Cranioencephalic cutaneous angiofibromatosis

A nine year old girl with no significant prenatal or perinatal history had presented, at the age of four months, with infantile spasms and later with partial crises with or without secondary generalization. On examination there was left hemiparesis and various skin lesions: a pink plaque in the frontal region with hair loss, fibrous plaques on the right side of the face and hypomelanic spots on the trunk and thighs and some cafe-au-lait spots on the thorax and abdomen. On neuroimaging by CT and MR right cerebral hemiatrophy, periventricular calcifications and lesions in the right hemisphere were seen. On AMR an angioma was seen on the anterior communicating artery. A skin biopsy of the frontal plaque showed perifollicular fibromatosis with marked vascular proliferation. We discuss whether the presentation of this case of encephalocraneo-cutaneous angiofibromatosis is a new phenotype of tuberose sclerosis or a new neurocutaneous syndrome.     

Cardiovascular risk and use of estrogens or estrogen-progestagen combinations. Stanford three-community study

A 59-year-old man was admitted to our hospital with the history of epileptick attack of six years' duration. The seizure was associated with Jacksonian march starting in the right hand and then generalized. Todd's paresis of the right arm followed occasionally to the seizure. He was admitted to neurosurgical unit of other hospital in 1968 and 1971, but on each occasion no tumor or vascular abnormality was detected by extensive examiniations such as brain angiography, pneumoencephalography or brain scanning. He continued his hob as an engineer with anticonvulsant. He once lived in Manchuria in 1930s and had history of pulmonary tuberculosis. He was suffering from diabetes mellitus and chronic otitis media. Recentry he developed headache, forgetfulness, speech disturbance and right hemiparesis and was admitted to our department through psychiatric unit. On examination he was fully conscious but showed typical Gerstmann's syndrome and conduction aphasia. He also revealed bilateral choked disc, right hemiparesis, right hemihypesthesia and right homonymous hemianopsia. The cerebral angiograms and peneumoencephalogram suggested a left parietal cystic tumor. Brain scan with technetium 99m was negative. The spinal fluid was clear but showed slight pleocytosis (99/3/ml). Leucocyte count in the peripheral blood was 6600 per cubic meter with eosinophils of 3%. On craniotomy, small white patches were scattered at the subarachnoidal space suggesting of history of some meningitis. In the left parietooccipital region at Brodmann's area 19, a greyish yellow transparent cystic tumor was found in the subarachnoidal space which was confirmed to be one of the multilocular grape-like cystic tumors extending from area 19, gyrus angularis towards the arcuate fasciculus without continuity with the left lateral ventricle. Microscopic examination showed the racemosal type of cysticercus but no scolex was found. The fluid of the cysts was similar to the spinal fluid. He is totally symptome-free after five months' lapse from the operation except for sporadic spikes on the electroencephalogram. Although some neurosurgeons are against direct operation of the cerebral cysticercosis, we are sure it is possible to cure these patients suffering from chronic cysticercosis with tumor-like symptoms i.e. the tumor type of Stepien. But it is essential not to rupture the cysts during the operative procedure to avoid dissemination of worms which might lead to acute severe cerebral edema. Besides, echinococcus cysts harbouring many worms are often hardly differenciated macroscopically from the cysts of cysticercosis.     

Diffuse cerebral artery vasospasm following total resection of posterior fossa meningioma: a case report]

Diffuse cerebral artery vasospasm following brain tumor resection is a rare complication. The authors reported a case of symptomatic diffuse cerebral artery vasospasm of early phase following resection of a left posterior fossa meningioma. A 50-year-old female patient was admitted to our hospital complaining of headache. No neurological deficits were detected at the time of admission. Computed tomography (CT) and magnetic resonance imaging (MRI) showed a large mass in the left posterior fossa. Cerebral angiography demonstrated mildly diffuse stenosis of the bilateral internal carotid artery. The tumor was resected totally. CT after operation showed a small amount of subarachnoid hematoma in the superior aspect of the cerebellum. Pathological specimen of the tumor showed fibrous meningioma. One day after this radical operation, the patient was found to have weakness in her left leg. Then she developed left hemiparesis, weakness in the right leg and left homonymous hemianopsia. MRI showed ischemic lesions in the bilateral parietal and the occipital lobe. Angiography demonstrated diffuse severe vasospasm throughout the whole cerebral artery. Ten days after the operation, angiographical findings were improved. This case indicates that vasospasm may occur even after resection of brain tumors which are localized outside the suprasellar area.     

Intraventricular, cystic, atypical meningioma

A 37-year-old male presented with a very rare cystic meningioma in the trigone of the left lateral ventricle. Neurological examination revealed mild conduction aphasia and right hemisensory disturbance. Computed tomography and magnetic resonance imaging showed a solid, enhancing tumor in the left trigone which had multiple cystic components located anteriorly and superiorly. The tumor was totally resected via a parietal, transventricular approach. Histological examination revealed an atypical meningioma with cellular pleomorphism and prominent nucleoli. Both the solid component and the cyst wall consisted of tumor cells.     

Cervical lymph node metastatic patterns of squamous carcinomas in the upper aerodigestive tract

We report a patient with medial medullary infarction who showed deep sensory impairment as his prominent neurological manifestation. A 54-year-old man with a history of hypertension was admitted to our hospital with numbness of the bilateral upper and lower extremities, followed by dysarthria and right hemiparesis. Physical examination revealed no abnormalities except for high blood pressure. He hiccuped continuously. On neurological examination, he exhibited dysarthria, mild dysphagia and right hemiparesis without facial or lingual paresis. Sensitivity to light touch and pinprick was normal, but sensitivity to vibration and joint position was severely decreased in the bilateral upper and lower extremities, predominantly in the lower extremities and on the right side in the upper extremities. He had been treated with antiedema agents and thromboxane synthetase inhibitor. His hiccups stopped within two weeks, and his right hemiparesis gradually improved within one month. However, his deep sensory impairments remained prominent. Blood examinations disclosed positive lupus anticoagulant. MRI showed bilateral infarction at the medial portion of the upper medulla oblongata, extending to both pyramids, especially on the left. Somatosensory evoked potentials (SEP) after median nerve stimulation showed P14 and the later components with prolonged latency. No SEP were recorded after posterior tibial nerve stimulation. The latency of P14 was well correlated with the severity of deep sensory impairments in the upper extremities. Neurological manifestations of our patient are not typical of medial medullary infarction, and are informative about the functional anatomy of the deep sensory tract in the medulla oblongata. We discuss the relation of the intractable hiccups to the bilateral medial medullary lesions, and emphasize the importance of lupus anticoagulant as one of the risk factors in brainstem infarction.     

Villoglandular adenocarcinoma of the cervix: cytologic presentation

A 69-year-old left-handed man developed Broca aphasia and jargon agraphia following a right cerebral infarction. He learned to write with his right hand. There is no family history of left-handedness. Neurological examination revealed hemiparesis, sensory impairment, and hyperreflexia with pathological reflexes on the left side. An MRI demonstrated lesions in the area including the pre- and post-central gyri, the posterior part of the middle and inferior frontal gyri, and inferior parietal lobule on the right side. Neuropsychologically he was alert and cooperative. He demonstrated severe Broca aphasia and his verbal output was limited to a few residual words. On the other hand, he could communicate through writing Kanji words. Writing words was relatively preserved with Kanji but not with Kana characters. Asked to write a Japanese folk tale, he showed jargon agraphia mixed with some correct Kanji words. His comprehension of spoken and written sentences was well preserved. He showed mild ideomotor and buccofacial apraxia, left unilateral spatial neglect, and constructional impairment. Dissociation between spoken and written language is a prominent feature of this patient's language output. In this patient, right hemisphere seems to be dominant for language and praxis, and left hemisphere for motor engram of writing. Learning to write with the right hand may have enhanced the establishment and maintenance of motor engram for writing in the left hemisphere. The intact motor engram of characters in the left hemisphere could be retrieved by the right hand without control from the language area in the diseased right hemisphere, resulting in jargon agraphia especially with Kana characters. In left handed people, the hand with which they learn to write may effect interhemisphere lateralization of language functions.     

Extracerebral metastases of a glioblastoma, in the absence of surgery

A 50 y.o. male presented with a right parietal tumor which was a glioblastoma on stereotactic biopsy. He was treated by radiation and steroids, with clinical improvement. Four months later, he presented with a left preauricular mass and cervical lymphadenopathy. CT scan showed destruction of the left mastoid and filling of the left tympanic cavity. One month later, he suffered progressive dyspnea. Chest X ray showed a mediastinal mass on the right side and numerous bilateral interstitial opacities in the lungs. A bronchial biopsy was inconclusive. His general condition worsened, and he died. Postmortem showed continuous neoplastic infiltration of the left part of the base of skull, extending into the neck. Numerous metastases were present in mediastinal lymph nodes, lung parenchyma, pleura and pleural aspect of the diaphragm. There were no subdiaphragmatic metastases. Neuropathological examination confirmed a poorly differentiated highly malignant glioblastoma with severe necrosis involving the internal part of the parietal lobe extending to the dura mater of the convexity and falx cerebri with invasion of the superior longitudinal sinus which was entirely occluded. The biopsy scar was not infiltrated. Visceral tumors were morphologically identical to the brain tumor. They were strongly GFAP positive and cytokeratin negative. Extraneural metastases of glioblastoma in the absence of surgery are uncommon in adults. Involvement of the dura mater and/or superior longitudinal sinus is an almost constant feature. In our case, this may have led to invasion of the base of skull and secondary regional, lymphatic, and hematogenous spread.     

A comparison of two oral ketamine-diazepam regimens for sedating anxious pediatric dental patients

A 62 year-old man was admitted with a right hemiparesis, sensory aphasia and right hemianopia which appeared on awakening. He was initially thought to have a stroke, but EEG showed diffuse slowing and both CT scan and MRI irregular white matter lesion suggesting a leucoencephalopathy. His neurological deficit regressed, and he was discharged after 2 weeks. He was readmitted 6 months later because of mental confusion. MRI revealed diffuse white matter lesions extending up to the frontal lobes, these were hyperintense on T2 weighted images and suggested the diagnosis of gliomatosis cerebri (GC). The patient became progressively comatose and died 6 weeks later. At autopsy the brain looked diffusely swollen with irregular greyish areas of the white matter of both centrum ovale and brain stem. On microscopic examination the cerebrum and brain stem were diffusely and asymmetrically infiltrated by numerous neoplastic glial cells without angiogenesis or disruption of architectonic boundaries. There were no mitoses nor necrosis. Many tumour cells were GFAP- and S100-positive. A high proportion of cells contained the leucocyte antigen Leu-7. This case of gliomatosis cerebri is compared to the 9 published cases of GC with an initial focal neurological deficit and to the 19 publications reporting MRI results. The controversial nosological boundaries and etiopathogenetic hypotheses of this peculiar neoplastic disease are discussed.     

The identification of the best thrombolytic. Reflections on a problem without resolution

A rare diffuse arteriovenous malformation (AVM) occurred in an 11-year-old boy with a 4-year history of transient ischemic attacks and weakness of the left extremities. Postcontrast computed tomography (CT) showed cord-like enhancement in the corpus callosum and pineal region. Magnetic resonance (MR) imaging demonstrated atrophy of the right hemisphere and multiple signal voids in the sulci. Cerebral angiography showed a diffuse AVM fed by the bilateral anterior cerebral and right middle cerebral arteries. The AVM nidus consisted of scattered nidi. Several draining veins appeared as multiple signal voids on MR images. No surgery was possible because of the scattered nidi. Left hemiparesis and mental retardation worsened, with progressive atrophy of the right hemisphere on serial MR images. Follow-up radiological examinations showed that the number of nidi increased, the draining vein enlarged, and the gyri calcified. The gyriform calcification on CT scans strongly resembled that seen in Sturge-Weber syndrome.     

Magnetic resonance imaging in the staging of solitary plasmacytoma of bone

PURPOSE: To assess prospectively the role of magnetic resonance (MR) imaging in the staging of patients with a solitary bone plasmacytoma (SBP). PATIENTS AND METHODS: Twelve consecutive patients with an apparent SBP underwent MR imaging of both the primary tumor and the thoracic and lumbosacral spine to seek additional foci of marrow involvement that might have been undetected by standard skeletal survey. All patients received megavoltage irradiation (total dose, 40 Gy) to the primary lesion. RESULTS: MR imaging of the thoracic and lumbosacral spine showed additional foci of marrow replacement in four of 12 patients, with signal characteristics identical to those of the primary tumor. In all four patients, the abnormal protein persisted at greater than 50% of the pretreatment value following radiation treatment. In contrast, the myeloma protein disappeared or was reduced by greater than 50% in five of the six patients with secretory disease and without additional marrow abnormalities. One of four patients progressed to multiple myeloma 10 months after diagnosis with new lesions on conventional radiographs in the same areas as detected previously by MR imaging. CONCLUSION: Four of 12 patients considered to have a SBP by standard criteria may have been understaged, because MR imaging showed additional marrow abnormalities consistent with myeloma. MR imaging of the spine may contribute to the initial staging of SBP, especially since some patients may be cured with radiotherapy.     

Preliminary experience with MR-guided thermal ablation of brain tumors

PURPOSE: To evaluate the feasibility of a technique of MR-guided stereotactic radio frequency ablation, which was developed as a minimally invasive treatment for brain tumors, and to determine MR characteristics and sequential evolution of radio frequency lesions created to ablate brain tumors. METHODS: Fourteen lesions in 12 patients with primary and metastatic brain tumors were treated with this technique and followed for up to 10 months. The stereotactic coordinates of the tumor and the angle of the radio frequency probe were calculated on MR imaging. The radio frequency lesion was generated in the awake patient by increasing the temperature to 80 degrees C within the tumor for 1 minute. This was repeated until the entire tumor volume was destroyed. MR imaging was performed before, during, and immediately after the radio frequency procedure, and sequential MR was obtained during clinical follow-up. RESULTS: MR imaging clearly showed well-defined radio frequency lesions and provided feedback for treatment planning. The radio frequency lesion boundary was well identified as a dark signal rim on T2-weighted images and showed ring enhancement on contrast-enhanced T1-weighted images. The sequential MR imaging showed the radio frequency lesions decreased in volume in all cases, suggesting focal control. CONCLUSION: Stereotactic MR-guided radio frequency brain tumor ablation is a feasible and promising technique that can be an attractive brain tumor treatment alternative. MR provided not only accurate tumor location but also visualization of feedback of thermal tissue changes that reflected therapeutic effect.     

A case of left ventricular hemangioma

Left ventricle hemangioma in a 54-year-old man is reported. He was admitted to our hospital for chest discomfort. Coronary angiograms showed normal coronary arteries and a contrast blush in the left interventricle septum. Transesophageal echocardiogram also demonstrated the small floating tumor in the ventricular septum below the right coronary cusp. With the aid of cardiopulmonary bypass, the tumor was resected completely through transaortic approach. Histological examination revealed capillary hemangioma. The patient had uneventful recovery. To our knowledge, this is the seventh case of a left ventricular hemangioma treated surgically reported in the world literature.     

Multiple angiomyolipomas of the liver (case report)

Angiomyolipoma of the liver is an extremely rare type of tumor and reported cases are few and far between. This case study reports on a 37-year-old woman who suffered from multiple angiomyolipomas of the liver. Ultrasonography (US) and computed tomography (CT) initially revealed the existence of two tumors located in the right lobe of the liver and measuring 20 x 15 mm and 8 x 7 mm in size. The patient underwent partial resections of the liver. Histopathologic examination of both the resected specimens revealed angiomyolipoma. This is the first reported case of multiple angiomyolipomas of the liver. Furthermore, the lesions of our case study were small in size and rich in fat component. Under such conditions, preoperative examination resulting in an angiomyolipoma diagnosis is difficult even with the aid of imaging modality and needle biopsy techniques.     

The role of luminal bacteria in colitis: more than an antigenic drive

Cardiac hemangioma is a rare benign vascular tumor. A 44-year-old woman with hemangioma arising from the left atrium and transient atrial fibrillation is reported. Echocardiography, computed tomography and magnetic resonance imaging suggested a hypervascular tumor with clear borders in the pericardial cavity. Selective coronary angiography demonstrated feeding arteries arising from the right and left circumflex coronary arteries. Under cardiopulmonary bypass the tumor was successfully resected. Pathological examination showed a cavernous hemangioma. There is no evidence of recurrence and the patient is doing well three years after the operation.     

A case of dural arteriovenous fistula in the transverse and sigmoid sinus which developed over five years

We report a case of dural arteriovenous fistula (dAVF) of the transverse and sigmoid sinus which developed over five years. The patient was a 64-year-old man, who had a history of congestive heart failure and transient ischemic attack (TIA). No abnormal lesions were detected in the cerebral angiograms five years prior to the time the etiology of his TIA was investigated. He suddenly suffered from left hemiparesis and the CT scan showed right parietal subcortical hemorrhage. Right carotid angiogram demonstrated dAVF of the transverse and sigmoid sinus supplied by the right occipital and ascending pharyngeal arteries. We thought the reason for the subcortical hemorrhage in this patient was due to the disturbance of venous return in the right cerebral hemisphere. We underwent direct embolization of the right transverse sinus by interlocking detachable coils. Postoperative cerebral angiograms demonstrated the disappearance of dAVF and he was discharged. Whether dAVF is a congenital anomaly or not, has been controversial. Since the dAVF in this case had newly developed over five years, it is suggested that dAVF is an acquired lesion.     

Mediastinal germ cell tumor complicated by visceral hemangiomatosis

This report describes an extremely rare combination of mediastinal germ cell tumor and visceral hemangiomatosis in a 17-year-old boy who initially presented with chest pain and dyspnea. He was treated with chemotherapy consisting of cisplatin, cyclophosphamide, bleomycin, vinblastine, and dactinomycin followed by surgery. Multiple low-density nodules developed in the spleen three weeks later, suggesting metastases from the primary tumor, but the resected specimen showed cavernous hemangiomas within the splenic parenchyma. The patient died of recurrence of germ cell tumor 19 months after the initial treatment. Postmortem examination disclosed multiple hemangiomas in the lung and liver similar to those in the spleen.     

Debt and career choices of underrepresented minorities

We report a case of right aortic arch with mirror-image branching and coarctation of the aorta and left ductus arteriosus in a 6-year-old boy. He was referred to Chang Gung Children's Hospital with suspected left ventricular failure. Physical examination revealed hypertension and a pulse discrepancy between the upper and lower extremities. Echocardiography and magnetic resonance imaging revealed a right aortic arch, a long segmental aortic coarctation, and a patent ductus arteriosus. In addition, mirror-image branching of the brachiocephalic vessels was found. The coarctation was resected and the ductus arteriosus was ligated. The postoperative recovery was uneventful and the blood pressure returned to normal. He remained normotensive and had no symptoms of heart failure within the 2 years of follow-up.     

Flap of the musculus latissimus dorsi to prevent alveolar air leakage from sectional plane of the lung after resection of metastatic pulmonary and chest wall tumor

Alveolar air leakage after pulmonary resection usually heals with adequate pleural drainage, but must be more actively treated if leakage may be severe. If left untreated, the postresection space can lead to empyema. We used a muscle flap to prevent alveolar air leakage from a large sectional plane of the lung resected because of metastases in the lung and chest wall. A 48-year-old man complained of pain and a mass on the right side of his back. He had undergone resection and chemotherapy for clear cell sarcoma that originated on the back of the left hand when 43 years of age, wedge resection of the right lower lobe of the lung for a metastatic pulmonary tumor at 46 years, and lobectomy of the same lobe for a recurrence of the metastatic pulmonary tumor at 47 years. The diagnosis was of a metastatic tumor to the right chest wall with peripheral pulmonary tumors of the right upper and middle lobes. Resection of the chest wall and the lung including the tumors was done. Much air leakage from the extensive sectional plane of the right upper and middle lobes was seen intraoperatively, and this plane was therefore covered with a flap of the musculus latissimus dorsi. Chest tubes were removed on day 7 postoperatively when air leakage was no longer seen. Subcutaneous emphysema, which appeared on day 14 postoperatively, required redrainage of the pleural air space, but pleurodesis was effective. Use of a muscle flap was simple and effective for covering of a sectional plane of the lung, and should be considered when alveolar air leakage may be extensive.     

A case of gliosarcoma associated with large cyst

A case of gliosarcoma with a large cyst is reported. A 22-year-old female was admitted to our hospital with complaints of blurred vision and headache. Plain skull x-ray films showed a radiolucent area in the right frontal area. Computed tomography (CT) revealed an iso-dense mass in the right frontal lobe with a large cyst. After administration of contrast medium, the solid part and cyst wall were well enhanced and the content of the cyst was slightly enhanced. CT number of the cyst fluid was increased from 64.2 to 83.5 Hounsfield units, after administration of the contrast medium. Axial T1-weighted magnetic resonance image (MRI) revealed an iso-intense mass with marked enhancement by Gd-DTPA in the same area. A large cyst was shown to be located in the dorsal part of the mass. A small round protrusion, 10 mm in diameter, was found on the anterior portion of the mass on this MRI. Right carotid angiogram showed a tumor stain fed by the frontopolar artery. Right frontal lobectomy including the tumor was carried out with a preoperative diagnosis of glioblastoma. The patient received radiation therapy of 60Gy (whole brain 40Gy; focal 20Gy) and chemotherapy postoperatively. Histologically, necrosis, hemorrhage and endothelial hyperplasia were revealed at the tumor lesion. The tumor was composed of proliferation of glial and mesenchymal elements. The glial element appeared as fibrillary astrocytoma and polar spongioblastoma. The mesenchymal element showed sarcoma. As mentioned above, this tumor was diagnosed as gliosarcoma. It was difficult to make a diagnosis of gliosarcoma preoperatively because of the complex findings similar to malignant gliomas in conventional neuroradiological imaging.(ABSTRACT TRUNCATED AT 250 WORDS)     

Tumor involvement in hepatic veins: comparison of MR imaging and US for preoperative assessment

PURPOSE: To compare use of magnetic resonance (MR) imaging and ultrasonography (US) for diagnosis of vascular involvement by tumor at the hepatic vein confluence. MATERIALS AND METHODS: Thirty-seven consecutive patients with tumors at the hepatic vein confluence were prospectively evaluated with spin-echo and gradient-echo MR imaging and gray-scale and Doppler US. Encasement, thrombosis, occlusion, and nonvisualization were considered to be evidence of vascular involvement. Imaging results were compared with surgical and pathologic examination findings in 27 patients who underwent resection. RESULTS: Sixteen hepatic veins (nine right, four middle, three left) were seen to be involved at surgery. Twelve of 16 involved veins were identified at MR imaging (75% sensitivity, 98% specificity, 92% positive predictive value, 94% negative predictive value). Thirteen of 16 involved veins were detected at US (81% sensitivity, 97% specificity, and 87% positive and 95% negative predictive values). There was one false-positive diagnosis of inferior vena cava involvement at both MR imaging and US. Ten patients had unresectable disease. One patient had motion artifact on MR images; in the remaining nine patients, MR imaging and US yielded identical findings at 26 of 27 hepatic vein sites. CONCLUSION: MR imaging and US provide comparable results for assessment of hepatic vein involvement by tumor.