Cases reported "Brain Stem Neoplasms"

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1/6. Diffuse brainstem glioma in a patient with Laurence-moon-(Bardet-)Biedl syndrome.

    An autopsy case of a patient with diffuse brainstem glioma associated with Laurence-moon-(Bardet-)Biedl syndrome is described. The subject was a 25-year-old woman who had been suffering from mental retardation, pigmented retinopathy, obesity, hexadactyly, amenorrhea and renal cysts. She developed dizziness, headache and consequent consciousness disturbance. Magnetic resonance images disclosed marked swelling of the pons without contrast enhancement. By means of combined chemotherapy and radiation, she survived for 15 months. Histopathological diagnosis for postmortem specimens obtained from the brainstem was glioblastoma multiforme. No pathogenetic association between the syndrome and brainstem gliomas is known, and the literature contains no cases of patients with this coincidence.
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2/6. diagnosis and results of treatment with radiation therapy in gliomatosis cerebri patient: case report.

    Gliomatosis cerebri (GC) is a rare disease loosely defined as a diffusely infiltrating glioma involving extensive areas of the brain. The prognosis is poor and no definite treatment has proven effective for GC. Little information exists regarding the role of radiation therapy (RT) for GC, but some researchers have suggested that it is a good choice of treatment from their limited experience. In this report, we present a case with imaging and histological diagnosis of GC and demonstrate the treatment results of RT. The patient was a 39-year-old woman with progressive symptoms of dizziness, unsteady gait, headache, vomiting, and consciousness disturbance for 6 months. She received a series of radiographic examinations and surgical interventions for diagnosis. The definite diagnosis of GC was made by a combination of magnetic resonance imaging (MRI) findings and histological examinations. Forty Gray (Gy) of whole brain irradiation followed by 14 Gy reduced-field boosts were given to her. The MRI, following treatment, showed regressive changes, and clinical symptoms were slightly improved. The patient survived 19 months after the diagnosis, which is longer than the average survival time of patients without treatment.
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3/6. Pontine malignant astrocytoma with hemorrhagic onset--case report.

    A 7-year-old boy presented with acute onset of left hemiparesis and headache, followed by disturbance of consciousness. neuroimaging studies showed pontine hemorrhage. Surgery was performed to remove a massive hematoma. Histological examination of the wall revealed anaplastic astrocytoma. Postoperative radiation therapy and several types of chemotherapy were administered. However, the tumor recurred and he died 9 months after onset. Hemorrhagic onset of pontine glioma is rare and carries an extremely poor prognosis.
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4/6. Locked-in syndrome due to metastatic pontomedullary tumor--case report.

    A 21-year-old man presented with an extremely rare case of locked-in syndrome caused by a metastatic brainstem tumor manifesting as quadriplegia, lower cranial nerve pareses, and irregular respiration. Cranial magnetic resonance imaging revealed a large pontomedullary tumor. An emergency operation was performed via a posterior fossa approach and the tumor was grossly totally removed. The histological diagnosis was malignant melanoma. The clinical status of the patient remained unchanged and he died on postoperative day 34 of diffuse bronchopneumonia. Locked-in syndrome is characterized by quadriplegia, lower cranial nerve paralysis, and mutism but with maintenance of consciousness, as well as vertical eye movements and eyelid blinking. This case suggests that locked-in syndrome should be considered in any patient seemingly comatose or stuporous, this syndrome may be due to a pontomedullary tumor, and malignant melanoma metastasis should be considered in the differential diagnosis of patients who present with brainstem tumor.
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5/6. A brainstem cavernoma demonstrating a dramatic, spontaneous decrease in size during follow-up: case report and review of the literature.

    BACKGROUND: Many reports have demonstrated a worse prognosis for patients whose cavernomas were subtotally removed than for those whose cavernomas were not surgically treated. Therefore, it is better not to touch the cavernoma if a surgeon is not prepared to totally remove it. This report describes a large brainstem cavernoma showing a spontaneous, dramatic reduction in size after removal of only the biopsy specimen of the lesion. CASE DESCRIPTION: A 42-year-old woman experienced facial numbness, diplopia, and ataxia. A magnetic resonance (MR) study revealed a pontine cavernoma with hemorrhage. Two weeks later, recurrence of the patient's symptoms and consciousness disturbance were noted. An MR study revealed massive hemorrhage from the cavernoma with a ventricle dilatation. An operation on the lesion was conducted 3 months after the initial hemorrhage. However, the operation was aborted when an exploration of the lesion showed a tight adhesion between the lesion and the pons. Removal of only a biopsy specimen and evacuation of the suckable hematoma were done. No neurologic recovery and no decrease in the size of the cavernoma were detected postoperatively. Her family did not wish for further treatments. She was in a bedridden state with severe brainstem dysfunction when she was transferred to a local hospital. Sixteen months after the surgery, her consciousness was clear, and MR imaging confirmed a marked reduction in the size of the cavernoma. CONCLUSION: A dramatic, spontaneous decrease in size does occur even in the case of a large brainstem cavernoma showing hemorrhages. Conservative therapy may be one of the treatment options for the symptomatic brainstem cavernoma.
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6/6. Excision of juvenile pilocytic astrocytoma of the midbrain after radiotherapy.

    A 13-year-old girl presented with consciousness disturbance, right hemiparesis, and impairment of hearing, swallowing, and ocular movements. A magnetic resonance image demonstrated marked hydrocephalus due to a large cystic tumor (40 x 40 x 30 mm) in the midbrain. Emergency ventricular drainage and stereotactic cyst puncture dramatically improved her condition. Since her family did not agree to surgery on the brain stem, radiotherapy was performed after ventriculoperitoneal shunting. Conventional radiotherapy of 50.4 Gy was temporarily effective, and the growth of the tumor was stabilized until approximately 8 months later when regrowth was noticed. At this stage, with the consent of her family, the tumor was removed via an infratentorial supracerebellar approach. The pathological diagnosis was juvenile pilocytic astrocytoma. The postoperative course was uneventful. No recurrence has been observed during the follow-up period of more than 6 years. This case study shows that initial excision may be appropriate for some low-grade focal lesions in the midbrain when the histological diagnosis and consent have been obtained.
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