A case of cystic metastatic intracranial amelanotic melanoma--analysis of findings in CT and MRI

A case of cystic intracranial metastatic amelanotic melanoma is presented. As far as we know, cyst formation in intracranial melanoma is rare, and only 15 cases of intracranial amelanotic melanoma have been reported until now. A 63-year-old man was admitted with headache and progressive visual disturbance. CT scan revealed a large low-density mass with ring-like enhancement in the left frontal lobe. Both T 1-and T 2-weighted MRI images revealed hyperintensity. A left frontotemporal craniotomy was performed. A yellowish mass was observed in the frontal lobe. The content of the cyst consisted of old hematoma, xanthochromic fluid and necrotic tissue, was evacuated and the cyst wall was totally resected. No abnormal pigmentation was noted in the cyst wall and surrounding brain tissue. The histological examination revealed amelanotic melanoma. Primary lesion was found on the left thigh later and resected. The patient died of further intracranial metastasis with repeated hemorrhage 5 months after the admission. Both CT and MRI findings of our case is atypical as an intracranial malignant melanoma. However, these are compatible with those of intracerebral hemorrhage in subacute stage. It is suggested that melanoma may make the diagnosis difficult when tumor hemorrhage modifies the images of CT or MRI.     

Variante amelanótica en melanoma cerebral primario: a propósito de un caso

Primary brain melanoma is a very rare tumour (only 0.07% of primary CNS neoplasms) which usually shows with abundant melanin content; whereas hypo/melanotic variants have been scarcely described.We introduce the case of a female patient with headache, left brachial paresis and frontal lobar syndrome. The MRI image showed a right frontal mass with homogeneous contrast uptake. As treatment, a complete surgical resection was performed. Pathology was diagnostic for melanoma, with very low melanin content and a high proliferative index. A thorough extension study was performed to rule out an extracranial primary origin. Due to several intercurrent complications, the patient evolved unfavorably, not being able to receive further treatment.The amelanotic variant of primary intracranial malignant melanomas has not been described in detail previously. We will review the literature, focusing on the particularities of management and diagnosis of this clinical entity.     

Glioblastoma multiforme with extracranial metastases without previous surgery: demonstration of extracranial metastases by peroxidase antiperoxidase staining and clinicopathological study

Spontaneous extracranial metastases of glioblastoma multiforme in the absence of previous surgery have been rarely reported (Table 1). We presented an autopsy case of glioblastoma multiforme which spontaneously metastasized to the lungs, bronchial lymph nodes, liver, kidney, heart and spleen. A 68-year-old man was admitted to the Department of Neurosurgery at our hospital with chief complaints of right sided weakness in July 1984. He was well until November 1983, when he noticed weakness of right lower extremity followed one month later by the weakness in the right arm. He was treated at another hospital under the diagnosis of cerebral infarction, but his right sided weakness gradually progressed. In June 1984, a diagnosis of brain tumor was made by the neurological findings and CT scan, and he was transferred to our hospital for further evaluation and treatment. Neurological examination revealed disorientation, bilateral papilledema, right hemiparesis, right hyperreflexia and right hemisensory disturbance. CT scan revealed abnormal low density area in the left fronto-parietal lobe (Fig. 1) with irregular enhanced lesions on contrast CT scan (Fig. 2). Chest x-ray showed abnormal shadow in the right middle and lower lobe (Fig. 3) and a diagnosis of pulmonary infarction was suspected. The clinical states of this patient took downhill course and he expired on July 13, 1984 by the complication of disseminated intravascular coagulation syndrome. The brain weight was 1400 gr. Dura mater and falx cerebri were tightly adherent to the left parietal lobe (Fig. 4). Primary brain tumor was found in the left fronto-parietal region. The tumor was poorly defined with necrosis and hemorrhage (Fig. 5).(ABSTRACT TRUNCATED AT 250 WORDS)     

A cystic amelanotic melanoma metastasis to the brain: case report

As far as we know, cyst formation in intracranial melanoma is rare, and only 15 cases of intracranial amelanotic melanoma have been reported until now. A yellowish mass was observed in the frontal lobe. The content of the cyst consisted of old hematoma, xanthochromic fluid and necrotic tissue, was evacuated and the cyst wall was totally resected. No abnormal pigmentation was noted in the cyst wall and surrounding brain tissue. The imaging features of metastatic melanomas are distinctive due to the presence of melanin and the propensity for hemorrhage. Both hemorrhage and melanin can produce T1-weighted hyperintensity and T2-weighted signal intensity loss.     

Clinical pathological conference. An unusual cause of a spontaneous intracerebral haematoma

A 61-year old man was admitted for sudden right hemiparesis. The first CT brain scan revealed a haemorrhage in the left frontal horn. The cause of the bleeding could not be discovered initially. Subsequent CT brain scans showed an isodense mass lesion in the left frontal horn. The patient died after a relapsing course of the hemiparesis. The postmortem examination demonstrated a large protoplasmic astrocytoma of the left basal ganglia with recurrent large bleedings.     

颅内转移性恶性黑色素瘤:一例报告并文献复习

目的探讨颅内转移性恶性黑色素瘤临床组织病理学及免疫组织化学特征。方法应用组织病理学及免疫组织化学检测方法对1例颅内多发转移性恶性黑色素瘤患者的两次手术标本进行观察,通过复习文献分析其组织病理学和免疫组织化学特征,提出诊断与鉴别诊断要点。结果男性患者,48岁。因颅内多发占位性病变、肿瘤卒中而行右侧额颞叶肿瘤切除术。两次手术标本组织病理学表现不一:首次右侧额叶病灶表现为异型性上皮样肿瘤细胞围绕毛细血管和纤维组织呈乳头状排列;再次手术标本为右侧颞叶,病灶表现为梭形细胞呈片巢状或束状排列,胞质丰富、淡染或透亮,胞核异型性明显、核仁呈明显嗜酸性。左侧腹股沟淋巴结活检提示肿瘤转移,且肿瘤组织局部可见大量黑色素颗粒。免疫组织化学染色肿瘤细胞弥漫表达S-100蛋白、人黑色素细胞瘤抗原45、Melan-A和波形蛋白,部分表达上皮膜抗原,而细胞角蛋白、高分子角蛋白、低分子角蛋白、胶质纤维酸性蛋白、突触素、神经微丝蛋白等表达阴性。结论颅内转移性恶性黑色素瘤的组织学表现复杂多样,临床病史和影像学资料对诊断具有重要参考价值,但明确诊断仍需依靠免疫组织化学检测结果。需注意与转移癌、脑膜肿瘤、淋巴瘤及其他含黑色素的颅内肿瘤相鉴别。     

Brain tumor presenting as an acute pure motor hemiparesis

Acute pure motor hemiparesis is a clinical syndrome of isolated hemiparesis usually related to lacunar infarction, although other etiologies have been described. We recently encountered three patients with the abrupt onset of pure motor hemiparesis as the initial manifestation of primary or metastatic brain tumor. In each patient, early computed tomography demonstrated a nonhemorrhagic, right frontal, enhancing mass lesion. While the mechanism whereby brain tumor may present abruptly and simulate a stroke remains uncertain, these cases illustrate that pure motor hemiparesis can be the initial symptom of intracranial tumor. Early computed tomography or magnetic resonance imaging is suggested for all patients who present acutely with pure motor hemiparesis.     

Nocardia abscess during treatment of brain toxoplasmosis in a patient with aids, utility of proton MR spectroscopy and diffusion-weighted imaging in diagnosis

We report the case of a 30-year-old man with known HIV-positive status who developed, 4 months prior to admission, recurrent left partial motor seizures followed by left hemiparesis. At another hospital, contrasted CT scan of the head revealed right frontal hypodense lesion with mass effect and focal contrast enhancement. A small left occipital lesion was also present. HIV-associated brain toxoplasmosis was considered and phenytoin, pyrimethamine, clindamycin and antiretrovirals were administered. Hemiparesis improved but, 3 weeks prior to admission, he developed progressive headache and bilateral visual defects. Upon admission to our center, he was found with left homonymous hemianopsia, right hemiparesis and a large hypodense left occipital lesion on a head CT scan. Proton MR spectroscopy showed lactate at 1.3ppm, amino acids at 0.9ppm, and diffusion-weighted imaging (DWI) revealed hyperintensity at the lesion, suggesting a pyogenic abscess. Aspiration yielded purulent material and Nocardia asteroides grew in culture. The patient was treated with trimethoprim-sulfametoxazole and recovered with a mild visual field residual defect.     

An autopsy case of neurocutaneous melanosis associated with intracerebral malignant melanoma

The authors reported the clinical course and the postmortem examination of a unique case of neurocutaneous melanosis with numerous anomalies and complications, which included congenital dislocation of lenses, hypogonadism, ectopia of prostatic duct, genuine phimose, retentio testis, psina bifida and neurogenic bladder. This 13-year-old boy with a large hairy nevus in a bathing trunk configulation and multiple small nevi over the whole body since his birth was admitted to our hospital for evaluation of headache and vomiting. Neurological examination showed bilateral papilledema and slight left hemiparesis. A CT scan revealed a large right frontal mass and craniotomy was performed with subtotal removal of this tumor which was confirmed as a malignant leptomeningeal melanoma. He initially made uneventful postoperative recovery, and two courses of chemotherapy with DTIC, ACNU and VCR were given; however, the currence of brain tumor ensued shortly thereafter, and he died in approximately six months after the onset of intracranial symptoms despite of the third course of chemotherapy. Thirty five cases of neurocutaneous melanosis associated with or without malignant melanoma have been reported in Japan. Twenty-eight cases were male and 7 female. Two cases showed the evidence of primary malignant melanoma outside of the central nervous system, whereas twenty eight leptomeningeal melanoma, in which 22 were solid and 6 diffuse, were shown intracranially. Other 5 cases had epileptic seizure and/or hydrocephalus caused by wide spreaded leptmeningeal melanosis. This high incidence of intracranial malignant melanoma in this disorder was remarkable compaired with the previous reports in other countries. Mean duration between deaths and the onset of symptoms of intracranial hypertension or focal neurological signs was 7 months, ranging from 1 to 24 months, showing the rapidly deteriorating course in this disorder.(ABSTRACT TRUNCATED AT 250 WORDS)     

Peritumoral edema after stereotactic radiosurgery for meningioma

Peritumoral edema induced by radiosurgery after the treatment of intracranial meningioma has been reported and its mechanism remains unclear. A 65-year-old woman presented with a history of intermittent dizziness. A CT scan and MRI of the brain revealed an extra-axial space-occupying lesion in the left frontal region. She was treated by stereotactic radiosurgery (SRS). Seven months later, the patient began experiencing general weakness and drowsiness. She developed peritumoral edema around the left frontal tumor. Similarly, a 55-year-old woman complained of unstable gait and dizziness 1 month prior to admission. A CT scan and MRI of the brain revealed an extra-axial space-occupying lesion in the right posterior temporal region. Six months after SRS, this patient experienced deteriorated consciousness and general weakness. Peritumoral edema in the right temporal region was noted. We report these two patients, discuss possible causes and review the relevant literature.     

Two primary brain tumors, meningioma and glioblastoma multiforme, in opposite hemispheres of the same patient.

We report a case with double primary intracranial tumors of different cell types without phacomatosis. The patient was hospitalized due to progressive memory impairment, headaches, dysarthria and right hemiparesis. Initial computed tomographic (CT) examinations revealed a large hyperdense tumor over the right frontal lobe, suggestive of an extra-axial meningioma. Additionally, there was unusual brain edema in the contralateral hemisphere that subsequently proved to originate from an intrinsic tumor. Staged craniotomies were used to treat the patient. Pathological examinations confirmed the two tumors to be a meningioma and a glioblastoma multiforme, respectively. The patient made an uneventful recovery after treatment. Although meningioma and glioma represent two common primary intracranial tumors, the simultaneous development of the two tumors is rare. A randomly occurring event most likely accounted for this linkage in the patient. We suggest that extraordinary brain edema far remote from the primary brain lesion warrants special attention for identifying other potentially undetected lesions.     

Schilder's disease: additional aspects and a therapeutic option

An 8-year-old boy presented with large diffuse myelinoclastic bifrontal and right parietal peripherally enhancing white matter lesions with high intracranial pressure. Intravenous treatment with ACTH and high dose cyclophosphamide (cytoxin) resulted in shrinkage of the lesion, loss of peripheral enhancement and clear clinical improvement. After nine months following the onset of the first episode, an isolated left frontal enhancing lesion appeared along with a new right hemiparesis and speech difficulty. The same therapy again resulted in prompt definitive improvement of the CT scan and reversal of the clinical course. This case was extensively investigated and characterized as the 1912 variety of Schilder's disease, to be distinguished from post-infectious encephalopathy and other forms of white matter disease such as adrenoleukodystrophy. No elevation of CSF IgG, oligoclonal bands, or characteristic abnormality of T-cell subset distribution were identified. However, the pathological picture most closely resembled that of multiple sclerosis. A striking feature of this child's clinical history was the relatively rapid clinical improvement with immunosuppression.     

Intracranial dural metastasis from uterine leiomyosarcoma with orbital extension

Intracranial metastases from uterine leiomyosarcoma are very rare and have been found mainly in the brain (17 cases); on the other hand, metastases to the skull, dura and orbit are really exceptional. The authors report the case of a 57-year-old woman who presented with a 6-week history of right proptosis, left hemiparesis, intracranial hypertension and torpor 8 months after surgery for uterine leiomyosarcoma. CT scan showed a very large right frontal tumor with both intracranial and intraorbital extension. At operation the tumor was found to arise from the dura of the right anterior cranial fossa; complete removal of the intracranial tumor mass and partial removal of the intraorbital component were performed. However, early tumor regrowth was observed 45 days after operation and death occurred 2 months later. Pathologic examination showed a high-grade sarcoma with smooth muscle differentiation and high mitotic activity. Immunohistochemical staining revealed positivity for actin and vimentin and negativity for S-100 protein, cytocheratin and desmin. This is the first reported case of uterine leiomyosarcoma metastatic to the dura of the anterior cranial fossa with intracranial and intraorbital extension. An aggressive surgical resection is the best treatment of intracranial metastatic leiomyosarcoma, because of the scarce response to radiotherapy and chemotherapy. However, the outcome is poor, with early recurrence.     

Atlanto-axial rotatory fixation caused by spontaneous intracerebral haemorrhage in a child

Introduction Atlanto-axial rotatory fixation is rare in children and usually associated with upper respiratory tract infection or trauma. We present a patient who developed the condition acutely as a consequence of rapidly evolving hemiplegia secondary to intracerebral haemorrhage.Case report A previously well 12-year-old boy suddenly developed left hemiparesis within 30 min. Soon after, he developed painful torticollis. On examination, a dense left hemiplegia was associated with left facial and hypoglossal weakness. On computerised tomography (CT) scan there was an intracerebral haemorrhage in the deep aspect of the right frontal lobe in the corona radiata, with extension in the ventricular system. The plain radiographs of the cervical spine, and CT scan later, confirmed the presence of C1–C2 rotatory subluxation. The haematoma was aspirated stereotactically through a burr hole, and at the same session, the subluxation was reduced by manipulation and traction of the cervical spine. The patient was treated with a hard collar. The torticollis did not recur. The hemiparesis resolved within 6 months. A subsequent magnetic resonance imaging and magnetic resonance angiography scan indicated possible arterio-venous malformation (AVM) at the site of the haemorrhage, which was confirmed with digital subtraction angiography. The AVM was surgically excised 1 year from the original haemorrhage.Discussion In the absence of any other predisposing factor, it is postulated that the acute atlanto-axial rotatory subluxation was secondary to the acute loss of muscular tone due to the acute hemiparesis. This explains that the subluxation has not recurred a year after, while the hemiparesis has completely resolved.     

颅内转移性恶性黑色素瘤：一例报告并文献复习

目的探讨颅内转移性恶性黑色素瘤临床组织病理学及免疫组织化学特征。方法应用组织病理学及免疫组织化学检测方法对1例颅内多发转移性恶性黑色素瘤患者的两次手术标本进行观察,通过复习文献分析其组织病理学和免疫组织化学特征,提出诊断与鉴别诊断要点。结果男性患者,48岁。因颅内多发占位性病变、肿瘤卒中而行右侧额颞叶肿瘤切除术。两次手术标本组织病理学表现不一：首次右侧额叶病灶表现为异型性上皮样肿瘤细胞围绕毛细血管和纤维组织呈乳头状排列;再次手术标本为右侧颞叶,病灶表现为梭形细胞呈片巢状或束状排列,胞质丰富、淡染或透亮,胞核异型性明显、核仁呈明显嗜酸性。左侧腹股沟淋巴结活检提示肿瘤转移,且肿瘤组织局部可见大量黑色素颗粒。免疫组织化学染色肿瘤细胞弥漫表达S一100蛋白、人黑色素细胞瘤抗原45、Melan—A和波形蛋白,部分表达上皮膜抗原,而细胞角蛋白、高分子角蛋白、低分子角蛋白、胶质纤维酸性蛋白、突触素、神经微丝蛋白等表达阴性。结论颅内转移性恶性黑色素瘤的组织学表现复杂多样,临床病史和影像学资料对诊断具有重要参考价值,但明确诊断仍需依靠免疫组织化学检测结果。需注意与转移癌、脑膜肿瘤、淋巴瘤及其他含黑色素的颅内肿瘤相鉴别。     

A case of intracranial hemorrhage in a young infant due to vitamin K deficiency: comparison of MRI and CT findings

We reported a case of intracranial hemorrhage due to vitamin K deficiency in a 43-day-old male infant whose MRI findings were compared with CT findings. The infant was vacuum delivered at the 40th gestational week and Apgar score was 9 at 5 min after birth. He weighed 2,750 g at birth and was breast-fed. His growth was normal after birth, but forty-one days after birth, the infant became febrile and vomited three times. Forty-two days after birth, right hemiconvulsions occurred and the infant vomited again. He was brought to our clinic forty-three days after birth, because cranial CT showed multiple intracranial hemorrhages. On admission, skin color was pale, and the patient was somnolent. The anterior fontanel was bulging and tense. Neurological examination revealed right hemiparesis. Since laboratory data indicated that intracranial hemorrhage resulted from vitamin K deficiency, administration of vitamin K and blood transfusion were carried out. MRI examination was made three days after admission, and demonstrated a posterior fossa subdural hematoma as well as a left frontal subdural hematoma associated with intracerebral hemorrhage. Five days after admission, left frontal subdural hematoma was removed by left frontal craniotomy, and twenty-six days after the operation he was discharged without any neurological deficits.(ABSTRACT TRUNCATED AT 250 WORDS)     

Intracranial occlusion of the internal carotid artery after minor closed head injury]

Thrombosis of the extracranial portion of the internal carotid artery as a result of nonpenetrating head and neck injury is not uncommon. However, intracranial occlusion of the internal carotid artery after minor head and neck injury without skull fracture is rare. We report a case of 14-year-old male who suffered a minor head injury during an athletic meeting of his school and developed a right hemiparesis and a lethargy state resulting from thrombosis of the supraclinoid portion of the left internal carotid artery. On admission, skull films and a CT scan revealed no abnormality. One hour later, he fully recovered. One day later, no definite lesions were detected on T1-weighted and T2-weighted image of MRI, but an abnormal high signal lesion in the left frontal lobe was detected on diffusion-weighted image of MRI. On additional MR angiography, intracranial occlusion of the internal carotid artery due to dissection was demonstrated.     

颅内原发黑色素瘤的MRI、CT分析

目的探讨颅内原发黑色素瘤的影像学表现,以提高对该病的认识和诊断正确率。方法回顾性分析经手术病理证实的4例颅内原发黑色素瘤MRI、CT表现;4例患者均进行MRI检查,其中3例同时行CT检查。结果CT平扫均为高密度,密度部分不均匀,增强扫描肿瘤明显强化。4例MRIT1WI肿瘤均表现为高信号为主,其中2例T2WI为低信号,2例为高低混杂信号,肿瘤内信号部分不均匀,增强后扫描肿瘤可见明显强化。结论颅内原发黑色素瘤MRI具有特征性表现,对于显示肿瘤范围、成分及脑膜的浸润和定性诊断较CT有优势。     

A rare case of subarachnoideal pneumocephalus after ear douche therapy

No detailed reports on pneumocephalus caused by any factors other than head trauma, and their courses accompanied with this disease have been so far available. We recently experienced a case of pneumocephalus complicated with severe clouding of consciousness, ocular deviation, and unilateral spatial neglect with the results being reported hereinafter. A 54-year-old man had often received ear douche therapy due to chronic sinusitis and tubal obstruction about 3 years before without any history of head trauma. On Jan. 6, 1984, sudden clouding of consciousness accompanying stiffness of the left arm occurred immediately after ear douche, and then he was transferred to our center. At admission, semicomatose, bilateral ocular deviation to the right, and left hemiparesis were observed. Plain skull X-ray films showed a retention of air in the frontal and temporal regions, while CT scan revealed air retention on the bilateral frontal region, bilateral temporal tip and suprasellar cistern. However, no abnormal findings were detected in the brain. Consciousness and hemiparesis recovered on the next day of hospitalization, however, the left hemispatial neglect still remained. This symptom was still observed on the 3rd day but disappeared by the 4th day of hospitalization. For clarifying its cause, cerebral angiography, CT scan and electroencephalography were then performed. CT scan revealed no anomalies in the brain, while cerebral angiography showed a cerebral circulation pattern in favor of the right internal carotid artery.(ABSTRACT TRUNCATED AT 250 WORDS)     

A case of intracerebral and subarachnoid hemorrhage of unknown origin with preceding headache]

We report a case of a 31-year-old female with multiple intracerebral hemorrhage and subarachnoid hemorrhage. She presented with headache one week before hemorrhage, and a CT scan performed at that time showed no abnormal findings. Neurological examination on admission revealed mild disturbance of consciousness, papilledema, and mild left hemiparesis. CT scans demonstrated intracerebral hemorrhage in the right caudate head and left frontal subcortex, and diffuse subarachnoid hemorrhage. Cerebral angiogram and laboratory examination revealed no abnormal findings. Erythrocyte sedimentation rate, C reactive protein and antiphospholipid antibody were within normal ranges. The patient underwent removal of hematoma by craniotomy. One week after the operation, a subcutaneous hematoma in the area of the craniotomy was found. Cerebral angiography demonstrated an aneurysm of the right superficial temporal artery, which was remote from the craniotomy. This aneurysm was surgically removed and examined. Histopathological examination revealed the presence of a pseudoaneurysm but no inflammation. Although primary angitis of the central nervous system was suspected to be the cause of this disease, a definite diagnosis could not be obtained.