Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0018681 (headache)
 56,091 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We describe a 34-year-old man who presented with headaches for about 3 months. Magnetic Resonance Imaging (MRI) revealed a large cystic tumor, involving the right frontoparietal lobe region. Pathological study revealed a papillary glioneuronal tumor (PGNT) with mitotic activity and a Ki-67 labeling index of approximately 15%. Five years after radical surgery and radiotherapy, the patient is symptom-free, without tumor recurrence or residual tumor. This case supports the existence of an atypical variant of PGNT, with mitotic activity and high proliferative index, and suggests that in these tumors, histological findings of malignity not necessarily indicate a short-term unfavorable behavior.
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PMID:Atypical papillary glioneuronal tumor. 1728 29

There have been only 26 cases of hypoglossal schwannomas reported to originate intradurally and extend extradurally into the hypoglossal canal. This 31-year-old mother of two children presented with a 5-day history of progressive headache, nausea, vomiting and vertigo. Her neurological exam was significant for nystagmus and left tongue deviation with marked atrophy. An initial head CT revealed extensive left hypoglossal canal erosion with 4th ventricle compression. T1-weighted MR images with contrast revealed a 4x3 cm left cerebellopontine angle non-homogeneously enhancing mass with an intracranial cystic component and prominent extension into the eroded hypoglossal canal. A left lateral suboccipital craniotomy was performed for subtotal microsurgical resection of the intradural posterior fossa mass. A schwannoma was diagnosed after resection and gamma knife surgery (GKS) was performed three months later for the extradural residual tumor without further deficits. This is a rare report of a hypoglossal schwannoma in a young patient who was treated with a multimodality approach in order to minimize risks. A review of the literature and discussion of the respective benefits of microsurgery versus GKS and long-term follow-up issues are presented.
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PMID:Intradural microsurgery and extradural gamma knife surgery for hypoglossal schwannoma: case report and review of the literature. 1821 Mar 63

We report a 3-year-6-month-old boy with chordoid meningioma in the foramen magnum. Chordoid meningioma represents between 0.5 and 1.0% of all meningiomas, and it is frequently located in the supratentorial region. The patient started with an episode of instability, falls, and headache; after which, he developed cuadriparesis, cervical pain, and neck stiffness, which slightly improved after medical treatment, so he was referred to our hospital. Physical examination revealed left hemiparesis and cervical muscle spasm with left torticollis. Magnetic resonance imaging (MRI) demonstrated an intradural-extramedullary well-circumscribed, homogeneous enhancing mass, in the anterior part of the foramen magnum with cervical extension. The operative technique was the extreme-lateral craniocervical retrocondylar approach with total removal and dural coagulation. Histopathological examination demonstrated a chordoid meningioma, with meningotelial basophilic mucoid chordoma-like component in 80% of the tissue. The immunohistochemical stains were positive to epithelial membrane antigen, vimentin, and S-100 protein. At 10 months follow-up, the patient showed improvement in his neurological deficit with physical rehabilitation, and motor response in his extremities are now normal; neck stiffness with cervical spasm disappeared postoperatively. MRI showed no residual tumor. To our knowledge, this is the first report of a chordoid meningioma on the foramen magnum in a child.
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PMID:Chordoid meningioma of the foramen magnum in a child: a case report and review of the literature. 1821 97

Pituitary tumor apoplexy is an uncommon syndrome resulting often spontaneously from hemorrhage or infarction of a pre-existing pituitary adenoma. As the primary event involves the adenoma, the syndrome should be referred to as pituitary tumor apoplexy and not as pituitary apoplexy. The sudden increase in sellar contents compresses surrounding structures and portal vessels, resulting in sudden, severe headache, visual disturbances, and impairment in pituitary function. Initial management of patients with pituitary tumor apoplexy includes supportive therapy (intravenous fluids and corticosteroids), following which many patients exhibit clinical improvement. Because those patients can be effectively managed with supportive measures, many who remain clinically and neurologically unstable might benefit from urgent surgical decompression by an experienced neurosurgeon. All patients presenting with this syndrome require long-term follow-up to treat any residual tumor and/or pituitary dysfunction. Close interaction between members of the management team is necessary for optimal patients' outcome.
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PMID:Pituitary tumor apoplexy: a review. 1837 48

Medulloblastoma is one of the most common malignant childhood brain tumors. It is a primitive neuroectodermal tumor (PNET) and predominantly arises in the cerebellum and 4th ventricle. Most cases of medulloblastoma are sporadic, but some predisposition syndromes are known, such as SUFU and Gorlin syndromes. Most often intracranial hypertension reveals the disease typically with headache and vomiting. However, the frequent atypical presentation should not delay neuroradiological investigations. Brain and spinal MRI can establish the diagnosis of posterior fossa tumor and define the extent of the disease. CSF study completes the staging. Histologic examination of the tumor confirms the diagnosis of medulloblastoma. Patients are classified into 2 risk groups: standard-risk medulloblastoma, defined by nonmetastatic disease treated by total or subtotal tumor resection; and high-risk patients who have disseminated disease and/or residual disease. Tumor molecular genetic findings allow the use of emerging prognostic factors and may ultimately contribute to the development of targeted therapy. Current treatment in the oldest children combines surgical resection followed by radiotherapy and chemotherapy. The aim of recent studies was to increase survival and decrease sequelae by reducing CSI in older children with standard risk medulloblastoma. Treatment in younger patients is as much as possible restricted to surgery and chemotherapy. However, long-term sequelae after treatment for medulloblastoma remain frequent and the detection and treatment of those sequelae is an essential part of the follow-up of the patients.
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PMID:[Childhood medulloblastoma]. 1899 98

Metastatic meningioma is extremely rare, occurring in an estimated 0.1% of cases. We report a case of pediatric meningioma metastatic to cervical soft tissue and lymph nodes. An 8-year-old boy presented with headaches, dizziness, and involuntary eye flickering. Magnetic resonance imaging (MRI) revealed a 7.5-cm parasagittal, dural-based mass with venous sinus encasement. Therapeutic embolization was followed by bilateral craniotomy, achieving subtotal resection. Histopathologic examination revealed an atypical meningioma with regions of hypercellularity, small cell morphology, sheeting architecture, increased mitoses, and brain invasion. Surveillance MRI studies showed growth of residual tumor and enlarging cervical soft tissue masses with posterior triangle lymphadenopathy. Radiation and surgical resection were employed for the intracranial tumor burden; resection of the soft tissue masses revealed metastatic meningioma, with soft tissue infiltration and metastasis to 8 lymph nodes. This case demonstrates the aggressive biologic potential of pediatric meningiomas, with potential for distant spread via cerebrospinal fluid leakage and lymphatic invasion.
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PMID:Aggressive pediatric meningioma with soft tissue and lymph node metastases: a case report. 1908 45

We reported a 32-year-old male with a sellar solitary fibrous tumor who presented with headache and affliction in the left eye. Serum biochemical examination revealed hypoglycemia. The tumor was assumed to be a nonfunctional pituitary adenoma preoperatively. A subtotal resection of the tumor was performed. Immunohistochemically, atypical solitary fibrous tumor was established. The residual tumor had no progression or distant metastasis at a 44-month follow-up after gamma-knife stereoradiotherapy.
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PMID:A primary atypical solitary fibrous tumor of the sella mimicking nonfunctional pituitary adenoma: a case report. 2022 65

We report a boy who in 1994, at the age of 11, presented with headache and vomiting. The fi nal diagnosis was papillary tumor of the pineal region (PTPR). Magnetic resonance imaging (MRI) revealed a heterogeneous mass. Hydrocephalus was addressed by immediate ventricle drainage; subsequently, we attempted tumor removal. As the intraoperative diagnosis of the hemorrhagic tumor was primitive neuroectodermal tumor (PNET), we did not proceed to total removal. After the delivery of radiotherapy (50.4 Gy) and one course of Nimustine hydrochloride (ACNU) chemotherapy, the residual tumor was completely resected. The diagnosis at that time (1994) was papillary pineocytoma. He was followed on an outpatient basis for 15 years and remained free of recurrence. This type of tumor was later proposed to represent a new distinct tumor subtype, papillary tumor of the pineal region (PTPR). Our data indicate that our patient's tumor should be included in this category.
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PMID:Successful treatment of neoadjuvant therapy for papillary tumor of the pineal region. 1985 18

Malignant sweat gland tumors are rare neoplasms with high recurrence and metastasis rates of over 50%. Clinically, they are often either not diagnosed or diagnosed improperly and are encountered as a histological surprise. Herein, we report a 50-year-old woman who suffered from chronic headaches and a left-side limping gait. Magnetic resonance imaging revealed a T1 and T2 heterogeneous intense dural-based lesion at right-frontal convexity. The pathological diagnosis of papillary meningioma was rendered at the time. Because there was no evidence of residual tumor, tumor recurrence, or distant metastases during the three-year follow-up, the clinician believed questioning the initial diagnosis was warranted. After pathological review, the final diagnosis was low-grade hidradenocarcinoma. A review of the literature suggests this is the first reported case of dural-based hidradenocarcinoma with local brain invasion. Given the lack of scalp or skull bone involvement, we speculated that the tumor may have arisen from ectopic sweat gland cells entrapped in the dural mater.
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PMID:Malignant sweat gland tumor presenting as an unusual dural-based lesion: case report. 2006 41

The approach to a patient with acromegaly and persistent disease after surgery requires a complex diagnostic assessment. Acromegaly is a chronic and insidious disease that is associated with multisystem comorbidities, including cardiovascular disease, hypertension, sleep apnea syndrome, colon polyposis, arthropathy, and metabolic complications including glucose intolerance and type 2 diabetes mellitus. Patients also have a variety of signs and symptoms, including headache, arthralgias, carpal tunnel syndrome, sweating, fatigue, and psychological issues that impact significantly on quality of life. The recommended approach to the evaluation of the postoperative patient includes a biochemical assessment, with measurement of serum IGF-I along with a glucose-suppressed GH value, radiological assessment to determine location of residual tumor and presence of mass effects, a physical examination for evidence of skeletal and soft tissue overgrowth and related signs of acromegaly, and a thorough clinical assessment for the presence of comorbidities. Repeat surgery is indicated if there is residual tumor that is surgically accessible and there may be a chance for surgical cure, or if there are persistent mass effects upon the optic chiasm. Otherwise, medical therapy is indicated, utilizing somatostatin analogs, dopamine agonists, and pegvisomant, a GH receptor antagonist. Radiation therapy is usually relegated to situations where medical therapy is ineffective or poorly tolerated or where patients would prefer not to sustain the cost of long-term medical therapy. The choice of therapy requires close dialog among endocrinologists, neurosurgeons, radiation therapists, and neuroophthalmologists for optimal care of patients.
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PMID:Approach to the patient with persistent acromegaly after pituitary surgery. 2082 64


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