Primary central nervous system angiosarcoma with recurrent acute subdural hematoma.

Angiosarcoma is an infrequent tumor among sarcomas, especially presenting as a primary tumor within the central nervous system, which can lead to a rapid neurological deterioration and death in few months. We present a 41-year old man with a right frontal enhancing hemorrhagic lesion. Surgery was performed with histopathological findings suggesting a primary central nervous system angiosarcoma. He was discharged uneventfully and received adjuvant chemotherapy and radiotherapy. At 5 months, the follow-up MRI showed two lesions with an acute subdural hematoma, suggesting a relapse. Surgery was again conducted finding tumoral membranes attached to the internal layer of the duramater around the right hemisphere. The patient died a few days later due to the recurrence of the subdural hematoma. This case report illustrates a rare and lethal complication of an unusual tumor. The literature reviewed shows that gross-total resection with adjuvant radiotherapy seems to be the best treatment of choice.

Primary central nervous system angiosarcoma with recurrent acute subdural hematoma.

Angiosarcoma is an infrequent tumor among sarcomas, especially presenting as a primary tumor within the central nervous system, which can lead to a rapid neurological deterioration and death in few months. We present a 41-year old man with a right frontal enhancing hemorrhagic lesion. Surgery was performed with histopathological findings suggesting a primary central nervous system angiosarcoma. He was discharged uneventfully and received adjuvant chemotherapy and radiotherapy. At 5 months, the follow-up MRI showed two lesions with an acute subdural hematoma, suggesting a relapse. Surgery was again conducted finding tumoral membranes attached to the internal layer of the duramater around the right hemisphere. The patient died a few days later due to the recurrence of the subdural hematoma. This case report illustrates a rare and lethal complication of an unusual tumor. The literature reviewed shows that gross-total resection with adjuvant radiotherapy seems to be the best treatment of choice.

Imaging and Radiologic-Pathologic Correlation in Granular Cell Astrocytomas: Report of 2 Cases.

BACKGROUND: Granular cell astrocytoma is a rare and aggressive subtype of astrocytoma that is histopathologically well defined in the literature. It is formed by polygonal cells with granular cytoplasm mixed with neoplastic astrocytes and usually a perivascular infiltrate of lymphocytes. Despite its unusual histologic appearance, relevant radiologic features have not yet been described. CASE DESCRIPTION: We report 2 middle-aged patients with neurologic symptoms secondary to a newly diagnosed brain tumor. The absence of central tumor necrosis as well as the presence of an atypical pattern of enhancement and areas of intense diffusion restriction on magnetic resonance imaging in both cases led to the diagnosis of primary central nervous system lymphoma. Histopathologic findings in both tumors showed an aggressive astrocytoma with a prominent granular cell population and perivascular lymphocytic cuffing in tissue, corresponding to a granular cell astrocytoma. Despite the favorable prognostic factors, including World Health Organization grades II and III astrocytomas and IDH mutations, the outcome was poor. CONCLUSIONS: Granular cell astrocytomas can show unusual aggressive radiologic features that do not correspond to their histopathologic grade of malignancy. The presence of perivascular lymphocytic infiltrate may alter the typical radiologic appearance of common astrocytomas.

Management of congenital midline nasofrontal dermoid cysts in two identical twins: Case report.

Midline dermoid cysts are uncommon lesions that can lead to severe complications when an intracranial extension exists. We report the cases of two twin sisters referred to surgery for removal of the masses and the intracranial extension. They represent an additional example of familial nasal dermoid cysts, providing further support for a genetic basis for the disorder.