Lipomatous meningioma: a study of five cases with brief review of literature

Lipomatous meningiomas are an uncommon subtype of meningiomas. In the present report, the clinical characteristics, neuroimaing and pathological features of 5 patients (4 males, 1 female; age range, 17-45 years; mean age, 35.2 years) with lipomatous meningioma were analyzed. The neuro-pathological reevaluation of lipomatous meningioma involved assessment of histological features proposed by the current WHO classification. The presence or absence of high whorls, fascicles, solid growth, glial invasion, necrosis and nuclear pleomorphism was assessed. The most common presenting symptom was seizures. Location of tumor was frontal (3 cases), parietal (1 case) and fronto-temporal (1 case). All cases showed radiological features of conventional meningioma. Complete excision of tumor along with involved dura was performed in all the patients. Three tumors were meningothelial and 2 were transitional. Tumoral lipid content was variable from 10% to 30%. In addition, there were microcystic features (2 cases) and psammoma bodies (1 case). No recurrence was noted in any of our cases till the last follow-up. Lipomatous meningioma represents a rare distinctive type of meningioma, with a good prognosis with complete removal.     

原发性中枢神经系统血管炎一例报道及文献复习

患者女性,20岁,间歇性头痛3个月.于当地医院未经任何辅助检查予对症药物治疗,症状缓解.2007年12月6日再次头痛发作,伴喷射状呕吐.     

Superficial siderosis of the CNS: report of three cases and review of the literature

Superficial siderosis (SS) is a rare entity. A chronic subarachnoid hemorrhage (SAH) leads to the deposition of ferric pigments and ions in the surface of the central nervous system (CNS). The cause of this hemorrhage is identified in half of the cases. The disease progresses slowly and the damage is often irreversible by the time diagnosis is established. The management focuses on the identification and ablation of the bleeding source. Knowledge about this entity is based on sporadic reports. We describe three cases of superficial siderosis and briefly review the literature.     

Primary intracranial myopericytoma: report of three cases and review of the literature

Myopericytoma is a benign tumour generally arising in the subcutaneous and superficial soft tissues of the extremities. Very few cases have been reported in other locations and intracranial examples are exceptional. We now report on three cases of primary intracranial myopericytoma and review the literature on that rare entity. The patients were women in their fifties who presented with decreased visual acuity in two cases and raised intracranial pressure in one case. The tumour involved, respectively, the anterior cranial fossa, the orbital apex and the pineal region. Gross total resection was achieved in all three cases. Histological analysis revealed oval-to-spindle shaped myoid-appearing cells with a striking tendency for concentric perivascular growth. The lesional cells showed apparent differentiation towards perivascular myoid cells as witnessed by smooth muscle actin expression. In one case, an epithelioid differentiation was also present. None of the patients received adjuvant therapy. One patient died of unrelated causes 6 months after surgery. The other two are alive and well at 9 and 12 month follow-up respectively. Myopericytoma is a recently described neoplasm, and it is likely that reappraisal of intracranial haemangiopericytoma with which it shares many histopathologic features will lead to more case reports of primary intracranial myopericytoma.     

Primary cerebral angiitis: a case history and literature review of its diagnosis

The authors present a case history of primary cerebral angiitis with four years of follow-up. The early diagnosis was based on typical clinical symptoms, brain MRI, intracerebral MRA and histology of sural nerve biopsy. Electroneurography suggested peripheral involvement, although the patient did not have clinical signs of peripheral neuropathy. Glucocorticoid and immunosuppressive treatment resulted in remission. The diagnostic difficulties of primary cerebral vasculitis are also summarized in the discussion.     

Primary CNS malignant rhabdoid tumor (MRT): report of two cases and review of literature

Primary CNS rhabdoid tumor is an enigmatic and extremely rare malignant tumor of early childhood, probably embryonal, that has often been histopathologically classified together with rhabdoid tumor of kidneys or other organs in infants. Only four previously documented cases of primary CNS malignant rhabdoid tumor (MRT) were found in the literature. We report two cases of primary CNS MRT with biopsy and complete autopsy findings that share close clinicopathologic, immunohistochemical and ultrastructural similarities between the two. It is concluded that the primary CNS MRT is an entity which is extremely malignant, easily mistaken as a primitive neuroectodermal tumor, and potentially derives from a meningothelial precursor cell which is embryonally equal to the serosal mesothelial precursor cells which surround the kidneys and other organs. Hence, it tends to anatomically occur at the location of abundant meningeal infoldings such as the cerebellar cortex and may be diffuse or multicentric in its meningeal involvement. Furthermore, it may concurrently or multicentrically occur in association with MRT originating from the serosal membrane of other organs, such as the kidney.     

Primary melanocytic tumors of the central nervous system: a neuroradiological and clinicopathological study of five cases and brief review of literature

Primary melanocytic tumors of the central nervous system (CNS) are uncommon lesions. These lesions arise from the melanocytes located within leptomeninges and include diffuse melanocytosis and meningeal melanomatosis (seen in neurocutaneous melanosis), melanocytoma, and malignant melanoma. To study, the clinical course, neuroradiological features, morphology and immunohistochemistry of primary melanocytic tumor of CNS. Demographic, clinical and surgico-pathologic findings of five patients with melanocytic tumors seen between 1996 and 2003 were studied. In this study, five cases of primary melanocytic tumors have been reported: four cases of malignant melanoma and one case of melanocytoma. Three of the 5 cases were intracranial and 2 were spinal. The mean age in the present study was 26 years. Presenting features varied according to the location. Primary melanocytic tumor of CNS are rare. Whenever possible, complete surgical excision is the best treatment.     

An autopsied case of the Crow-Fukase syndrome: a neuropathological study with emphasis on spinal roots

Summary An autopsied case of the Crow-Fukase syndrome is reported. Neuropathological findings were as follows: (1) in the sural nerve, there was marked decrease of large and small myelinated fibers. Myelinated fibers showing axonal degeneration and segmental demyelination and remyelination were moderately increased. (2) In the lumbar spinal roots, myelinated fibers showing segmental demyelination and remyelination were frequently observed. The density of myelinated fibers of the ventral root was less at the dural site than the spinal site, while that of the dorsal roots was less at the spinal site than the dural site. (3) In the dorsal root ganglion, there were Nageotte's residual nodules and satellitosis; (4) in the lumbar and thoracic spinal cord, there was pallor of the dorsal column; and (5) nerve cells showing central chromatolysis were frequently observed in the spinal anterior horn cells. Segmental demyelination and remyelination in the spinal roots and loss of myelinated fibers with axonal degeneration in the sural nerve are fibers with axonal degeneration in the sural nerve are main neuropathological features of this syndrome.     

Thanatophoric dysplasia: report of 2 cases with neuropathological study

We report two cases of thanatophoric dysplasia (TD) with detailed neuropathologic evaluation. One case was representative of TD type I and the other TD type II. The case with TD type I showed macrocephaly, narrow thoracic cage, pulmonary hypoplasia and bowed limbs. Radiological study showed flat vertebral bodies, short curved appendicular skeleton and flaring of metaphyses. The other case (TD type 2) showed macrocephaly, cleft palate, short limbs and cloverleaf skull. Radiological findings were generalized platyspondy with excessive intervertebral disc space heights and a large head. Microscopic examination of both cases revealed temporal lobe polymicrogyria, abnormalities of the hippocampus and heterotopic neuroglial tissue within the meninges. There were no noticeable differences in CNS abnormalities between TD type I and II.     

Congenital myasthenic syndrome: report of four cases and brief review of literature

The term 'congenital myasthenic syndrome' (CMS) encompasses a number of heterogeneous disorders characterised by myasthenic symptoms since birth, usually with positive family history and absence of acetyl choline receptor antibodies. Recent advances in electrophysiology and ultrastructural analysis of neuromuscular junction have made it possible to identify the various defects underlying these disorders. We report four cases of CMS, with a review of literature.     

Recurrent leukoencephalopathy with microhemorrhages: gradient-echo MRI study diagnostic value in CNS primary angiitis

Diagnosis of primary angiitis of the central nervous system (PACNS) is difficult in relation to variability in its clinical manifestations and absence of specific signs in neuroimaging. A young patient presented with a recurrent encephalopathic clinical course. T2 and fluid-attenuated inversion recovery-magnetic resonance imaging (FLAIR-MRI) showed hyperintense lesions in the cerebral white matter suggesting demyelination. Those lesions decreased or even disappeared after treatment with steroids and immunoglobulins. In echo gradient MRI (T2*-MRI), there were permanent cortical-subcortical petechial hypointense lesions (microhemorrhages). Definite diagnosis was established after cerebral biopsy. Intravenous cyclophosphamide was administrated with no new relapses in more than 18 months of follow-up. In a compatible clinical course, the finding of petechial hemorrhages in T2*-WI could play an important role in early diagnosis of PACNS.     

脊髓神经节胶质细胞瘤三例并文献复习

目的 探讨脊髓神经节胶质细胞瘤的临床特点和手术治疗.方法 收集北京天坛医院神经外科自2010年10月至2011年3月的3例脊髓神经节胶质细胞瘤手术患者资料,结合文献复习进行临床分析.结果 3例患者均以肢体感觉或运动功能障碍起病;MRI检查具低T1高T2信号特征,强化多样,瘤周水肿明显,需与星形细胞瘤、室管膜瘤等其他类型神经上皮来源肿瘤相鉴别;患者行肿瘤全切除或近全切除术,术中肿瘤边界分辨困难;手术病理诊断为神经节胶质细胞瘤、WHOⅠ级,病理切片上混合表达肿瘤性神经节细胞及神经胶质细胞;术后临床症状缓解明显,未行放疗等其他辅助治疗.结论 脊髓神经节胶质细胞瘤相对少见,致残率高,术前诊断困难,手术切除难度大;如手术切除满意预后良好,不需常规行放疔等辅助治疗.     

Levodopa and melanoma: three cases and review of literature.

Three patients are reported who developed a melanoma while taking levodopa for Parkinson's disease. There were two cutaneous melanomas and one metastatic melanoma with occult primary. The literature on the association of Parkinson's disease, levodopa therapy and melanoma is reviewed. The capacity of levodopa to induce melanomas and its alleged adverse effect on the clinical course of the disease are questioned.     

Hemimegalencephaly: a fetal case with neuropathological confirmation and review of the literature

Hemimegalencephaly (HME) is a developmental abnormality of the central nervous system, identified by an abnormal increase in the size of one cerebral hemisphere. HME may present as either a syndromic or isolated case. To date the literature on HME has focused primarily on non-fetal pediatric patients, largely related to surgical resection specimens of the HME hemisphere. We present the case of a male fetus at 22 weeks gestation with intracranial abnormalities identified on a follow-up ultrasound. Gross examination of the fetal brain confirmed the increased size of the right cerebral hemisphere. The ipsilateral brain stem and cerebellum were not involved. Light microscopy demonstrated the presence of accelerated cortical differentiation along with several migrational anomalies in the HME hemisphere. Based on the gross and microscopic findings, a diagnosis of fetal hemimegalencephaly was made. The periventricular proliferative zone of the abnormal hemisphere contained a normal population of neuroepithelial precursor cells. An exhaustive immunohistochemical study found immunoreactivity for calretinin and synaptophysin, while the Ki-67 proliferation labeling was not increased in the HME hemisphere. Our case is the first autopsied report on fetal hemimegalencephaly and confirms that the key pathogenic changes may present as early as 20–22 weeks gestation. The major pathological features of our case are in keeping with a disturbance in accelerated neuronal differentiation and migrational abnormalities.     

Primary dural lymphoma: a report of two cases with review of the literature

Primary lymphoma arising in dura is exceedingly rare. We report the clinicopathologic findings of two patients with primary B-cell lymphoma of dura. Both were female, 38 and 45 years old. Prior to biopsy they were felt to have meningioma on preoperative magnetic resonance imagery. Histologically, tumors were classified as MALT-type lymphoma. Literature describe only 14 reports of similar entity. Primary lymphomas arising in dura appear to have a more favourable clinical course compared to PCNSL and may require a less aggressive treatment.     

Primary spinal melanoma of the cervical leptomeninges: report of a case with brief review of literature

Central nervous system primary malignant melanoma accounts for approximately 1% of all melanomas. Primary spinal melanomas are even more unusual. We report a patient with primary spinal melanoma of the cervical leptomeninges. The histology of the tumor showed tumor cells arranged in sheets, ill-defined fascicles and nests and displayed a moderate grade of cellular and nuclear pleomorphism and mitoses with abundant pigment in the cytoplasm. The tumor cells were immunoreactive for HMB-45, and for S-100.