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✓主要骨骼非霍奇金淋巴瘤是罕见的。The authors report a case of a small lymphocytic B-cell lymphoma of the skull occurring in a 53-year-old man who presented with right-hand apraxia. Initial computed tomography and magnetic resonance imaging revealed a hematoma-mimicking lesion in the left frontoparietal subdural area. A frontotemporoparietal craniectomy and biopsy procedure yielded a diagnosis of small lymphocytic B-cell lymphoma, with a metastatic nodule in the retrobulbar area. Three years after undergoing radiation therapy and surgery, the patient has shown neurological improvement without systemic dissemination of the malignancy. The lesion in this case was misdiagnosed as a subdural hematoma, and shows the importance of including lymphoma in the differential diagnosis of subdural mass lesions.

✓ The authors present the clinical, radiological, pathological features, and autopsy findings of a patient with malignant triton tumor of the acoustic nerve, which probably arose from a pre-existing acoustic schwannoma. The term “malignant triton tumor” is applied to malignant schwannomas with rhabdomyoblastic differentiation. A cerebellopontine angle tumor with spinal drop metastasis occurred in this patient 10 months after near-total removal of the original tumor.

Kaposiform血管内皮瘤(KHE)是一种罕见的弗吉尼亚州scular tumor of infancy and early childhood that occurs mainly in soft tissue and the retroperitoneum. The pathological characteristics of a KHE are fascicles of spindleshaped endothelial cells and slitlike vascular channels with irregular tumor margins. In spite of benign features such as rare mitoses and a low MIB-1 labeling index, a KHE is categorized as an intermediate malignancy because of local invasiveness into adjacent lymph nodes or organs. Resection is the treatment of choice for this type of tumor and adjuvant medical therapy is prepared for the tumor remnants and the associated Kasabach-Merritt syndrome. In this paper the authors report a case of a KHE without Kasabach-Merritt syndrome arising from the right tentorium cerebelli in a 13-month-old male child. The biological behavior of the tumor, including its pathological traits and clinical course, was malignant in nature. This case may be not only the first intracranial extraaxial KHE, but also the first truly malignant variant. Kaposiform hemangioendothelioma should be considered in the differential diagnosis of intracranial and extraaxial tumors.

✓Fetus-in-fetu is a very rare condition in which one fetus is contained within another. About 100 cases have been reported, and in most of these the fetus was located in the retroperitoneum. The authors describe an extremely rare case of an intracranial fetus-in-fetu in an extraaxial location. This is the eighth intracranial fetus-in-fetu to be reported, the first intracranial extraaxial case, and involves the oldest documented patient with this condition.

Histopathological analysis of the mass revealed a degenerated amnionic membranelike tissue, well-differentiated extremities (including fingerlike structures), skin, matured lungs, well-formed intestines, cerebellar and cerebral tissue, and a notochord with ganglion cells. DNA analysis using short tandem repeat polymorphisms confirmed that the fetus-in-fetu mass and the host infant had heterozygous alleles and were of identical sex and genotype.

Meningiomas are rare in children. Chordoid meningioma is a very rare variant, as only 16 cases in children have been reported. The authors report the first case of a chordoid meningioma in the third ventricle.

A 12-year-old boy presented with headache, abnormal behaviors, and ataxia. Brain MR imaging revealed a 2-cm, well-enhanced mass in the third ventricle and hydrocephalus. Positron emission tomography with [¹⁸F]fluorodeoxyglucose showed that the mass was hypermetabolic. Gross-total removal of the mass was performed using a left frontal transcortical and transventricular approach. The mass originated from the left caudate head and was connected to the choroid plexus. A chordoid meningioma was diagnosed on the basis of the histological characteristics of the tumor, which was composed of cords and nests of eosinophilic vacuolated cells with an abundant myxoid matrix, similar to the features of a chordoma. A typical focal meningiomatous pattern was observed. The tumor cells were immunoreactive for vimentin and epithelial membrane antigen. The patient's headache and gait disturbance improved after the tumor was removed. The tumor showed no signs of recurrence during 12 months of follow-up.

✓ The authors analyzed 13 central neurocytomas diagnosed at Seoul National University Hospital between January 1982 and December 1993 to clarify the proliferative potential and biological behavior of these tumors. The tumor was confined to the lateral and third ventricles in 12 cases and in one case extended from the posterior thalamus to the body and trigone area of the lateral ventricle. In all 13 cases, typical clinical and radiological findings were observed, and histological diagnosis was performed via craniotomy. The diagnosis was made using light microscopic examination, immunohistochemical staining for neuronal markers, and electron microscopic findings of neuronal differentiation. One patient died due to tumor progression with recurrence 26 months after subtotal removal plus radiation therapy. Another patient had a recurrence 18 months after total tumor removal. The remaining 11 patients are free of recurrent tumor after a follow-up period that ranged from 14 to 109 months (median 50 months). To predict the proliferative potential, immunoreactivity to proliferating cell nuclear antigen (PCNA), silver colloid staining for nucleolar organizing regions (AgNORs), and DNA flow cytometry were performed in 10 of the 13 cases. The proportion of PCNA-positive cells was less than 1% in all cases and the AgNORs score ranged from 1.11 to 2.0 (mean 1.67). The DNA flow cytometry revealed diploidy in all cases and the calculated proliferation index ranged from 5.1% to 9.6% (mean 7.8%). The one case of tumor recurrence, in which the authors performed the study of proliferative potential, and another case that demonstrated mild nuclear pleomorphism also showed low percentages of PCNA-positive cells, low AgNORs scores, and diploidy in DNA flow cytometry.

It is suggested that most central neurocytomas follow a benign clinical course with low proliferative potential assessed by PCNA, AgNORs, and DNA flow cytometry; however, recurrence is possible within a relatively short time period.

Primary sarcomas of the CNS are rare and are sometimes associated with chronic subdural effusion (SDE). Approximately 10 patients with such presentations have been reported. The authors report the case of a 5-year-old boy with multiple subdural masses and SDE. An SDE had been diagnosed when the patient was 2 months old, and he received a subduroperitoneal shunt when he was 5 months old. Since then, he had been clinically stable and well for 5 years. When he presented with acute headache, nausea, and vomiting, a newly developed tumor was found. Near-total resection of the tumor was performed, and the mass was diagnosed as an embryonal-type rhabdomyosarcoma. The child was given radiation therapy and 13 cycles of chemotherapy and is still free of disease 13 months after surgery.