Less common histologies include craniopharyngiomas and ependymomas. There is a strong predilection for supratentorial locations, different from tumors of infants and children. Differential diagnoses include spontaneous intracranial hemorrhage that can occur in the presence of coagulation factor deficiency or underlying vascular malformations, and congenital brain malformations, especially giant heterotopia. The prognosis for patients with congenital tumors is generally poor, usually because of the massive size of the tumor. However, tumors can be resected successfully if they are small and favorably located. The most favorable outcomes are achieved with choroid plexus tumors, where aggressive surgical treatment leads to disease-free survival.

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Gliosarcoma

2016

Diagnostic Imaging: Brain

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Congenital Gliosarcoma: Detailed Clinicopathologic Documentation of a Rare Neoplasm

Cited by 8 publications

References 12 publications

Gliosarcoma – Clinico-pathology, Genetics and a Review of Rare Congenital Cases

Gliosarcoma – Clinico-pathology, Genetics and a Review of Rare Congenital Cases

Congenital tumors of the central nervous system

Gliosarcoma

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