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)

Few cases of teratoma in the third ventricle have been reported in detail. We describe a 3-year-old Chinese boy presenting with increased intracranial pressure, headache, and vomiting. Preoperative computed tomography and magnetic resonance imaging demonstrated a teratoma in the third ventricle. Complete excision of the tumor was achieved through the interhemispheric transcallosal approach. The patient did well and was without neurologic deficits postoperatively. Although the majority of tumors located in the third ventricle are malignant and can penetrate the brain tissues that constitute the wall of the third ventricle, mature teratomas are an exception and total removal may be possible.
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PMID:Successful resection of a teratoma of the third ventricle in a 3-year-old boy. 954 75

A 21-year-old male presented with a pineal dermoid cyst manifesting as headache and diplopia. He had undergone gross total removal of a pineal mature teratoma 18 years before and had done well until recently. Diffusion-weighted magnetic resonance imaging showed a region of high signal intensity due to a round mass in the pineal region and extending into the trigone of the right lateral ventricle. Subtotal excision of the tumor was achieved. Histological examination showed an epidermoid cyst consisting of keratinized stratified squamous epithelium and connective tissue. Intraoperative observation had detected black hairs, so the diagnosis was dermoid cyst. The dermoid cyst may have arisen from a microscopic remnant of the cyst wall of the original pineal mature teratoma. The regrowth of a dermoid cyst 18 years after gross total removal of a mature cystic teratoma in the pineal region is exceptional. However, careful follow-up of patients who undergo gross total removal of a pineal teratoma is recommended for a period more than that of the patient's age at surgery plus 9 months.
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PMID:Pineal dermoid cyst developing 18 years after gross total removal of a pineal mature teratoma. 964 Sep 67

We reported two cases of chemotherapy-refractory testicular cancer treated with all trans-retinoic acid (ATRA). Case 1. A 21-year-old male patient underwent salvage surgery for lung metastasis which had developed after treatment with three different cisplatin-based chemotherapy regimens for malignant teratoma. After recovery from surgery, he was treated with oral ATRA at daily dose 80 mg/m2 for four weeks. Case 2. A-45-year-old patient suffered from lung metastasis after orchiectomy for teratocarcinoma. The patient failed to achieve a complete response despite two different cisplatin-based chemotherapy and high dose chemotherapy regimens with bone marrow rescue. He was treated with oral ATRA for five weeks. Both patients showed disease progression with increase in tumor size and elevation of tumor marker during ATRA therapy. Side effects were acceptable except the headache in Case 2, who needed a dose reduction of ATRA. In conclusion, oral ATRA with this dose failed to show clinical antitumor activity in patients with refractory testicular cancer.
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PMID:[Cases of refractory testicular cancer treated with all trans-retinoic acid]. 1041 Jan 56

A 26-year-old, short statured, obese male presented with a mature teratoma located entirely within the dural confines of the sella manifesting as headaches and progressive loss of vision. He had panhypopituitarism. Magnetic resonance imaging showed a large sellar-suprasellar but entirely infradiaphragmatic tumor of varying consistency. The tumor was resected through a trans-sphenoidal route. The tumor had elevated the diaphragma sellae to a significant extent but did not pass through. Histological examination confirmed a mature teratoma.
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PMID:Mature teratoma arising from the sella--case report. 1148 1

This study evaluates the diagnosis, therapy and survival of 14 patients with primary intracranial germ cell tumors during the period from 1991 to 2001. There were 11 males and 3 females. Mean age was 12.2 years old (20 days-18 years). On admission, the most common symptoms were headache (10/14), vomiting (6/14) and visual (6/14). The tumor was in pineal and hypothalamic region in 10 cases, suprasellar in 3 cases, and in the cerebral parenchyma in 1 case. Histologically there were 1 embryonal carcinoma, 5 germinomas, 2 mature teratomas, 1 immature teratoma and 5 mixed germ cell tumors. Treatment differed among the patients according to the type of tumor. Three patients died after tumor progression or relapse and one patient died from another condition. The remaining patients are alive and without disease.
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PMID:[Primary intracranial germ cell tumors in children: evaluation of fourteen cases]. 1625 65

Extragonadal teratomas in adulthood are exceptionally rare and usually not located within the cerebellum. We here report on a 66-year-old male patient clinically presenting with chronic occipital headache and episodes of severe vertigo. Neuroradiological investigations revealed a hemorrhagic tumor mass in the cerebellar vermis which was surgically removed and histologically diagnosed as mature teratoma. Hence, the presented case is extraordinary with regard to age, late clinical onset of symptoms and cerebellar location. Late clinical manifestation of the tumor in this case is probably due to an acute late-onset hemorrhage within the tumor.
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PMID:Mature cerebellar teratoma in adulthood. 1862 82

Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is a new category of treatment-responsive encephalitis associated with "anti-NMDAR antibodies", which are antibodies to the NR1/NR2 heteromers of NMDAR. The antibodies are detected in the CSF/serum of young women with ovarian teratoma, who typically develop schizophrenia-like psychiatric symptoms, usually preceded by fever, headache, or viral infection-like illness. After reaching the peak of psychosis, most patients developed seizures followed by an unresponsive/catatonic state, decreased level of consciousness, central hypoventilation frequently requiring mechanical ventilation, orofacial-limb dyskinesias, and autonomic symptoms. Brain MRI is usually unremarkable but focal enhancement or medial temporal lobe abnormalities can be observed. The CSF reveals nonspecific changes. EEG often reveals diffuse delta slowing without paroxysmal discharges, despite frequent bouts of seizures. This is a highly characteristic syndrome evolving in 5 stages, namely, the prodromal phase, psychotic phase, unresponsive phase, hyperkinetic phase, and gradual recovery phase. The hyperkinetic phase is the most prolonged and crucial. This disorder is usually severe and can be fatal, but it is potentially reversible. Once patients overcome the hyperkinetic phase, gradual improvement is expected with in months and full recovery can also be expected over 3 or more years. Ovarian teratoma-associated limbic encephalitis (OTLE) was first reported in 1997 when this syndrome was reported independently in 1 Japanese girl and 1 woman, both of whom improved following tumor resection. In 2005, Dalmau and his research group first demonstrated antibodies to novel neuronal cell membrane antigens in 4 women with OTLE in a non-permeabilized culture of hippocampal neurons. Two years later, they identified conformal extracellular epitopes present in the NR1/NR2B heteromers of NMDAR, which are expressed in the hippocampus/forebrain. The target extracellular epitopes are not detectable by immunoblotting, and should not be confused with the linear epitopes of NR2B subunits (also known as epsilon2). The antibodies disappear with clinical improvement, suggesting their pathogenic role. Autopsies revealed IgG deposits in the hippocampus, extensive microgliosis, rare T-cell infiltrates, and neuronal degeneration predominantly involving, but not restricted to, the hippocampus. The nervous tissues of the tumors exhibit not only strong expression of the NR2B subunits but also reactivity with the patients' antibodies. The pathogenesis remains unknown; however, this disorder is considered to be an antibody-mediated encephalitis. Based on the current NMDAR hypofunction hypothesis of schizophrenia, we speculate that the antibodies may cause inhibition rather than stimulation of NMDARs in presynaptic GABAergic interneurons, causing a reduction in GABA release. This results in disinhibition of postsynaptic glutamatergic transmission, excessive release of glutamate in the prefrontal/subcortical structures, and glutamate and dopamine dysregulation that might contribute to development of schizophrenia-like psychosis and bizarre dyskinesias. The antibodies were initially found only in young women with teratoma in the ovaries. However, recent studies show that this disorder can occur even in the absence of teratoma in up to 35% of patients, and even boys and adult men had been affected. Although recovery occurs without the need for tumor removal, the severity and extended duration of symptoms support tumor removal. Combined therapy including tumor resection and immunotherapy is recommended. In this review, we also discuss the relationship between anti-NMDAR encephalitis and related disorders, including acute diffuse lymphocytic meningoencephalitis and acute juvenile female non-herpetic encephalitis (AJFNHE).
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PMID:[Anti-nMDA receptor encephalitis--clinical manifestations and pathophysiology]. 1880 39

Sinonasal teratocarcinosarcoma is a highly malignant, polymorphous neoplasm that combines features of carcinosarcoma and teratoma. We describe the clinicopathologic features and management of a well-documented example of this unique entity that involved a 41-year-old Hispanic man. The patient presented with a history of multiple episodes of epistaxis, nasal obstruction and frontal headaches. Computerized tomography scans and magnetic resonance imaging revealed a large mass filling the left nasal cavity and extending to the cribriform plate with involvement of the ethmoid sinuses, lamina papyracea, and orbit. The patient underwent a complex procedure for a T3N0 tumor. Histologic examination revealed a heterogeneous admixture of epithelial, mesenchymal, and neuroepithelial elements. The mesenchymal components consist of fibrous stroma and myxomatous areas, labeled with calponin and smooth muscle actin. The epithelial components vary from clear cells, nonkeratinizing epithelium to glandular pattern, and keratin containing cysts. Immature neuroepithelium and olfactory neuroblastomalike tissue are highlighted with neuroendocrine markers. Postoperatively, the patient had a rapid local recurrence of the tumor and underwent reexcision, and was treated with radiotherapy and chemotherapy. Twelve months after his primary resection, computerized tomography scans revealed an intrathoracic tumor with dominant mass in the left hilum and metastases to the mediastinum, left pleural space, and both lungs. The histologic nature of his chest mass remains undetermined. Among 54 cases of reported sinonasal teratocarcinosarcoma, 67% of patients with initial single surgical resection and 80% of patients primarily treated with radiotherapy had recurrence, or metastatsis, or unresponsiveness to treatment. The high rate of local recurrence and metastasis is indicative of its highly aggressive biologic behavior. Almost half of the patients died of tumor within 3 years of diagnosis, despite aggressive therapy. Seventy percent of the patients who survived more than 1 year had the initial therapeutic regiments of combined surgery and adjuvant therapies, suggesting that aggressive therapeutic approaches may improve the treatment outcome.
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PMID:Sinonasal teratocarcinosarcoma: report of a case with review of literature and treatment outcome. 1899 6

Sinonasal teratocarcinosarcoma (SNTCS) is a rare and highly malignant tumour with combined features of a teratoma and carcinosarcoma. We report the first case of a SNTCS in 23 year old male treated with neo-adjuvant chemotherapy followed by cranio-facial resection. The resection specimen displayed cellular maturation in the neuroectodermal component. The patient presented with a short history of nasal obstruction, epistaxis and headache. On imaging, a bone destroying lesion of left paranasal sinuses and nasal cavity was identified. The diagnosis of SNTCS could be offered only on the third biopsy which showed heterogeneous admixture of primitive neuroectodermal, epithelial and mesenchymal elements. An adequate sampling with high index of suspicion is needed to catch hold this rare tumor. Tumor was excised after 4 cycles of neo-adjuvant chemotherapy. On microscopic examination, it showed similar epithelial and mesenchymal components as the pretreatment biopsies. However, the primitive neuroectodermal component displayed extensive neuronal maturation. The undifferentiated neuroectodermal cells were completely absent in the post chemotherapy specimen. This case throws light on the morphologic evidence of chemotherapy induced maturation in the neuroectodermal component within SNTCS, an event hitherto not reported in the literature in case of SNTCS.
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PMID:Chemotherapy-induced neuronal maturation in sinonasal teratocarcinosarcoma--a unique observation. 2059 86

Mature cystic teratoma (dermoid cyst) inside the Sylvian fissure is rare. A 14-year-old boy presented with 2 episodes of generalized tonic-clonic seizures. Using a fat-suppressed, T1-weighted sequence, magnetic resonance imaging revealed a hypointense nonenhancing mass in the left Sylvian fissure. He underwent left pterional craniotomy for total tumor resection. The pathological diagnosis was mature cystic teratoma (dermoid cyst). Headache and seizures are the leading symptoms. Cyst rupture causes inflammation of cholesterol crystals, and the cyst contents may cause seizure. Surgical resection is the treatment of choice, but radical resection is not advised if critical neurovascular structure can be injured.
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PMID:Mature cystic teratoma (dermoid cyst) in the sylvian fissure: a case report and review of the literature. 2219 May 4


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