Primary Ewing's sarcoma of the cranial vault. Report of 2 cases

Primary Ewing sarcoma of the calvarial skull is very rare, found in less than 1% of the cases. Frontal and parietal convexities are the common sites of occurrence. We report two cases of primary Ewing sarcoma of the skull (in 13- and 14-year-old boys. The first tumor involved the right temporal region and exhibited unclear osteolytic appearance on skull x-rays while computed tomography showed an extraaxial enhanced mass and bone sclerosis with spiculated periosteal reaction. In the second case, the tumor was temporoparietal. Plain films demonstrated a large osteolysis and computed tomography revealed extensive bone destruction involving both the inner and outer tables. In both cases, the C scan revealed extraaxial enhanced masses with intracranial and extracranial extension. Radial resection was performed. Adjuvant chemotherapy was given and no recurrence or metastasis has occurred two years later.     

Teratoma in the cerebellar hemisphere of an infant

We report here a 4-month-old male infant with a cerebellar teratoma. After vomiting for 5 days, he had a tonic-clonic seizure on the left showing secondary generalization. Neuroimaging studies showed severe hydrocephalus and a large tumor in the left cerebellar hemisphere, which showed calcification. Postictal scalp electroencephalogram showed right hemispheric spikes and spike-waves. He underwent an emergency operation and had the tumor totally removed. Histological diagnosis of the tumor was immature teratoma. Intracranial teratomas have a predilection for supratentorial and midline sites. They are the most common in the neonatal period. We presented a very rare case of teratoma in the cerebellar hemisphere of an infant.     

An elderly case of malignant small cell glioma with hemorrhage coexistent with a calcified pilocytic astrocytoma component in the cerebellar hemisphere

Pilocytic astrocytoma is a less aggressive form of glial tumor that commonly occurs in the pediatric population, and its malignant transformation is extremely rare. Here, we report an elderly case of malignant small cell glioma with hemorrhage coexistent with a calcified pilocytic astrocytoma component. An 80‐year‐old male was found to have a right cerebellar non‐enhanced tumor with hematoma adjoining a calcified nodule. The lesion was surgically removed, and a histological examination verified that the tumor was a malignant small cell glioma with hemorrhagic change and the calcified nodule showed features of pilocytic astrocytoma. Genetic analyses revealed no glioma‐relevant genetic alterations such as IDH and BRAF mutations. Although calcification is generally observed in slowly growing gliomas, the aggressive clinical course of calcified cerebellar pilocytic astrocytoma has been previously reported. Our extremely rare case shows that careful follow‐up is necessary even for calcified pilocytic astrocytomas.     

Primary reticulum cell sarcoma of the brain in Wiskott-Aldrich syndrome. Report of a case.

We report a case of Wiskott-Aldrich syndrome in a 3 1/2-year-old boy. The patient developed reticulum cell sarcoma primarily in the brain; a biopsy specimen was obtained, and treatment with irradiation and chemotherapy was begun. Computerized tomography performed after three months of therapy showed remarkable regression of tumor. When the patient died five months postoperatively, no viable tumor was present; death was the result of pneumonia and sepsis. Only two previous cases of lymphoreticular neoplasm confined to the brain and associated with this syndrome have been described. We discuss the relationship between immunodeficiency and the occurrence of neoplasia in this syndrome.     

Fractured cutaneous projections to the granule cell layer of the posterior cerebellar hemisphere of the domestic cat

Snider's pioneering studies of tactile responses in the cerebellar cortex of cats and monkeys suggested that posterior regions of the cerebellar hemispheres receive somatotopically organized projections. However, recent studies in rats, using high-density, in depth microelectrode mapping methods, have shown that tactile projections to the granule cell layer of the cerebellar hemispheres are somatotopically disrupted. We reexamined the Organization of cutaneous projections to eortibellar hemispheric cortex in cats by using micromapping methods. Natural stimulation of cutaneous surfaces evokes short-latency (mossy-fiber-induced) multiple unit responses in the cerebellar granule cell layer of crus II and paramedian lobule in both ketamine- and barbiturate-anesthetized cats. Facial structures are represented in several of the most caudo-medial folia of crus II as well as in three of the rostral folia of the paramedian lobule. In several of these paramedian folia, facial projections are interspersed with projections from the forelimb. Forelimb structures alone are represented in two intermediate folia of the paramedian lobule. No cutaneous projections were found from the trunk or hindlimb. All projections were from ipsilateral receptive fields. In four folia of crus II and six folia of the paramedian lobule, cutaneous projections form a mosaic of patchlike projections. Within single patches, projections are somatotopically organized, but projections to adjacent patches come from noncontiguous body regions. Within a single folium, a particular facial region may be represented in two or three spatially separated patches. Facial patches are small, usually less than 1 mm². Forelimb patches are usually larger, often extending the full length and breadth of a folial crown. Patches with like receptive fields are not organized in zonal sagittal strips. Rather than being somatotopically organized, cutaneous mossy fiber projections to granule cells in cats, as in rats, reveal a more complex mosaic pattern of organization.     

A case of giant cell glioblastoma

The pathological entity of intracranial giant cell tumor remains controversial, which has been considered a glioma (giant cell glioblastoma) by some and a sarcoma (monstrocellular sarcoma) by others. The authors present a case in which CT scan shows a wedge-shaped high density area at the left parietal lobe. This tumor expands explosively only a month on serial CT scan. The microscopic speciemen demonstrates the bizarre irregular giant cells with large vesicular nuclei, and glial proliferation and abundant reticulin fibers. Then we reviewed and discussed with emphasis on the historical aspect of giant cell tumor and also radiologic, therapeutic problem.     

A case of giant skull myofibroma occupying left anterior cranial fossa]

We report a rare case of giant skull myofibroma occupying left anterior cranial fossa. A 53-year-old woman presented with left exophthalmos for 2 years. Neurological examination showed left exophthalmos, disturbance of bilateral visual acuity, and bitemporal hemianopsia. A CT scan revealed an ossifing mass at left anterior cranial fossa. On magnetic resonance images, the tumor showed iso-intensity on T 1-weighted image, heterogeneous high intensity on T 2-weighted image, and was heterogeneously well-enhanced after administration of Gd-DTPA. The tumor was fed mainly by middle meningeal artery. The patient underwent surgery and the tumor was removed totally. Histological diagnosis of the tumor was myofibroma. The patient has been followed every other month by MRI without any adjuvant therapy. There has been no tumor recurrence for 19 months. There is no other myofibroma in her body, therefore the patient was diagnosed as solitary myofibroma of the skull. Our case is the first report of solitary myofibroma of the skull because we could not find any reports on solitary myofibroma of the skull in the past literature.     

Primary cerebellar extramedullary myeloid cell tumor mimicking oligodendroglioma

Extramedullary myeloid cell tumors (EMCTs) are tumors consisting of immature cells of the myeloid series that occur outside the bone marrow. Most of them are associated with acute myelogenous leukemia or other myeloproliferative disorders, and a small number occur as primary lesions, i.e., are not associated with hematological disorders. Occurrence inside the cranium is rare, and there has been only one case of primary EMCT involving the cerebellum reported in the literature. The case we report here is a blastic EMCT occurring in the cerebellum of a 3-year-old boy who had no signs of leukemia or any hematological disorder throughout the entire course. The cerebellar tumor was at first misdiagnosed as an “oligodendroglioma” because of the uniformity and “fried egg” artifact of the tumor cells. The tumor disappeared during chemotherapy consisting of 12 treatments. However, it recurred and metastasized to the cerebrospinal fluid (CSF) shortly after the therapy was completed. A diagnosis of EMCT was suspected because of the presence of immature myeloid cells in the CSF, and was confirmed by anti-myeloperoxidase and anti-lysozyme immunoreactivity of the cerebellar tumor. The patient succumbed 1 year and 3 months after the first presentation of the disease. Received: 6 December 1996 / Revised, accepted: 14 March 1997     

Properties of two temperature-sensitive rous sarcoma virus transformed cerebellar cell lines

Cells from the cerebellum of 3-day-old BD-IX rats were obtained as permanent lines by transforming them with temperature-sensitive Rous sarcoma virus. The presence or absence of veratridine-stimulated Na+-uptake (voltage-dependent channels) was used to operationally classify them as neuronal or glial. When incubated at 34 degrees C, the permissive temperature for transformation, the cerebellar cells exhibit a transformed phenotype determined by anchorage independence, rounded morphology, high growth rate and absence of density-dependent inhibition of growth. In contrast, when the transformed cerebellar cell lines are kept at a temperature (38 degrees C) non-permissive for transformation, they exhibit a normal cellular phenotype with respect to the above properties. Moreover, changes toward neuronal morphology, increase in veratridine-stimulated Na+-uptake, decreased growth rate and the expression of the astrocyte specific protein, glial fibrillary acidic protein, suggest that a degree of differentiation is expressed at the non-permissive temperature.     

Primary lumbar epidural Ewing's sarcoma: case report

We report a case of a 15-years old female with an acute paraparesis secondary of an epidural mass and histopathological diagnosis was consistent with an Ewing's sarcoma without bone compromise. An exaustive literature review showed only 17 cases of primary lumbar epidural extraosseus Ewing's sarcoma.     

小脑半球髓母细胞瘤的诊断和治疗

目的：探讨小脑半球髓母细胞瘤的临床特点。方法：收集经手术证实的小脑半球髓母细胞瘤16例,对其临床资料进行回顾性分析。其中男10例,女6例,平均年龄20.3岁,常见症状和体征为高颅压征和共济失调,术前有脑积水者6例,占37.5%,16例患者均行开颅肿瘤切除术。结果：本组患者13例获随访,术后平均生存时间为74.9个月,术后5年生存率为65.2%。结论：小脑半球髓母细胞瘤与中线区髓母细胞瘤的发病年龄、临床症状及预后均有所不同,临床上较易误诊,小脑半球髓母细胞瘤发病年龄较大,转移较少,预后较好。     

Neutron-capture therapy in a case of cerebellar sarcoma treated initially with X-radiation

Summary This paper deals with a girl who at birth had a large retroauricular mass diagnosed clinically as hemangioma. It responded well to X-ray therapy. When she was 11 years old, a sarcoma was removed from the cerebellum. No evidence could be found that the sarcoma had originated from the retroauricular tumor. After the use of nitrogen mustard locally and after three courses of X-ray therapy to the tumor over a period of 9 1/2 months — a total radiation dose of approximately 7,953 r — the patient was growing moribund. During 4 neutron-capture treatments, which were directed chiefly toward the suboccipital region, striking improvement occurred — to such an extent that the patient was able for a time to sit up in a wheelchair and converse. As a result of the therapy, all tumor which had spread suboccipitally and into the neck vanished, as did also virtually all tumor in the dorsal third of the cerebellum, i.e., in the region receiving the largest concentration of thermal neutrons. In the middle third of the cerebellum large and small tumor aggregates, some of them calcified, were necrotic and were walled off by hyperplastic connective tissue. In the ventral third of the cerebellum, in a region presumably out of the range of an effective concentration of thermal neutrons, the tumor grew unimpeded.Evidence indicated that the sarcoma in our case originated in the vascular sheath, chiefly about vessels in the granular layer of the cerebellum.Life was prolonged approximately 8 months by the neutron-capture therapy.

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Résumé Ce cas est celui d'une enfant qui présentait à la naissance une large masse rétroauriculaire que le diagnostique clinique identifiait comme étant un hémangiome. La radio thérapie eut de très bons résultats. A onze ans on pratiqua l'ablation d'un sarcome cérébelleux qui ne semblait pas avoir été causé par la tumeur rétroauriculaire. Après traitement à moutarde nitrogénée et après trois appliquations de rayon X s'échelonnants pendant neuf mois et demi-soit une dose de radiation d'environ 7,953 r — son état s'aggrava. Pendant quatre traitements de «neutron capture», dirigés principalement sur la région sous-occipitale, on remarqua une amélioration spectaculaire, la malade pouvant s'asseoir et étant même capable de converser pendant quelque temps. Après ce traitement, la tumeur qui s'étendait dans la région sous-occipitale et dans la cou disparut, ainsi que presque toute la tumeur qui se trouvait dans le tiers dorsal du cervelet particulièrement dans la région la plus proche de l'aire sous-occipitale. Dans le tiers moyen cérébelleux on trouva également des agrégats de tumeurs plus ou moins grands, dont quelques-uns calcifiés, qui étaient isolés par un tissu conjonctif hyperplasique. Dans le tiers ventral du cervelet la tumeur ne cessa de croître dans la région non-irradiée par les neutrons. La tumeur était un sarcome d'origine périvasculaire située principalement dans la couche réticulaire cérébelleuse. La vie de la malade fut prolongée d'environ huit mois par le traitement de «neutron capture».

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With 20 Figures in the Text

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Read at the 36th Annual Meeting of the American Association of Neuropathologists, Boston, June 12, 1960.

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Research supported by the United States Atomic Energy Commission.     

Solitary cerebellar metastasis from Ewing's sarcoma: case report and review of the literature

A rare case of Ewing's sarcoma metastatic to the cerebellum is presented. Neurosurgical intervention was required which played a significant role in the treatment of this patient. The incidence and treatment of central nervous system involvement from Ewing's sarcoma is reviewed and discussed.