Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0017636 (glioblastoma)
 18,345 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 21-year-old man with nasopharyngeal tumor was first admitted to the Nagoya University Hospital on April 15, 1972. He had difficulty in speaking and swallowing, and developed double vision prior to admission. A soft and yellow tumor was found in the nasopharynx and revealed typical features of chordoma. The patient underwent Co60 irradiation after the operation. On January 25, 1973, the patient developed double vision of severe degree. Microscopic examination of the specimen which was obtained at the time of the second operation in February 9, 1973, disclosed a coexistence (collision) of chordoma and hemangioblastoma. The two different tumors were situated in juxtaposition on histological examination. Co60 irradiation was added during his second hospitalization. Three months after the second operation, he developed symptoms of meningitis and was hospitalized for the third time on June 3, 1973, at which time the tumor tissue extended through the right frontal and middle fossa. The third operation was done with frontal craniotomy and tumor was partially removed. The histological diagnosis was hemangioblastoma. Postoperatively the patient went downhill and died on September 19, 1973. The report of a collision tumor of intracranial chordoma and hemangioblastoma is not found in the previous literature. There have been many theories as to the origin of collision tumor. Some investigators have proposed that the existence of hyperplastic blood vessels within the glioblastoma is responsible for the collision tumor of sarcoma and glioblastoma. Since the advent of radiotherapy, several examples of sarcoma have been discovered at postmortem examination in patient irradiated for treatment of cerebral neoplasm, both gliogeneous and nongliogenous, suggesting a possible relationship between the tumor and the radiation therapy. In our case, the chordoma showed neither hyperplastic blood vessels nor malignant pattern on histological examination. It was suspected that post-operative radiation induced the hemangioblastoma. The etiology was discussed from the review of literature.
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PMID:[Intracranial collision tumor--A case report (author's transl)]. 103 89

We report a case of intracranial chondrosarcoma of the skull base with fatal intra- and peritumoral hemorrhage. A 75-year-old woman complained of right blepharoptosis and diplopia in 1989. An initial diagnosis of Tolosa-Hunt syndrome was made, and the patient was treated with steroid hormone therapy at a local hospital. Because the symptoms had not been relieved, she was admitted to our hospital. Computed tomography (CT) scan and magnetic resonance (MR) images demonstrated a large mass extending from the right side of the clivus to the parasellar region and petrous apex. The mass was partially calcified and had destroyed the base of the middle cranial fossa. The lesion had homogeneous enhancement with contrast medium. Preoperative diagnosis was chordoma or chondroma. A biopsy of the tumor was made. The pathological diagnosis of biopsy specimen was chondroid chordoma. The patient was followed up but no palliative treatment such as radiotherapy was given. On June 25, 1991, she suffered from cerebral infarction. On June 29, 1993, she died of sudden respiratory failure. Autopsy was performed. It revealed intra- and peritumoral hematoma compressing the medulla oblongata, pons and midbrain. Histologically immature chondroid cells proliferated in a myxoid-rich extracellular matrix. The tumor cells were composed of hyperchromatic nuclei and eosinophilic cytoplasm, but there was no evidence of notochordal differentiation. Compared with biopsy findings, the tumor showed high cellular density. Immunohistochemically, the tumor cells reacted positively for S - 100 protein, vimentin and cytokeratin, but negatively for epithelial membrane antigen (EMA) and carcinoembryonic antigen (CEA). In view of these histopathological findings, the diagnosis of low-grade myxoid chondrosarcoma was established. Intratumoral hemorrhage often occurs in malignant brain tumors such as glioblastoma and metastatic brain tumor, but chondroid tumors rarely develop a fatal type of intratumoral hemorrhage. Only 8 cases have been reported in detail to date. We discuss the immunohistochemical features and spontaneous intratumoral hemorrhage of chondrosarcoma.
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PMID:[Chondrosarcoma of the skull base associated with fatal intratumoral hemorrhage : report of an autopsy case and review of the literature]. 884 79

The authors retrospectively reviewed 48 patients treated at Seoul National University Hospital (SNUH) between 1986 and 1995. There were 35 children and 13 adults, accounting for 10.1% of 345 pediatric and 0.68% of 1914 adult brain tumors in SNUH during the same period. The 48 cases consisted of 33 cases of germ cell tumor (69%, GCT); 6 of pineoblastoma (PB, 12.5%); 3 of pineocytoma (PC, 6.3%); 3 of anaplastic astrocytoma (6.3%); 1 of astrocytoma; 1 of glioblastoma; and 1 of ependymoma. The median age was 13 years (range 1-59) and the male-to-female ratio was 3.36:1. The most frequent presenting symptom was due to increased intracranial pressure (90%), followed by Parinaud syndrome or diplopia (50%). Patients with a benign tumor, such as teratoma (TE), astrocytoma, or ependymoma, underwent surgery by the occipital transtentorial approach (OTT) for attempted radical resection without adjuvant therapy, while patients with immature teratoma (imTE), PC, and anaplastic astrocytoma underwent regional radiotherapy (RT) after debulking via OTT. Seven patients with nongerminomatous malignant GCT (NG-MGCT) and 3 with germinoma (GE) underwent craniospinal radiation only, 6 with GE, a NG-MGCT, and 2 with GE+TE received craniospinal radiotherapy (CSRT) after debulking via OTT. Three patients with GE, 4 with NG-MGCT, and 3 with PB underwent radiochemotherapy after debulking via OTT. Forty-four patients were followed up after treatment. The median follow-up period was 36 months. All patients with GE were alive after RT at 36 months (median) of follow-up (range 7-70 months). All with GE+TE and TE were alive. Three patients with PC or astrocytoma were also alive with stable or no evidence of disease. In 1 of the 3 cases of imTE there was a recurrence. However, 4 patients with NG-MGCT died, all of whom had undergone CSRT only; 2 PB patients were alive (12, 19 months), 1 in a moribund status (36 months), and 2 were dead (6, 60 months). The overall mean survival time with pineal tumors was 66 months and the 3-year survival rate was 84% with minimal posttreatment complications. It is concluded that pineal region tumors have male and childhood predominances, and the most common tumor is GCT. The majority of pineal region tumors are malignant. Pineal region tumors can be approached safely and effectively and the surgical complications are mostly transient. Their prognosis is dependent on the pathologies and treatment modalities.
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PMID:Pineal tumors: experience with 48 cases over 10 years. 954 42

Glioblastoma, a malignant tumor of neuroepithelial origin, is relatively uncommon in childhood, during which it accounts for 7%-9% of brain tumors. A few patients (about 3%) live more than 5 years. We report a 13-year-old girl who was admitted because one month earlier she had begun to present headache and diplopia. Brain computed tomography (CT) showed a right frontal tumor. At operation, complete excision of the visible tumor was performed. Histologic examination showed that the tumor was a glioblastoma multiforme. The patient underwent 52 Gy of external beam radiotherapy to the enhancing tumor mass plus 3-cm border, and chemotherapy with nitrosourea (BCNU). Fourteen years, 9 months later, the patient presents neither neurological deficits nor radiological relapse. We confirm that younger age, the one immutable prognostic factor, supports a particularly aggressive approach to the treatment of glioblastomas.
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PMID:Long-term survival in a patient with supratentorial glioblastoma: clinical considerations. 1093 61

Astroblastoma is one of the very unusual type of tumors, whose histogenesis has not been clarified. It occurs mainly among children or young adults. Astroblastoma is grossly well-demarcated, and shows histologically characteristic perivascular pseudorosettes with frequent vascular hyalinization. Perivascular pseudorosettes in astroblastoma have short and thick cytoplasmic processes and blunt-ended foot plates. A 15-yr-old girl presented with headache and diplopia for one and a half year. A well-demarcated mass, 9.7 cm in diameter, was found in the right frontal lobe in brain MRI, and it was a well-enhanced inhomogenous mass. Cystic changes of various sizes were observed inside the tumor mass as well as in the posterior part of the mass, but no peritumoral edema was found. Histologically, this mass belongs to a typical astroblastoma, and no sign of anaplastic astrocytoma, gemistocytic astrocytoma or glioblastoma was found in any part of the tumor. Immunohistochemically, the tumor cells showed diffuse strong positivity for glial fibrillary acidic protein, S-100 protein, vimentin and neuron specific enolase, and focal positivity for epithelial membrane antigen and CAM 5.2, while showing negativity for synaptophysin, neurofilament protein, pan-cytokeratin and high molecular weight keratin.
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PMID:Astroblastoma: a case report. 1548 62

An atypical teratoid/rhabdoid tumor (AT/RT) is a highly malignant embryonal tumor most often occurring in the posterior fossa in children younger than 3 years of age. Adult cases of AT/RT are very rare, and 27 cases with a diagnosis of either AT/RT or (malignant) rhabdoid tumor have been reported to date. The authors report an adult case of an AT/RT occurring in the pineal region with molecular cytogenetic and immunohistochemical confirmation. A 33-year-old woman presented with a 2-month history of headache and blurred vision progressing to diplopia, and was admitted emergently due to deteriorating mental status. An MR image showed a heterogeneously enhancing mass involving the posterior third ventricle and pineal region with mild hydrocephalus. She underwent a subtotal resection of the tumor and was then treated with chemoradiation. Thirteen months after surgery, she was still alive with radiological evidence of recurrence/residual lesions. Histological sections showed epithelioid cellular sheets of rhabdoid tumor cells with scattered mitotic figures. Immunohistochemically, the tumor cells were diffusely and strongly positive for epithelial membrane antigen and vimentin, and showed focal expression of glial fibrillary acidic protein, pancytokeratin, and neurofilament protein. Loss of nuclear immunoreactivity for INI1 protein was observed. Fluorescence in situ hybridization analysis showed monosomy 22. Histologically, this tumor consisted exclusively of epithelioid tumor cells with rhabdoid features. The differential diagnoses include rhabdoid glioblastoma, metastatic carcinoma, and rhabdoid meningioma. Molecular testing to identify monosomy 22 or deletions of the chromosome 22q11 containing the INI1/hSNF5 gene and/or immunohistochemical staining with INI1 antibody is of great importance for the diagnosis of this tumor.
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PMID:Atypical teratoid/rhabdoid tumor of the pineal region in an adult. 1991 85

This 13-year-old boy with a history of cranial irradiation for the CNS recurrence of acute lymphocytic leukemia developed a glioblastoma in the right cerebellum. Resection and chemo- and radiotherapy induced remission of the disease. However, recurrence was noted in the brainstem region 8 months later. Because no effective treatment was available for this recurrent lesion, the authors decided to use convection-enhanced delivery (CED) to infuse nimustine hydrochloride. On stereotactic insertion of the infusion cannula into the brainstem lesion, CED of nimustine hydrochloride was performed with real-time MR imaging to monitor the co-infused chelated gadolinium. The patient's preinfusion symptom of diplopia disappeared after treatment. Follow-up MR imaging revealed the response of the tumor. The authors report on a case of recurrent glioblastoma infiltrating the brainstem that regressed after CED of nimustine hydrochloride.
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PMID:Regression of recurrent glioblastoma infiltrating the brainstem after convection-enhanced delivery of nimustine hydrochloride. 2152 93

We present an unusual case of a 41-year-old male patient who presented to the ophthalmology department giving a 3-month history of right sided ptosis, weight loss, diplopia and headache. Clinical examination revealed a right sided relative pupil-sparing third nerve palsy. MRI scan of brain showed thickening of both third nerves. Further investigations revealed a glioblastoma.
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PMID:Gliomatosis presenting as a relative pupil-sparing third nerve palsy in a hypertensive diabetic. 2280 34

We report the case of a young man with sudden onset of diplopia after an upper respiratory tract infection. Based on the first radiological findings acute hemorrhagic leukoencephalitis, a variant of acute disseminated encephalomyelitis, was suspected and treatment with high dose intravenous dexamethasone was started but it was stopped for intolerance. The patient clinically worsened, developing gait instability, ataxia and ophthalmoplegia; brain MRI performed 20 days later showed severe progression of the disease with subependymal dissemination. After brain biopsy of the right temporal lesion the histological diagnosis was glioblastoma. These findings suggest that MRI features of acute hemorrhagic leukoencephalitis may dissimulate the diagnosis of diffuse glioma/glioblastoma. This case underscores the importance of considering diffuse glioma in the differential diagnosis of atypical signs and symptoms of acute hemorrhagic leukoencephalitis and underlines the relevant role of integrating neuroradiologic findings with neuropathology.
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PMID:Diffuse glioblastoma resembling acute hemorrhagic leukoencephalitis. 2918 69

Binocular diplopia and right hemifacial numbness developed in a 52-year-old woman after resection of a right temporal lobe glioblastoma. Based on the Parks-Bielschowsky 3-step test, she was diagnosed with a right cranial nerve (CN) IV palsy in addition to right CN V dysfunction. Iatrogenic diplopia may result from temporal lobe surgery due to the intimate relationship of CN IV and CN III to the mesial temporal lobe. In addition, injury to CN V within Meckel cave is believed to be the cause of facial numbness in some patients after temporal lobe surgery. The anatomy of the intracranial portion of CN IV is reviewed, and the etiologies of CN IV palsy are discussed.
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PMID:Fourth down and five. 2995 65


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