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)

Thirty-seven patients with retinoblastoma were evaluated prospectively by clinical examination, lumbar puncture, and CT. Eight (22%) of the 37 were found to have meningeal dissemination. The tumor was bilateral in three patients. Two cases showed no CT signs of local recurrence. Headache, nausea/vomiting, and restlessness were the most common symptoms. CT scans in these patients showed diffuse meningeal contrast enhancement, nodular masses, ependymal-subependymal enhancement, and ventricular dilatation. Our series of eight patients with meningeal spread illustrates a considerable range of dissemination patterns referable to retinoblastoma. One case illustrated the CT finding of multiple epidural metastases.
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PMID:Meningeal dissemination of retinoblastoma: CT findings in eight patients. 212 Oct 4

Two cases of brain tumors which developed after radiotherapy against retinoblastomas are reported. A 17-year-old girl was admitted with a chief complaint of swelling in her forehead after head injury in July, 1981. At 7 months old her left eye had been enucleated and she had received radiation therapy of 40 Gy to the right eye because of her bilateral retinoblastoma. On admission a CT scan revealed a high density mass with a partial low density area in her right middle fossa. A right frontotemporal craniotomy was performed and the tumor was removed subtotally, which was diagnosed as a malignant fibrous histiocytoma. The second case was a 14-year-old boy, who had received radiotherapy of 39.6 Gy against retinoblastoma of the right eye after enucleation at 2 months old. He had been well for 14 years after the therapy and was admitted to the hospital with complaints of headache, nausea, vomiting and unsteady gait in January 1985. A CT scan demonstrated a large contrast enhancing mass in the righ middle fossa, which was removed subtotally on January 14, 1985. A histological diagnosis of fibroblastic meningioma was made. Each patient developed a secondary brain tumor after radiotherapy against retinoblastoma. Those tumors appear to be radiation induced, although 10 to 15% of the patients who survived retinoblastoma without radiotherapy had been reported to develop secondary nonocular tumors. The patients with retinoblastoma should be followed up carefully after the initial treatment.
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PMID:[Two cases of the middle fossa tumor following radiotherapy against retinoblastoma]. 282 45

Of 23 cases of metastatic retinoblastoma treated between 1922 and 1979, seven had metastases limited to the cranial vault and 13 had cranial metastases plus distant metastases. Globe pathology showed invasion of the optic nerve and/or the choroid in all but two patients, but was not predictive of the metastatic pattern. Initial signs of metastases were neurologic impairment and an orbital or body mass; first symptoms were anorexia or weight loss, vomiting, and headache. Most cases occurred by 3 years of age. Death occurred within 5.8 months on the average, despite therapy. Useful tests for determining the extent of disease were bone marrow aspiration, lumbar puncture, skull films, EEG, and brain scan. Computed tomographic scans of the head, bone scans, bone marrow aspiration, automated blood chemistry analysis, and lumbar puncture with immediate ethyl alcohol processing should prove to be useful to detect metastatic disease.
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PMID:Metastatic patterns of retinoblastoma. 670 86

Among the 185 retinoblastoma patients seen at the Lausanne Retinoblastoma Clinic from 1963-1993, 24 (14%) first presented with another sign than classical leukocoria (60.5%) or strabismus (21.5%). Most of these atypical signs were related to inflammatory complications of unrecognized retinoblastoma; they consisted of low vision (1.5%), hypopyon (2%), ocular redness and pain (1.5%), ocular redness and buphtalmia (1.5%), as well as photophobia and headaches (1.5%). The presence of unexplained chronic ocular signs during childhood should always raise the possibility of an underlying retinal malignancy.
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PMID:[Unusual presentation of retinoblastoma]. 805 60

The majority of hematopoietic malignancies have aberrancies in the retinoblastoma (Rb) pathway. Loss in Rb function is, in most cases, a result of the phosphorylation and inactivation of Rb by the cyclin-dependent kinases (cdks), main regulators of cell cycle progression. Flavopiridol, the first cdk modulator tested in clinical trials, is a flavonoid that inhibits several cdks with evidence of cell cycle block. Other interesting preclinical features are the induction of apoptosis, promotion of differentiation, inhibition of angiogenic processes and modulation of transcriptional events. Initial clinical trials with infusional flavopiridol demonstrated activity in some patients with non-Hodgkin's lymphoma, renal, prostate, colon and gastric carcinomas. Main side-effects were secretory diarrhea and a pro-inflammatory syndrome associated with hypotension. Phase 2 trials with infusional flavopiridol in CLL and mantle cell lymphoma, other schedules and combination with standard chemotherapies are ongoing. The second cdk modulator tested in clinical trials, UCN-01, is a potent protein kinase C inhibitor that inhibits cdk activity in vitro as well. UCN-01 blocks cell cycle progression and promotes apoptosis in hematopoietic models. Moreover, UCN-01 is able to abrogate checkpoints induced by genotoxic stress due to modulation in chk1 kinase. The first clinical trial of UCN-01 demonstrated very prolonged half-life (approximately 600 h), 100 times longer than the half-life observed in preclinical models. This effect is due to high binding affinity of UCN-01 to the human plasma protein alpha-1-acid glycoprotein. Main side-effects in this trial were headaches, nausea/vomiting, hypoxemia and hyperglycemia. Clinical activity was observed in patients with melanoma, non-Hodgkin's lymphoma and leiomyosarcoma. Of interest, a patient with anaplastic large cell lymphoma refractory to high-dose chemotherapy showed no evidence of disease after 3 years of UCN-01 therapy. Trials of infusional UCN-01 in combination with Ara-C or gemcitabine in patients with acute leukemia and CLL, respectively, have commenced. In conclusion, flavopiridol and UCN-01 are cdk modulators that reach biologically active concentrations effective in modulating CDK in vitro, and show encouraging results in early clinical trials in patients with refractory hematopoietic malignancies. Although important questions remain to be answered, these positive experiences will hopefully increase the therapeutic modalities in hematological malignancies.
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PMID:Development of cyclin-dependent kinase modulators as novel therapeutic approaches for hematological malignancies. 1124 75

The majority of human malignancies have aberrancies in the Retinoblastoma (Rb) pathway. Loss in Rb function results from the phosphorylation and inactivation of Rb by the cyclin-dependent kinases (cdks), main regulators of cell cycle progression. Thus, modulators of cdks may have a role in the treatment of human malignancies. Flavopiridol, the first cdk modulator tested in clinical trials, demonstrates interesting preclinical features: cell cycle block, induction of apoptosis, promotion of differentiation, inhibition of angiogenic processes and modulation of transcriptional events. Initial clinical trials with infusional flavopiridol demonstrated activity in some patients with lymphomas and renal, colon gastric carcinomas. Main side effects were diarrhea and hypotension. Phase 2 trials with infusional flavopiridol, other schedules and combination with standard chemotherapies are ongoing. The second cdk modulator tested in clinical trials, UCN-01, is a PKC inhibitor that can also modulate cdk activity. Similar to flavopiridol, UCN-01 blocks cell cycle progression and promotes apoptosis. Moreover, UCN-01 may abrogate checkpoints induced by genotoxic stress due to inhibition of chk1 kinase. The first clinical trial of UCN-01 demonstrated very prolonged half-life (approximately 600 h), due to high binding affinity of UCN-01 to the human alpha-1-acid glycoprotein. Main side effects were headaches, vomiting, hypoxemia and hyperglycemia. Clinical activity was observed in some patients with melanoma and lymphoma. Trials of shorter infusions of UCN-01 or in combination with standard chemotherapeutic agents are ongoing. Although several important basic and clinical questions remain unanswered, development of cdk modulators is a reasonable strategy for cancer therapy.
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PMID:Small molecule modulators of cyclin-dependent kinases for cancer therapy. 1142 45

We present an extremely rare case of pinealoblastoma with retinoblastic differentiation in a 32-year-old woman who presented with a history of intermittent headache of 2 years duration and diminution of vision for 2 months which eventually lead to total loss of vision. The fundus examination showed bilateral secondary optic atrophy. She did not have any previous history of retinoblastoma. The family history was non-contributory. Paraffin section of the tumor showed a primitive neuroectodermal tumor with numerous Flexner-Wintersteiner rosettes and the tumor cells were strongly positive for synaptophysin and negative for GFAP, S-100 protein and epithelial membrane antigen. This is the first case in the literature of a sporadic case of pinealoblastoma with prominent retinoblastic differentiation as evidenced histomorphologically by the presence of numerous Flexner-Wintersteiner rosettes in an adult female.
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PMID:Pinealoblastoma with prominent retinoblastic differentiation: an unusual case in an adult. 2005 Oct 15

Reversible cerebral vasoconstriction syndrome is a rare cause of headache and stroke in the pediatric population. Reversible vasoconstriction is reported in a 19-month-old girl with retinoblastoma who underwent selective ophthalmic artery infusion chemotherapy with melphalan. Procedure-related cerebral vasoconstriction was specifically triggered during coadministration of adjunctive medications, which included mydriatic eye drops containing phenylephrine, intranasal oxymetazoline, nebulized albuterol, intravenous hydrocortisone, and intravenous diphenhydramine. The course of cerebral vasospasm, which began with a severe hypertensive surge and resolved spontaneously within hours of blood pressure normalization, was documented by angiography in real time. Subsequent brain magnetic resonance imaging showed no evidence of perfusion abnormality, cerebral infarction, or cerebral hemorrhage, and the patient was discharged home without any neurologic sequelae. In this report, we highlight the potential risk of reversible cerebral vasoconstriction in children administered vasoactive drugs and discuss its relevance during treatment of retinoblastoma by intraarterial chemotherapy.
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PMID:Cerebral vasoconstriction triggered by sympathomimetic drugs during intra-atrerial chemotherapy. 2333 8

Although cancer in children is rare, it is the second most common cause of childhood mortality in developed countries. It often presents with nonspecific symptoms similar to those of benign conditions, leading to delays in the diagnosis and initiation of appropriate treatment. Primary care physicians should have a raised index of suspicion and explore the possibility of cancer in children who have worrisome or persisting signs and symptoms. Red flag signs for leukemia or lymphoma include unexplained and protracted pallor, malaise, fever, anorexia, weight loss, lymphadenopathy, hemorrhagic diathesis, and hepatosplenomegaly. New onset or persistent morning headaches associated with vomiting, neurologic symptoms, or back pain should raise concern for tumors of the central nervous system. Palpable masses in the abdomen or soft tissues, and persistent bone pain that awakens the child are red flags for abdominal, soft tissue, and bone tumors. Leukokoria is a red flag for retinoblastoma. Endocrine symptoms such as growth arrest, diabetes insipidus, and precocious or delayed puberty may be signs of endocranial or germ cell tumors. Paraneoplastic manifestations such as opsoclonus-myoclonus syndrome, rheumatic symptoms, or hypertension are rare and may be related to neuroblastoma, leukemia, or Wilms tumor, respectively. Increased suspicion is also warranted for conditions associated with a higher risk of childhood cancer, including immunodeficiency syndromes and previous malignancies, as well as with certain genetic conditions and familial cancer syndromes such as Down syndrome, Li-Fraumeni syndrome, hemihypertrophy, neurofibromatosis, and retinoblastoma.
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PMID:Signs and symptoms of childhood cancer: a guide for early recognition. 2393 97