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Four cases of cerebral fibrosarcomas occurring in two families are reported. To our knowledge, no similar cases have been reported. Evidence that a heritable factor is involved in the genesis of some brain tumors is suggested by these cases.
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The Authors present six cases of sarcoma of the brain. After doing a short review of the cases described in literature, they discuss the clinical anathomo-pathological and terapeutic aspects of these peculiar tumours.
Primary cerebral reticulum cell sarcoma appeared as unexplained posterior uveitis. A retrospective review of 19 cases of cerebral reticulum cell sarcoma seen at Massachusetts General Hospital has not confirmed the previously described high incidence of ocular involvement in patients with this tumor. The presence of posterior uveitis remote from a definite cerebral mass should suggest the possible diagnosis of primary cerebral reticulum cell sarcoma.
An anatomo-clinical study is presented of a reticulum cell sarcoma (or lymphosarcoma), limited to the eye-ball and the brain. The eye disease developed as a chronic, diffused hypertensive uveitis. The diagnosis of the nature of the brain tumour was made by cytological study of the cerebro-spinal fluid.
A case of acute non lymphoblastic leukemia in a 9 year-old girl is reported. This case presented with intracranial hypertension with exophthalmos and parietal subcutaneous tumor; imaging techniques showed their subcutaneous, orbital and intracranial localizations. Complete remission was obtained within 7 months with polychemotherapy. The rare cases of granulocytic sarcoma of central nervous system in children are reviewed.
A case of primary sarcoma that arose in the right temporal lobe of a 59-year-old man is reported. The patient had a history of resection of a "pituitary tumor" and postoperative radiotherapy about 40 years earlier. The cerebral tumor consisted of loose and diffuse proliferation of large pleomorphic cells in a matrix showing a markedly myxoid change and containing a variable amount of delicate collagen fibers. Some tumor cells had a large intracytoplasmic vacuole containing acid mucopolysaccharide, thus featuring "pseudolipoblasts." Differentiation along other specific mesenchymal lineages was not found. Many tumor cells contained densely eosinophilic hyaline globules in the cytoplasm. Tumor cells showed a loss of the nuclear expression of H3K27me3. Although cerebral sarcomas showing similar histopathological features have not been previously reported, a markedly myxoid change of the stroma admixed with delicate collagen fibers and "pseudolipoblasts" suggests a close pathological kinship to myxofibrosarcoma. Another notable finding was the appearance of many hyaline globules, which has been considered a characteristic finding of primary intracranial sarcoma, DICER1-mutant. In the cytogenetic study of the present case, however, no DICER1 gene mutations were demonstrated.
Soft tissue and bony sarcomas rarely metastasize to the central nervous system, particularly to the cerebral hemispheres. In 456 patients with metastatic sarcoma, only 6 (1.3%) had cerebral metastases documented by brain scan at the time of referral for chemotherapy. Adriamycin-containing combination chemotherapeutic regimens have led to a significant increase in the median survival of patients from the start of chemotherapy (18 + months for responders compared, to 7 months in nonresponders). Of 14 patients relapsing after a response or stabilization of disease of 6 months or greater, the cause of relapse was the development of cerebral metastases in 5 (36%). Two of these cases, one a patient with leiomyosarcoma and one with chondrosarcoma, were documented by autopsy and are reported in detail because of their rarity in the medical literature. Although the numbers are small, the increased incidence of cerebral metastases in the group relapsing after a lengthy response suggests that improved chemotherapy for sarcomas resulting in improved survival may be chaning the pattern of metastatic disease, and may require new the;apeutic approaches.
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Osteogenic sarcoma frequently disseminates by hematogenous routes. A 32-year-old patient underwent evaluation for an acute cerebral infarction. Cardiac auscultation disclosed an abnormal diastolic sound. Echocardiographic examination revealed a large left atrial mass, which was found at thoracotomy to be metastatic osteogenic sarcoma. Cerebral computed tomographic scans at the time of initial examination and 3 months later demonstrated new cerebral lesions consistent with metastatic growths. The abrupt cerebral infarction, other clinical findings, and results of diagnostic studies strongly suggested that the acute cerebrovascular event was the result of metastatic cerebral embolization from the tumor material found in the thorax. Cerebral infarction is an unusual and catastrophic complication of thoracic metastatic lesions of osteogenic sarcoma.
A patient with a peculiarly refractoy uveitis and vitritis was later found to have an infiltrative mass lesion of the posterior portion of the corpus callosum. The histopathological diagnosis of reticulum-cell sarcoma (large-cell lymphoma) was made after study of vitreous tissue obtained by pars plana vitrectomy of the right eye. Intraocular involvement with cerebral reticulum-cell sarcoma has been reported previously.
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Two patients with cerebral reticulum cell sarcoma (CRCS) are reported in whom neurologic abnormalities and radiologic (computerized tomographic [CT] scan) evidence of tumor remitted. In one patient, remission followed craniectomy and corticosteroid therapy and lasted for eight months. In the other patient, at least four remissions occurred over a span of seven years, each in conjunction with the administration of corticosteroids. Corticosteroids may favorably alter the biologic activity of tumor tissue in some cases of CRCS, predisposing to clinical remission and disappearance of tumor on CT scan.
A case of Ewing's sarcoma with intracranial metastasis is reported with special reference to positron emission tomography (PET) evaluation of cerebral hemocirculation and metabolism after brain irradiation.
An unusual neurological syndrome occurred in 4 of 158 patients treated for osteogenic sarcoma with combination chemotherapy. There was an abrupt onset of focal cerebral deficits approximately ten days after chemotherapy with vincristine and high-dose methotrexate plus citrovorum factor rescue. The syndrome was short lived and always occurred early in the course of treatment. Prolonged neurological deficits remained in 2 patients. When similar chemotherapy was reinstituted in the 4 patients, no further neurological complications ensued. Possible causes include a leukoencephalopathy related to methotrexate or an embolic cerebral vasculopathy related to necrotic tumor microemboli emanating from the lungs.
A forty year old male patient presented with swollen, deformed right hand with multiple irregular ulcers. The axillary lymph nodes were enlarged, firm and tender. The biopsy of nodules present on the hand revealed areas of haemorrhage and necrosis. The histopathological examination confirmed the clinical diagnosis of epithelioid sarcoma. Following below-elbow amputation, patient failed to regain consciousness and expired. On autopsy, distant metastases were found in the regional lymph nodes, pleura, kidney and cerebrum.
A case of Kaposi's sarcoma involving the brain is described, this being the first of its kind in Uganda. The patient was a 40-year-old woman who presented with a 2-year history of skin tumors on the left ankle that proved resistant to chemotherapy. She therefore underwent an above-knee amputation. Six months after amputation, she developed signs of cerebral involvement, and 6 weeks later she died. Necropsy showed large tumor deposits in bone and lungs as well as in the brain. Histologically the cutaneous tumors consisted of the monomorphic variety, whereas the cerebral and other deposits were anaplastic. In the three cases of cerebral Kaposi's sarcoma hitherto reported from other countries, only minute foci of tumor were found in the brain.
This study describes the preliminary characterization of a Rous sarcoma virus (RSV)-transformed rat cerebral microvascular endothelial cell (RCE-T1)-derived growth factor with biological properties similar to transforming growth factor-beta (TGF-beta). A significant portion of this RCE-T1-derived TGF-beta-like growth factor was secreted in an active form and its secretion was elevated 2- to 3-fold in late passage cultures comprised of rapidly growing, less differentiated cells exhibiting reduced sensitivity to the growth-inhibitory effects of exogenous TGF-beta 1. These data suggest that the RCE-T1 cell line may be a useful system in which to study the role of autocrine TGF-beta production in normal and aberrant endothelial cell growth.