Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027651 (tumor)
 685,946 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The eye of a 47 year old man with tyrosinase-positive oculocutaneous albinism, photophobia, nystagmus and visual acuity 0, 4-0, 5 was histologically examined after orbital exenteration for neoplasia. Histologic serial sections of the centre of the retina showed a continuous 6-8 cell-layer of ganglion cells, without any suggestion of a foveolar pit. The outer layers of the macular retina were altered secondarily by tumor-impression-folds; they were unremarkable at the periphery as were the acid mucopolysaccharides in the receptor region. Electron microscopy of the uvea and the retinal pigment epithelium showed a normal number of pigment granules but a deficiency of melanin, as well as structural anomalies. The absence of the foveolar pit and the decrease of visual acuity in tyrosinase-positive albinism is caused by definite morphologic alteration in the arrangement of ganglion cells in the macular region in the sense of a foveolar aplasia. The etiology is discussed. An identic anomaly has been described in aniridia, similar ones in other congenital ocular diseases.
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PMID:[Foveolar aplasia in tyrosinase-positive oculocutaneous albinisim (author's transl)]. 82 41

The new anticancer agent lonidamine has been recently revisited for the treatment of various solid tumors, due to its peculiar and unusual mechanism of action (ie, interference with energy metabolism of tumor cells, morphologically displayed by the appearance of "condensed mitochondria"). First generation trials have in fact demonstrated therapeutic activity and an unusual toxicity profile. Lonidamine is devoid of conventional side effects induced by antiproliferative agents (ie, myelosuppression, stomatitis, cystitis, alopecia, renal, hepatic, and cardiac toxicity). No serious or life-threatening adverse reactions have been recorded even over long term treatment periods. Given as a single agent (in daily doses ranging between 300 and 900 mg) lonidamine induces the following side effects: myalgia, testicular pain, asthenia, ototoxicity, nausea and vomiting, gastric pain, and drowsiness. Hyperesthesia and photophobia have also been reported. In combination with radiotherapy (in oral daily doses ranging between 300 and 450 mg) lonidamine was well tolerated, without any reported evidence of additional toxicity. When associated with cytotoxic agents no enhanced toxicity was observed. In particular, myelosuppression and other conventional nonhematological adverse reactions were never greater than would be expected with chemotherapy alone. The same applies to toxicity and tolerance of lonidamine when used concurrently with hypertermia. The data collected from large series of cancer patients treated with this new agent show that lonidamine is a safe drug whether used alone or in combination with other effective anticancer treatments. The reported therapeutic efficacy and the peculiar toxic profile make lonidamine an interesting new drug for future clinical trials.
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PMID:Toxicity and clinical tolerance of lonidamine. 203 Nov 92

Based on earlier clinical and preclinical investigations, we designed two different pilot trials for patients with nodular lymphoma or chronic lymphocytic leukemia. These studies evaluated the use of either 41.8 degrees C whole body hyperthermia (WBH), or the nonmyelosuppressive chemotherapeutic drug, lonidamine (LON), as an adjunct to total body irradiation (TBI) (12.5 cGy twice a week, every other week for a planned total dose of 150 cGy). Whole body hyperthermia was initiated approximately 10 min after total body irradiation; lonidamine was administered orally (420 mg/m2) on a daily basis. Although entry to the studies was nonrandomized, the two patient populations were accrued during the same time frame and were comparable in terms of histology, stage of disease, performance status, and prior therapy. Of 8 patients entered on the TBI/WBH study, we observed 3 complete responses (CR), 4 partial responses (PR), and 1 improvement (i.e., a 48% decrease in tumor burden). Of 10 patients entered in the TBI/LON study, there was 1 CR and 4 PR. For the TBI/WBH study, myelosuppression was not treatment-limiting; there were no instances of infection or bleeding and platelet support was never required. The median survival time for the TBI/WBH study is 52.5 months based on Kaplan Meir estimates. Two patients remain in a CR. The median time to treatment failure (MTTF) is 9.4 months (90% confidence interval = 7-15.4 months). In the TBI/LON study, 50% of patients receiving TBI required treatment modification due to platelet-count depression during therapy, but there were no instances of infection or bleeding. Frequently observed LON-related toxicities included myalgias, testicular pain, photophobia and ototoxicity. For the TBI/LON study, median survival is 7.6 months; MTTF was 2.4 months. In analyzing the results of these pilot studies, our subjective clinical impressions lead to the hypothesis that WBH protected against TBI-induced thrombocytopenia during therapy, whereas LON had no effect on TBI-induced myelosuppression. This speculation was tested and confirmed in a series of in vitro and in vivo experiments.
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PMID:Adjunctive therapy (whole body hyperthermia versus lonidamine) to total body irradiation for the treatment of favorable B-cell neoplasms: a report of two pilot clinical trials and laboratory investigations. 218 81

Three young children presented with photophobia, epiphora, and torticollis as the initial manifestation of a posterior fossa tumor. In each case there was a delay in treatment due to the presumptive diagnosis of a local ocular inflammatory condition. We recommend that children with unexplained photophobia, epiphora, and torticollis undergo an imaging technique to evaluate the posterior fossa.
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PMID:Photophobia, epiphora, and torticollis: a masquerade syndrome. 239 22

A 38-year-old woman was diagnosed as acute lymphoblastic leukemia (L2) in Oct. 1985. After VP and AdVEMP therapy, complete remission was obtained. In Oct. 1987, she noticed bilateral breast tumors and leukemic cell infiltrations were shown in a biopsy specimen of the breast tumor. Bone marrow was occupied with 94 percent blasts. The second complete remission was achieved by the AdVP therapy. In Nov. 1988, she developed double vision and photophobia. The examinations of CT and MRI showed cavernous sinus tumor, and 20 percent blasts were recognized in a bone marrow aspirate. The leukemic cells were negative for peroxidase, but were positive for both lymphoid and myeloid cell surface markers (CD2, CD5, CD7, CD33). The two color flowcytometry showed that CD5 and CD33 were simultaneously expressed on the leukemic cells.
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PMID:[CD33-positive acute lymphoblastic leukemia with breast tumor and cavernous sinus tumor]. 262 3

Report of a 5 year old Yugoslav girl with xeroderma pigmentosum. This disease was associated with ophthalmological symptoms such as photophobia, blepharospasm and a tumor of the cornea. Based on this case the ocular abnormalities in xeroderma pigmentosum are reviewed in the literature.
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PMID:[Eye changes in xeroderma pigmentosum]. 739 11

A 62-year-old male had undergone lung lobectomy for adenocarcinoma which was discovered after visual loss from paraneoplastic retinopathy. He had photopsia, decreased visual acuity, ring scotoma, extremely reduced electroretinogram and narrowing of retinal artery. Fluorescein angiography revealed progressive diminution of peripheral retinal blood flow, slow perfusion and staining of venules. The surgical removal of the tumor resulted in slight recovery of visual acuity and decrease of photopsia and photophobia, which recurred after 8 months. He has been under steroid therapy to prevent the progression of the disease for 10 months.
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PMID:A case of paraneoplastic retinopathy. 832 Aug 62

We report the case of a 60-year-old woman in whom migraine with typical aura heralded the presence of an occipital lobe tumor. Her headache was characterized by recurrent episodes of visual aura confined to the left visual field followed by right hemicranial throbbing headaches accompanied by nausea, photophobia, and phonophobia. Interictal neurologic and ophthalmologic examinations were negative, as was an unenhanced brain CT scan. The headaches increased in frequency over 4 months despite a number of medications known to prevent attacks of migraine. A low-grade right occipital lobe tumor was eventually discovered on MR scan. This case illustrates that headache fulfilling the International Headache Society (IHS) criteria for migraine with typical aura can occur in association with an occipital lobe tumor.
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PMID:Occipital lobe tumor presenting as migraine with typical aura. 907 98

An eight-year-old boy presented with left eye pain, photophobia, proptosis, third nerve paresis and decreased visual acuity. Magnetic resonance imaging revealed a nonenhancing mass filling the cavernous sinus. Using an extradural fronto-orbitozygomatic approach, the cavernous sinus was approached laterally, and a teratoma was removed from within the cavernous sinus. This is the first case of a truly intracavernous teratoma in a child and the fourth case of a teratoma reported in the cavernous sinus region overall. This report outlines the diagnosis and treatment of this unusual cavernous sinus tumor.
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PMID:Intracavernous teratoma in a school-aged child. 1035 16

Bryostatin-1, a macrocyclic lactone, appears to elicit a wide range of biological responses including modulation of protein kinase C (PKC). PKC, one of the major elements in the signal transduction pathway, is involved in the regulation of cell growth, differentiation, gene expression, and tumor promotion. Because of the potential for a unique mechanism of interaction with tumorgenesis, a Phase I trial of bryostatin-1 was performed in children with solid tumors to: (a) establish the dose-limiting toxicity (DLT) and maximum-tolerated dose (MTD); (b) establish the pharmacokinetic profile in children; and (c) document any evidence of antitumor activity. A 1-h infusion of bryostatin-1 in a PET formulation (60% polyethylene glycol 400, 30% ethanol, and 10% Tween 80) was administered weekly for 3 weeks to 22 children (age range, 2-21 years) with malignant solid tumors refractory to conventional therapy. Doses ranged from 20 to 57 microg/m2/ dose. Pharmacokinetics were performed in at least three patients per dose level. The first course was used to determine the DLT and MTD. Twenty-two patients on five dose levels were evaluable for toxicities. At the 57 microg/m2/dose level dose-limiting myalgia (grade 3) was observed in three patients; two of those patients also experienced photophobia or eye pain, and one experienced headache. Symptoms occurred in all patients within 24-72 h after the second dose of bryostatin-1 with resolution within 1 week of onset. Other observed toxicities (grades 1 and 2) included elevation in liver transaminases, thrombocytopenia, fever, and flu-like symptoms. The bryostatin-1 infusion was typically well tolerated. Although stable disease was noted in several patients, no complete or partial responses were observed. The recommended Phase II dose of bryostatin-1 administered as a 1-h infusion weekly for 3 of every 4 weeks to children with solid tumors is 44 microg/m2/dose. Myalgia, photophobia, or eye pain, as well as headache, were found to be dose limiting.
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PMID:A Phase I trial of bryostatin-1 in children with refractory solid tumors: a Pediatric Oncology Group study. 1049 3


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