Dataset Information

Primary central nervous system angiosarcoma: two case reports.

ABSTRACT: Primary angiosarcoma of the brain is extremely rare; only 15 cases have been reported in adults over the last 25 years.We describe two cases of primary angiosarcoma of the brain that are well characterized by imaging, histopathology, and immunohistochemistry. Case 1: our first patient was a 35-year-old woman who developed exophthalmos. Subtotal resection of a left extra-axial retro-orbital mass was performed. Case 2: our second patient was a 47-year-old man who presented to our facility with acute visual loss, word-finding difficulty and subtle memory loss. A heterogeneously-enhancing left sphenoid wing mass was removed. We also review the literature aiming at developing a rational approach to diagnosis and treatment, given the rarity of this entity.Gross total resection is the standard of care for primary angiosarcoma of the brain. Adjuvant radiation and chemotherapy are playing increasingly recognized roles in the therapy of these rare tumors.

SUBMITTER: Hackney JR

PROVIDER: S-EPMC3459733 | biostudies-literature | 2012 Aug

REPOSITORIES: biostudies-literature

ACCESS DATA

Publications

Primary central nervous system angiosarcoma: two case reports.

Hackney James R JR Palmer Cheryl Ann CA Riley Kristen O KO Cure Joel K JK Fathallah-Shaykh Hassan M HM Nabors Louis B LB

Journal of medical case reports 20120821

<h4>Introduction</h4>Primary angiosarcoma of the brain is extremely rare; only 15 cases have been reported in adults over the last 25 years.<h4>Case presentations</h4>We describe two cases of primary angiosarcoma of the brain that are well characterized by imaging, histopathology, and immunohistochemistry. Case 1: our first patient was a 35-year-old woman who developed exophthalmos. Subtotal resection of a left extra-axial retro-orbital mass was performed. Case 2: our second patient was a 47-yea ...[more]

PMID: 22909122

Similar Datasets

Project description:BackgroundCentral nervous system (CNS) melioidosis is rare. However, delayed diagnosis and treatment could lead to fatality. To identify knowledge of CNS melioidosis, we systematically review case reports and case series.Methodology/principal findingsWe searched through PubMed, Web of Science and Thai-Journal Citation Index databases as well as Google Scholar with the last date on July 10, 2018. The diagnosis of CNS melioidosis had to be confirmed with culture, serology or polymerase chain reaction. We excluded the animal cases and the studies that the clinical data were not available. We identified 1170 relevant studies, while 70 studies with a total of 120 patients were analyzed. Ninety-three percent of patients were reported from the endemic area of melioidosis. Median age was 40 years (IQR 18-53), and 70% were men. A total of 60% had one or more risk factors for melioidosis. The median duration from clinical onset to diagnosis was ten days (IQR 5-25). Fever (82%), headache (54%), unilateral weakness (57%) and cranial nerve deficits (52%) are among the prominent presentation. Most patient (67%) had at least one extraneurological organ involvement. The CSF profile mostly showed mononuclear pleocytosis (64%), high protein (93%) and normal glucose (66%). The rim-enhancing pattern (78%) is the most frequent neuroimaging finding in encephalomyelitis and brain abscess patients. Both brainstem (34%) and frontal lobe (34%) are the most affected locations. Mortality rate was 20%.Conclusions/significanceThis study is the most extensive systematic review of case reports and case series of CNS melioidosis in all age groups. However, the results should be cautiously interpreted due to the missing data issue. The propensity of brainstem involvement which correlates with prominent cranial nerve deficits is the characteristic of CNS melioidosis especially encephalomyelitis type. The presenting features of fever and neurological deficits (especially cranial nerve palsies) along with the mononuclear CSF pleocytosis in a patient who lives in the endemic area and also has the risk factor for melioidosis should raise the CNS melioidosis as the differential diagnosis.

Project description:BackgroundLi-Fraumeni syndrome (LFS) is a rare autosomal dominant cancer predisposition syndrome caused by germline TP53 gene mutations. It is characterized by high risk of early-onset cancer, and has been confirmed as associated with multiple tumors clinically. So pediatricians should be more alert to LFS in children with tumors. Choroid plexus carcinoma (CPC) is a rare, malignant tumor which account for less than 1% of all central nervous system (CNS) tumors. However, when such tumorigenesis occurs, it is important to be vigilant for the presence of LFS.Case presentationThe first patient is a 32-month-old boy admitted for convulsions and then was found intracranial space-occupying lesion. Underwent operation, he was diagnosis as choroid plexus carcinoma (WHO Grade III). After 5 months, his elder sister, a 13-year-old girl, was brought to emergency department for confusion and intermittent convulsions. Surgery was performed immediately after head CT examination found the lesion. The pathology result indicated glioblastoma. Because the siblings of the same family have successively suffered from malignant tumors, we performed genetic testing on this family. TP53 gene mutation occurred in both children of these two cases from their father, and their other brother was not spared either. So the two siblings both met the diagnostic criteria of LFS. Then they all received systematic anti-tumor therapy, and follow-up hitherto.ConclusionHere we reported a rare LFS case that two siblings were inherited the same TP53 germline mutations from their father. They suffered from choroid plexus carcinoma and glioblastoma and were finally diagnosed with LFS. In this LFS family, the primary tumors of the two children were both central nervous system tumors, which were not reported in the previous literature. It is suggested that clinicians should be alert to LFS related tumors, which is helpful for early diagnosis. Timely detection of TP53 gene is an important way for early diagnosis of LFS, especially in children with tumor. The incidence of secondary tumor in LFS patients is significantly higher, and other family members of the LFS patient also have an increased risk of suffering from the tumors. Therefore, early diagnosis and timely tumor surveillance can obtain better therapeutic effect and prognosis for both proband and their family.

Dataset Information

Primary central nervous system angiosarcoma: two case reports.

Publications

Primary central nervous system angiosarcoma: two case reports.

Similar Datasets

OmicsDI is part of the ELIXIR infrastructure

Tweets