Cases reported "Skull Base Neoplasms"

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1/16. Destruction of the glenoid joint fossa by a tenosynovial giant-cell tumour of the skull base: a case report.

    A 63-year-old man is presented in whom a tenosynovial giant-cell tumour had destroyed the temporomandibular joint fossa and expanded intracranially. The lesion was not diagnosed for a period of at least two years. Treatment included wide resection including the surrounding bone, dura and condyle.
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2/16. Giant prolactinomas presenting as skull base tumors.

    BACKGROUND: Prolactinomas invading the skull base are rare, and could easily be confused with skull base tumors of nonpituitary origin. CASE DESCRIPTION: We report a series of 4 cases of giant prolactinomas invading the skull base and presenting with atypical symptoms. Case 1 presented with a short history of headache and nasal obstruction. Case 2 presented with progressive hypoacusia, dizziness, and ophthalmoplegia. In Case 3, the patient developed rapid progressive visual failure and psychiatric symptoms. Case 4 presented with a 1-year history of headache and retrorbital pain. The diagnosis of prolactinoma was made on the basis of tumor immunohistochemistry and/or high plasma prolactin levels (range from 650-6,500 ng/mL). Medical treatment with the dopamine agonist cabergoline was given; it was effective in normalizing prolactin levels and inducing tumor shrinkage. CONCLUSION: Prolactin levels should be measured in all large skull base tumors involving the pituitary region before any surgery or inappropriate radiotherapy is performed.
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3/16. Application of recombinant activated factor vii during surgery for a giant skull base hemangiopericytoma to achieve safe hemostasis. Case report.

    The authors report on a 64-year-old woman with a huge recurrent skull base hemangiopericytoma, in whom they encountered severe difficulty in attaining intraoperative hemostasis. Standard surgical hemostatic methods and the administration of fresh-frozen plasma and prothrombin complex concentrates failed to stop diffuse bleeding from an inoperable tumor remnant. At a critical point during the operation, the intravenous administration of recombinant activated factor vii, combined with mechanical compression, finally led to satisfactory hemostasis. The rationale for using recombinant activated factor vii in situations of uncontrolled bleeding during neurosurgical procedures is discussed, along with the literature in which the use of recombinant activated factor vii as a maneuver of last resort is reported for hemostasis in other surgical fields.
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4/16. 3-D-conformal radiation therapy for pediatric giant cell tumors of the skull base.

    BACKGROUND: giant cell tumors (GCT) of the base of skull are rare neoplasms. This report reviews the treatment of four pediatric patients presenting with aggressive giant cell tumor, using fractionated and combined, conformal proton and photon radiation therapy at massachusetts General Hospital and Harvard Cyclotron Laboratory. patients AND methods: Three female patients and one adolescent male, ages 10-15 years, had undergone prior, extensive surgical resection(s) and were treated for either primary (two patients) or recurrent (two patients) disease. Gross residual tumor was evident in three patients and microscopic disease suspected in one patient. Combined proton and photon radiation therapy was based on three-dimensional (3-D) planning, consisting of fractionated treatment, one fraction per day at 1.8 CGE (cobalt-gray equivalent) to total target doses of 57.6, 57.6, 59.4, and 61.2 Gy/CGE. RESULTS: With observation times of 3.1 years, 3.3, 5.3, and 5.8 years, all four patients were alive and well and remained locally controlled without evidence of recurrent disease. Except for one patient with partial pituitary insufficiency following radiotherapy for sellar recurrent disease, thus far no late effects attributable to radiation therapy have been observed. CONCLUSIONS: 3-D-conformal radiation therapy offers a realistic chance of tumor control for aggressive giant cell tumor in the skull base, either postoperatively or at time of recurrence. Conformal treatment techniques allow the safe delivery of relatively high radiation doses in the pediatric patient without apparent increase of side effects.
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5/16. Complex tumors of the glomus jugulare: criteria, treatment, and outcome.

    OBJECT: Tumors of the glomus jugulare are benign, slow-growing paragangliomas. Their natural history, surgical treatment, and outcome have been well addressed in the recent literature; however, there remains a subgroup of complex tumors--multiple, giant, malignant, neuropeptide-secreting lesions, and those treated previously by an intervention with an adverse outcome--that is high risk, presents surgical challenges, and is associated with treatment controversy. In this article the authors report on a series of patients with complex glomus jugulare tumors and focus on treatment decisions, avoidance of complications, surgical refinements, and patient outcomes. methods: In this retrospective study, the patient population was composed of 11 male and 32 female patients (mean age 47 years) with complex tumors of the glomus jugulare who were treated by the senior author within the past 20 years. These include 38 patients with giant tumors, 11 with multiple paragangliomas (seven bilateral and four ipsilateral), two with tumors that hypersecreted catecholamine, and one with a malignant tumor. Six patients had associated lesions: one dural arteriovenous malformation, one carotid artery (CA) aneurysm, two adrenal tumors, and two other cranial tumors. All but one patient presented with neurological deficits. Cranial nerve deficits, particularly those associated with the lower cranial nerves, were the prominent feature. Twenty-eight patients underwent resection in an attempt at total removal, and gross-total resection was achieved in 24 patients. Particularly challenging were cases in which the patient had undergone prior embolization or CA occlusion, after which new feeding vessels from the internal CA and vertebrobasilar artery circulation developed. The surgical technique was tailored to each patient and each tumor. It was modified to preserve facial nerve function, particularly in patients with bilateral tumors. Intrabulbar dissection was performed to increase the likelihood that the lower cranial nerves would be preserved. Each tumor was isolated to improve its resectability and prevent blood loss. No operative mortality occurred. In one patient hemiplegia developed postoperatively due to CA thrombosis, but the patient recovered after an endovascular injection of urokinase. In four patients a cerebrospinal fluid leak was treated through spinal drainage, and in five patients infection developed in the external ear canal. Two of these infections progressed to osteomyelitis of the temporal bone. There were two recurrences, one in a patient with a malignant tumor who eventually died of the disease. CONCLUSIONS: Despite the challenges encountered in treating complex glomus jugulare tumors, resection is indicated and successful. Multiple tumors mandate a treatment plan that addresses the risk of bilateral cranial nerve deficits. The intrabulbar dissection technique can be used with any tumor, as long as the tumor itself has not penetrated the wall of the jugular bulb or infiltrated the cranial nerves. Tumors that hypersecrete catecholamine require perioperative management and malignant tumors carry a poor prognosis.
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6/16. Garcin syndrome resulting from a giant cell tumor of the skull base in a child.

    Garcin syndrome is characterized by a progressive ipsilateral involvement of cranial nerves, culminating in paralysis of all or at least seven of them, without sensory or motor long-tract disturbance, with no intracranial hypertension, and with osteoclastic involvement in the skull base on radiographic computed tomography. Giant cell tumor is a primary bone tumor rarely affecting the skull base. An 8-year-old female presented with a 3-month history of increasingly worsening right otalgia, tinnitus, hearing loss, right facial numbness, and diplopia. She was admitted with a 2-week history of swallowing difficulties, voice change, and right shoulder pain. neurologic examination disclosed unilateral paralysis of the right fifth through twelfth cranial nerves, with no other abnormal neurologic findings. Skull radiographic computed tomography revealed lytic lesions in the right temporal petrous portion. Computed tomographic scan indicated a destructive mass involving the right greater wing of the sphenoid bone and temporal petrous apex. magnetic resonance imaging demonstrated a tumor arising from the temporosphenoidal region, infiltrating neither the brain nor the brainstem. No hydrocephalus was observed. biopsy revealed giant cell tumor. Posterior treatment consisted of radiotherapy. At an 8-year follow-up, the patient was well but with functional sequelae. There is no magnetic resonance imaging evidence of tumor growth. No other giant cell tumor presenting as Garcin syndrome is known to have been reported.
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7/16. Secondary aneurysmal bone cyst derived from a giant-cell tumour of the skull base.

    We report a 20-year-old man with a giant-cell tumour of the skull base which showed multiple fluid levels on MRI.
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8/16. Malignant giant cell tumor of the skull base originating from clivus and sphenoid bone.

    We present a case report of a giant cell tumor located in the skull base orginating from clivus and sphenoid bone treated by surgery and external beam radiotherapy (EBRT).
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9/16. Xanthogranuloma of the sellar region.

    The incidence of diagnosed xanthogranuloma of the sellar region is very low [1, 2, 5, 6]. We report about two cases 1) in a 57-year-old female and 2) in a 5-year-old boy. In both cases radiographic findings revealed an inhomogeneous, contrast enhancing sellar lesion. Histopathology showed the typical features of a xanthogranuloma of the sellar region with cholesterol clefts, lympho-plasmacellular infiltrates, marked hemosiderin deposits, multinucleated foreign body giant cells around cholesterol clefts, accumulation of macrophages and only small epithelial cell clusters [6]. As xanthogranuloma of the sellar region are rarely diagnosed we want to draw attention to this rather unusual diagnosis.
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10/16. Major depression with psychosis after resection of a giant middle fossa hemangiopericytoma.

    BACKGROUND: Postoperative onset of acute major depression in a patient with no previous history of psychiatric disorder is highly unexpected after skull base surgery. CASE DESCRIPTION: A 38-year-old woman with no previous physical or mental illness presented with a 3-month history of left ear pain, short-term memory disturbance, and motor dysphasia. magnetic resonance imaging revealed a large extraaxial tumor in the left middle fossa. Left temporal craniotomy was performed, achieving complete tumor resection. The patient showed signs of confusion, disorientation, and severe depression 3 days after the surgery. She developed insomnia and auditory hallucinations along with expressed suicidal ideation, then deteriorated rapidly, necessitating a transfer to a psychiatric unit. In spite of the intensive treatment with antidepressive and antipsychotic medications, she continued to have prolonged psychotic symptoms and depression for several months after surgery. CONCLUSION: Although the incidence is rare, psychiatric complications should be anticipated in patients undergoing resection of a large skull base tumor affecting the temporal lobe. The exact mechanism in this process is not clear; however, it is important to clinically differentiate treatable etiologies such as steroid-induced psychosis and postoperative delirium.
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