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Metastatic alveolar soft part sarcoma presenting as a dural-based cerebral mass.

Sarcoma metastatic to the brain is uncommon and rarely occurs as the initial manifestation of tumor. Alveolar soft-part sarcoma, a rare but well studied subtype of a soft tissue sarcoma with a propensity for central nervous system invasion, presenting with brain metastases, has been reported only once previously. We report the case of a 28-year-old man who presented with partial seizures and who was found to have a homogeneously enhancing frontal lesion on a broad dural base disclosed by computed tomography. preoperatively, the lesion was thought to be a meningioma. The tumor was excised easily and had features typical of an alveolar soft-part sarcoma, which were revealed by light and electron microscopy as well as immunohistochemical analysis. Multiple lung nodules compatible with metastases were found on a chest film. Meningeal dissemination has been reported in a variety of sarcoma types, including rhabdomyosarcoma, fibrosarcoma, and leiomyosarcoma. We add alveolar soft-part sarcoma to this list and suggest that increased recognition of the propensity for these tumors to exhibit metastatic spread to the dura should eliminate diagnostic confusion and provide an earlier diagnosis of these rare lesions. The patterns of spread in metastatic sarcoma deserve further study.

Adult↗

Primary intracranial fibrosarcoma with intratumoral hemorrhage: neuropathological diagnosis with review of the literature.

Primary intracranial sarcomas are rare tumors of mesenchymal origin in the central nervous system (CNS). Spontaneous hemorrhage, while not an uncommon presentation of brain tumors, has not been described in primary cerebral sarcoma. We report the case of a 43 year old woman presenting with spontaneous hemorrhage into a primary cerebral fibrosarcoma, and discuss this case in the context of the diagnostic criterions of these rare tumors previous reports of intracranial sarcomas and mechanisms of intratumoral hemorrhage.

Adult↗

Increased incidence of cerebral metastases in sarcoma patients with prolonged survival from chemotherapy. Report of cases of leiomysarcoma and chondrosarcoma.

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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Transformation of rat cerebral endothelial cells by Rous sarcoma virus.

Rat cerebral microvascular endothelial cells were infected with Schmidt-Ruppin Rous sarcoma virus-strain D (SR-RSV-D), an avian retrovirus. A single focus of transformed cells was isolated and the resultant cell line designated RCE-T1. The specificity for SR-RSV-D transformation was determined by virus rescue assay and demonstration of virus-specific antigens. RCE-T1 cells are virogenic when fused with chicken embryo fibroblasts (CEF) and do not produce infectious virus as demonstrated by the absence of detectable virus in culture fluid from these cells alone. Studies using an enzyme-linked immunosorbent assay (ELISA) for avian retrovirus-coded internal proteins show that RSV-transformed endothelial cells contain mainly p27 and react to some extent to p19 and p15 viral antigens. These data demonstrate conclusively that the transformation event was indeed due to SR-RSV-D. In addition, chromosome analysis confirmed these cells to be of rat origin. RSV-transformed endothelial cells express the typical array of transformation-related properties such as anchorage-independent cell growth in soft agar, decreased cell adhesiveness, ability to grow in low serum, and capability of producing tumors in newborn rats. Demonstration of differentiated endothelial characteristics included positive immunofluorescent staining for factor VIII antigen and angiotensin-converting enzyme and histochemical localization of gamma-glutamyl transpeptidase activity. This cell line should provide a useful model to study not only specialized biochemical and other functional characteristics of cerebrovascular endothelium but also the cellular mechanisms that involve the transition from normal to neoplastic expression.

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Cerebral reticulum cell sarcoma after multiple renal transplants.

A case is reported of a 34 year old white male with chronic renal failure secondary to glomerulonephritis who received four renal transplants over a period of five years. He died 25 months after the fourth transplant. Necropsy revealed a reticulum cell sarcoma-microglioma of the brain. The possibility that multiple transplants may have had a synergistic effect in the development of a malignant cerebral lymphoma in this patient is briefly discussed in the light of the current theories concerning the pathogenesis of the tumours in transplant recipients and in the context of the present therapeutic approach to graft rejection.

Adult↗

Synovial sarcoma--unusual presentation with cerebral hemorrhage.

INTRODUCTION: Soft-tissue sarcomas account for 1% of all malignancies in adults. Typical symptoms comprise a growing and painless mass. Occasionally, the diagnosis is facilitated by secondaries. MATERIALS AND METHODS: Here we report the first case presented with intracranial hemorrhage caused by a metastasis of a previously unknown synovial sarcoma. The 74-year-old female patient was under anticoagulation for recurrent thrombosis of the right popliteal vein due to compression by the sarcoma, misdiagnosed as Baker's cyst. The brain metastasis was resected, and after an incisional biopsy of the suspected tumor, which confirmed the diagnosis of a synovial sarcoma, an above knee amputation was performed because of the invasion of the neurovascular structures in the popliteal fossa. RESULTS: The patient died 11 months after the initial event from pulmonary and further brain metastases. CONCLUSION: Brain metastases are seldom the primary factor in the management of advanced synovial sarcoma. Initially, the primary tumor was thought to be a benign cyst; we therefore recommend a biopsy/excision of lesions greater than 5 cm in size and with growth tendency to allow appropriate treatment.

Aged↗

[A case of alveolar soft-part sarcoma with recurrent cerebral metastasis--operative management of the cerebral metastasis].

A female patient noticed a painless swelling at the right thigh at the age of 12. Two years later, she had total removal for the tumor and irradiation. The diagnosis of the tumor was alveolar soft-part sarcoma. At the age of 16 she had left pneumonectomy for its metastasis. The next year the metastatic tumor is detected in the right lung. At 21 she was admitted to our service with a diagnosis of cerebral metastasis. Her chief complaints were headache, nausea and visual disturbance. Neurological examination revealed advanced choked disc and left homonimous hemianopsia. CT scan revealed a large vascular tumor in the right occipital lobe and chest film showed multiple metastatic shadows in right lung. A large bloody tumor was removed totally through right occipital craniotomy. She was discharged with a marked improvement of neurological symptom. Since then she had had 8 times of surgical removal for 10 cerebral metastatic tumors. Several chemotherapy was attempted for three times. At first adriamycin was administered after the first removal of cerebral metastasis. Methyl-CCNU and Picibanil was administered after the second removal. Soon after the chemotherapy, metastatic tumor recurred. Cisplatin was administered for the ninth metastasis. None of these chemotherapy had seemed to be benefit in the management. At 27 she had removal of metastatic tumor on chest wall. She died of respiratory embarrassment in thorax at 30. At autopsy she had metastatic tumors in right frontal lobe, right lung, mediastinum, heart, chest wall, and intestine. During her illness, she graduated from high school and college, and got a good job as a secretary.

Adult↗

Cardiopulmonary metastatic lesions of osteosarcoma and associated cerebral infarction.

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.

Adult↗

Uveitis and cerebral reticulum-cell sarcoma (large-cell lymphoma). Case report.

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.

Aged↗

[A case of alveolar soft-part sarcoma with lung and cerebral metastasis (author's transl)].

A 37-year-old housewife who had episodes of surgical removal for an alveolar soft-part sarcoma of her right leg 15 years ago, and total removal for right lung metastasis 2 years ago, was admitted to our service with complaints of headache, vomiting, dyscalculia and recent memory disturbance which started about 4 months prior to admission. Neurological examination revealed papilledema and disorientation. Left carotid angiography and CT scan showed a ball-like vascular tumor in the left frontal lobe. Presumptive diagnosis of metastatic brain tumor in the left frontal lobe was made in our service. Left fronto-parietal craniotomy was carried out on Feb. 22, 1977 and total removal for the metastatic tumor were made. Specimens of the tumor showed typical feature of alveolar soft-part sarcoma histologically. Postoperative course was well and she was discharged without neurological deficits. Two years after the previous surgery for cerebral metastasis, another metastatic tumor was found in the left frontal lobe again. Total removal for this solitary metastatic tumor was performed again. Histological and electron microscopical examination was made with this tumor specimen. Typical feature of alveolar soft-part sarcoma was confirmed. She was discharged with a marked improvement of her symptoms. At present, it has been passed about a year after the second surgery on her, she is doing well without neurological deficits. Clinical features of alveolar soft-part sarcoma were discussed, especially with the comparison of clinical feature between the reported cases of Japan and the cases of foreign countries. Regarding the choice of the treatment of this tumor, total removal was seemed to be the best management. Therefore, all efforts should be focussed to discover in the earlier stage of this tumor.

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